CASOS RELEVANTES

DE
DERMATOPATOLOGIA
André Cartell
schleper@terra.com
O QUE PODE CAIR NO TED
 a intenção não é adivinhar o que vai cair no
TED, mas dentro de diagnósticos difíceis
prevenir o residente para algumas
possibilidades interessantes
 durante o curso foram lembrados algumas
possibilidades, pois geram fotos elegantes –
líquen plano, cilindroma, CBC, micoses, etc
 o centerfold dos archives é sempre uma
boa fonte de estudo
 se eu acertar 3 questões, tem de pagar
uma janta!
O QUE PODE CAIR NO TED
1. porokeratosis ptychotropica
2. síndrome de Bannayan-Riley-Ruvalcaba
3. prototecose
4. amebíase
5. microscopia confocal de ocronose
6. dermatoscopia de acantoma de células claras
7. complexo de Carney (LAMB/NAME)
8. dermatopatia fibrosante nefrogêncica
9. variantes de MF – Woringer-Kollop,
granulomatous slack skin e mucinose folicular
10.ATCL – flower cell
O QUE PODE CAIR NO TED
11. síndrome de Birt-Hogg-Dubbé
12. síndrome de Brooke-Spiegler
13. hemangioma glomeruloide e síndrome de POEMS
14. estrongiloidíase
15. doença de Flegel
16. pyostomatitis vegetans
17. tatuagem por amálgama
18. síndrome de Sneddon
19. malacoplaquia e von Kossa
20. granuloma secundário a preenchedor dérmico
CASOS LEGAIS NA CLÍNICA COM FOTOS RUINS NA
PATOLOGIA – alguns exemplos
 ICTIOSES – Netherton, Sjögren–Larsson, eritroqueratodermia
variabilis e pitiríase rotunda
 HIPERCERATOSES PALMO-PLANTARES – todas
 BOLHOSAS – epidermólises bolhosas genéticas, penfigoide
gestacional e penfigoide cicatricial
 ECZEMAS & DESCAMATIVAS – pitiríase alba, síndrome de
Gianotti-Crosti, eritrodermia
 INTERFACE – liquen striatus, keratosis lichenoide chronica;
atrofodermia, dermatomiosite
 VASCULOPÁTICAS – eritemas figurados, EPS, VLINs –
perniose, DPP, PUPPP; pioderma gangrenoso e Behçet
 PROLIFERATIVAS – acantose nigricante X PRCGC
 FANTASMAS – vitiligo, nevo de Becker, anetodermia,
hipomelanose de Ito, Ehler-Danlos, dermatocalázio
POROKERATOSIS PTYCHOTROPICA
Clin Exp Dermatol. 2009 Dec;34(8):e895-7.
Porokeratosis ptychotropica: a lesser-known
variant. Tallon B, Blumental G, Bhawan J.
Abstract – We report a case of the rare porokeratosis
variant porokeratosis ptychotropica (PP). A
circumferential perianal plaque and the characteristic
histology of multiple cornoid lamellae with underlying
dermal amyloid deposition were seen. Amyloid
deposition was seen in the biopsied intertriginous area
of the plaque only, which, in concordance with other
cutaneous amyloid deposition disorders, may suggest a
role for friction in the pathogenesis of this histological
finding. We review the literature on PP and summarize
the poor response seen to treatments.
POROKERATOSIS PTYCHOTROPICA
J Cutan Pathol. 2010 Jul;37(7):802-7
Verrucous porokeratosis of the gluteal cleft (porokeratosis
ptychotropica): a rare disorder easily misdiagnosed.
Takiguchi RH, White KP, White CR Jr, Simpson EL.
Abstract – porokeratosis represents a heterogeneous group of
disorders characterized clinically by a distinctive ridge-like border
and histologically by cornoid lamellae. A verrucous variant of
porokeratosis involving the gluteal cleft has been recently
described. We present 5 new cases and review the current literature
to highlight the clinical and histopathologic features of this disorder.
Descriptive terms including hyperkeratotic porokeratosis,
genitogluteal porokeratosis, porokeratoma, follicular
porokeratosis, and porokeratosis ptychotropica have all been
used to describe this verrucous variant of porokeratosis involving
the gluteal cleft. To avoid further confusion, we propose a
consolidation of terminologies and suggest verrucous porokeratosis
be added to the commonly described variants of porokeratosis.
1-POROKERATOSIS PTYCHOTROPICA
J Am Acad Dermatol. 2009 Mar;60(3):501-3.
Porokeratosis ptychotropica: a clinically distinct variant
of porokeratosis.
McGuigan K, Shurman D, Campanelli C, Lee JB.
Abstract – porokeratosis represents a spectrum of clinical
disease. Multiple variants have been described including
porokeratosis ptychotropica, a rare subtype. The clinical
presentation of porokeratosis ptychotropica frequently
resembles an inflammatory perianal disease. We report a
patient with porokeratosis ptychotropica with coexistent
disseminated superficial actinic porokeratosis. We
review the current literature on porokeratosis ptychotropica
including the clinical presentation, histopathology, cause,
and pathogenesis of this rare variant of porokeratosis.
2-S. DE BANNAYAN-RILEY-RUVALCABA
rare hamartomatous disorder with occurrence of
multiple subcutaneous lipomas, macrocephaly and
hemangiomas
the disease is inherited in an autosomal dominant
form, but sporadic cases have been reported
belongs to a family of hamartomatous polyposis
syndromes, which also includes Peutz-Jeghers
syndrome, juvenile polyposis and Cowden syndrome
mutation of the PTEN gene underlies this syndrome,
as well as Cowden syndrome, Proteus syndrome, and
Proteus-like syndrome; collectively, these four
syndromes are referred to as PTEN Hamartoma-Tumor
Syndromes (PHTS)
2-S. DE BANNAYAN-RILEY-RUVALCABA
J Pediatr Surg. 2006 Sep;41(9):1601-3.
Cutaneous lipoma in children: 5 cases with Bannayan-Riley-
Ruvalcaba syndrome.
Buisson P, et al.
Abstract – cutaneous lipoma is rare in children, but it can be part
of a syndrome such as the Bannayan-Riley-Ruvalcaba syndrome
(BRRS). The BRRS is a dominant autosomal disorder
characterized by cutaneous lipomas, macrocephaly, intestinal
polyps, and developmental delay associated with PTEN gene
mutations. This syndrome is thought to represent a pediatric form
of the Cowden syndrome, characterized among other features by
an increased risk of cancer. We report 5 cases of BRRS, all
diagnosed in children with lipoma and macrocephaly. Children
presenting with lipomas need a complete physical examination to
look for other signs of BRRS, because they may need further
follow-up for tumor screening in adulthood.
2-S. DE BANNAYAN-RILEY-RUVALCABA
Genet Med. 2009 Oct;11(10):687-94.
PTEN hamartoma tumor syndrome: an overview. Hobert JA, Eng C.
Abstract – PTEN hamartoma tumor syndrome (PHTS) encompasses
four major clinically distinct syndromes associated with germline mutations
in the tumor suppressor PTEN. These allelic disorders, Cowden
syndrome, Bannayan-Riley-Ruvalcaba syndrome, Proteus syndrome,
and Proteus-like syndrome are associated with unregulated cellular
proliferation leading to the formation of hamartomas. Thus far, an increased
risk of malignancy has only been documented in Cowden syndrome;
however, current recommendations advise that all individuals with PTEN
hamartoma tumor syndrome follow the cancer surveillance strategies
suggested for Cowden syndrome until further data indicate otherwise.
Because any individual phenotypic feature of Cowden syndrome and
Bannayan-Riley-Ruvalcaba syndrome are frequently present in the
general population, many individuals often go undiagnosed and
consequently do not benefit from available cancer surveillance strategies.
Therefore, it is critical for clinicians to recognize the phenotypic features
associated with these syndromes to accurately diagnose and provide
preventative care. This overview details the clinical description of the PTEN
hamartoma tumor syndrome and associated disorders, their diagnosis and
molecular/genetic testing, as well as differential diagnosis for assessment of
other hamartoma-associated syndromes.
2-S. DE BANNAYAN-RILEY-RUVALCABA
Hum Mutat. 2003 Sep;22(3):183-98.
PTEN: one gene, many syndromes. Eng C.
Abstract - PTEN, on 10q23.3, encodes a major lipid phosphatase which signals down the
phosphoinositol-3-kinase/Akt pathway and effects G1 cell cycle arrest and apoptosis.
Germline PTEN mutations have been found to occur in 80% of classic Cowden syndrome
(CS), 60% of Bannayan-Riley-Ruvalcaba syndrome (BRRS), up to 20% of Proteus
syndrome (PS), and approximately 50% of a Proteus-like syndrome (PSL). CS is a
heritable multiple hamartoma syndrome with a high risk of breast, thyroid, and endometrial
carcinomas. BRRS is a congenital autosomal dominant disorder characterized by
megencephaly, developmental delay, lipomatosis, and speckled penis. PS and PSL had
never been associated with risk of malignancy. Finding germline PTEN mutations in patients
with BRRS, PS, and PSL suggests equivalent risks of developing malignancy as in CS with
implications for medical management. The mutational spectra of CS and BRRS overlap, with
many of the mutations occurring in exons 5, 7, and 8. Genotype-phenotype association
analyses have revealed that the presence of germline PTEN mutations is associated with
breast tumor development, and that mutations occurring within and 5' of the phosphatase
motif were associated with multi-organ involvement. Pooled analysis of PTEN mutation series
of CS and BRRS occurring in the last five years reveals that 65% of CS-associated mutations
occur in the first five exons encoding the phosphatase domain and the promoter region, while
60% of BRRS-associated mutations occur in the 3' four exons encoding mainly the C2
domain. Somatic PTEN mutations occur with a wide distribution of frequencies in sporadic
primary tumors, with the highest frequencies in endometrial carcinomas and glioblastoma
multiform. Several mechanisms of PTEN inactivation occur in primary malignancies derived
from different tissues, but a favored mechanism appears to occur in a tissue-specific manner.
Inappropriate subcellular compartmentalization and increased/decreased proteosome
degradation may be two novel mechanisms of PTEN inactivation. Further functional work
could reveal more effective means of molecular-directed therapy and prevention.
3-prototecose
aspectos gerais
agente: Chlorella (alga verde), P. wickerhamii
1. microorganismo ubíquo
2. infecção por inoculação ou via ferimento pré-existente
3. não ocorre transmissão pessoa-a-pessoa
4. baixa virulência
5. imunocomprometidos – formas sistêmicas: pele, sangue,
peritônio, TGI, fígado e meninges
6. menos de 100 casos publicados nos EUA desde 1964
7. endosporos arredondados com 6 a 10 micrômetros,
intracitoplasmáticos ou livres, padrão moruliforme
8. granulomas supurativos clássicos
3-PROTOTECOSE
Med Mycol. 2004 Apr;42(2):95-106.
Protothecosis.
Leimann BC, Monteiro PC, Lazéra M, Candanoza ER, Wanke B.
Mycology Service, Evandro Chagas Institute of Clinical Research,
IPEC/FIOCRUZ, Rio de Janeiro, Brazil. leimann@centroin.com.br
Abstract
Protothecosis is an infection caused by achlorophyllic algae of the genus
Prototheca which rarely affects humans. Some 100 cases have been
described in the medical literature, the majority caused by the species P.
wickerhamii. The skin is the organ most frequently involved. Diagnosis is
performed by isolation of the microorganism in culture or by histopathology.
The ideal treatment has not been defined, with amphotericin B and the
azoles having been employed. Surgical excision is recommended for small,
localized lesions. We describe a case of cutaneous protothecosis on the
right fourth finger of a female patient 59 years old with no underlying
disease. Administration of itraconazole 400 mg/day for 6 weeks failed to
produce an adequate clinical response. Treatment was then changed to
fluconazole 200 mg/day, with regression of the lesion.
3-PROTOTECOSE
Clin Microbiol Rev. 2007 Apr;20(2):230-42. Lass-Flörl C, Mayr A.
Human protothecosis.
Abstract – human protothecosis is a rare infection caused by members of
the genus Prototheca. Prototheca species are generally considered to be
achlorophyllic algae and are ubiquitous in nature. The occurrence of
protothecosis can be local or disseminated and acute or chronic, with
the latter being more common. Diseases have been classified as (i)
cutaneous lesions, (ii) olecranon bursitis, or (iii) disseminated or systemic
manifestations. Infections can occur in both immunocompetent and
immunosuppressed patients, although more severe and disseminated
infections tend to occur in immunocompromised individuals. Prototheca
wickerhamii and Prototheca zopfii have been associated with human
disease. Usually, treatment involves medical and surgical approaches;
treatment failure is not uncommon. Antifungals such as ketoconazole,
itraconazole, fluconazole, and amphotericin B are the most commonly used
drugs to date. Among them, amphotericin B displays the best activity
against Prototheca spp. Diagnosis is largely made upon detection of
characteristic structures observed on histopathologic examination of tissue.
4-AMEBÍASE
Arch Dermatol. 2008 Oct;144(10):1369-72.
Cutaneous amebiasis in pediatrics.
Magaña ML, Fernández-Díez J, Magaña M.
Abstract – BACKGROUND: cutaneous amebiasis (CA), which is
still a health problem in developing countries, is important to
diagnose based on its clinical and histopathologic features.
OBSERVATIONS: Retrospective medical record review of 26
patients with CA (22 adults and 4 children) treated from 1955 to
2005 was performed. CONCLUSIONS: Cutaneous amebiasis
always presents with painful ulcers. The ulcers are laden with
amebae, which are relatively easy to see microscopically with
routine stains. Erythrophagocytosis is an unequivocal sign of
CA. Amebae reach the skin via 2 mechanisms: direct and indirect.
Amebae are able to reach the skin if there is a laceration (port of
entry) and if conditions in the patient are favorable. Amebae are
able to destroy tissues by means of their physical activity,
phagocytosis, enzymes, secretagogues, and other molecules.
4-AMEBÍASE
Am J Dermatopathol. 2004 Aug;26(4):280-4.
Histopathology of cutaneous amebiasis.
Magaña M, Magaña ML, Alcántara A, Pérez-Martín MA.
Abstract – cutaneous amebiasis (CA) is the manifestation in the
skin and underlying soft tissues of the pathogenic properties of
Entamoeba histolytica, which may be the only expression of the
infection or may be associated with disease in other organs. So
far, there have been only isolated case reports on this disease.
We herein report the histopathologic findings on a series of seven
cases, six adults and one child, of CA. The most common findings
include ulcers, areas of necrosis, mixed inflammatory
infiltrates, and the presence of trophozoites, the invasive form
of the parasite. CA is a very rare and severe disease, it is
progressive and destructive; erythrophagocytosis, a microscopic
sign of pathogenicity, is always seen in CA.
acantamoeba
5-OCRONOSE
ochronosis is the syndrome caused by the accumulation of
homogentisic acid in connective tissues
the phenomenon was first described by Rudolf Virchow in 1865
was named after the yellowish (ocher-like) discoloration of the tissue
seen on microscopic examination. However, macroscopically the
affected tissues appear bluish grey because of a light scattering
phenomenon known as the Tyndall effect
is most often associated with alkaptonuria but can occur from
exogenous administration of phenol complexes like hydroquinone
alkaptonuria is often asymptomatic, but the sclera of the eyes may
be pigmented (often only at a later age), and the skin may be darkened
in sun-exposed areas and around sweat glands
sweat may be coloured brown
urine may turn brown if collected and left exposed to open air,
especially when left standing for a period of time
kidney stones and stone formation in the prostate are common and
may occur in more than a quarter of cases
5-OCRONOSE
Br J Neurosurg. 2008 Dec;22(6):805-7.
Alkaptonuria presenting with ochronotic
spondyloarthropathy.
Al-Mahfoudh R, Clark S, Buxton N.
Abstract – alkaptonuria is a rare autosomal recessive
metabolic disease that leads to the deposition of
homogentisic acid. Ochronotic arthropathy is the articular
manifestation of alkaptonuria with the most common clinical
feature being severe spondyloarthropathy. We present the
case of a 58-year-old woman with back pain. Radiographs
and magnetic resonance imaging (MRI) revealed
characteristic features of ochronotic spondyloarthropathy.
The literature regarding management of alkaptonuria is
reviewed.
5-OCRONOSE
Arch Dermatol. 2010 Sep;146(9):1021-5. Dermoscopic and reflectance confocal
microscopic features of exogenous ochronosis. Gil I, Segura S, Martínez-Escala E
Abstract – exogenous ochronosis presents as an acquired asymptomatic
hyperpigmentation on photoexposed areas, predominantly over bony prominences,
and is caused by the topical application of several skin-lightening agents.
OBSERVATIONS: We describe a 63-year-old Hispanic woman who developed
exogenous ochronosis lesions on her face after using topical bleaching creams
containing hydroquinone, 2% to 3%, and oxybenzone, 2%, for several years.
Dermoscopy revealed irregular brown-gray globular, annular, and arciform
structures that corresponded to focal deposition of ochronotic pigment on the dermis.
These deposits correlated with multiple banana-shaped nonrefractile structures seen
using reflectance confocal microscopy. Histopathologic sections revealed the deposition
of a banana-shaped, yellow to brown material in the papillary and middle dermis.
Ultrastructural examination revealed an amorphous electron-dense material mostly
located in the core of elastic fibers and also in smaller amounts in the interstitium with
prominent degenerative changes in the elastic fibers. A good correlation was observed
between the results of both noninvasive techniques and the diagnostic histologic
features of this condition. CONCLUSIONS: We characterized by means of dermoscopy,
reflectance confocal microscopy, and electronic microscopy a case of exogenous
ochronosis. To our knowledge, this is the first description of reflectance confocal
microscopic findings in this condition. Dermoscopy and reflectance confocal microscopy
are proved to be useful noninvasive techniques for the diagnosis of this pigmentary
disorder.
OCRONOSE
6-acantoma de células claras
achados histopatológicos
 acantoma de células claras de Degos
1. acantose com proliferação bem demarcada de células claras em
relação ao epiderma normal
2. poupa o epitélio anexial
3. pode haver fusão dos cones interpapilares; predominantemente
endofítico
4. pode haver erosão e crosta
5. neutrófilos infiltrando o epiderma; pode haver espongiose
6. as células claras são PAS positivas e diastáse sensíveis
aspectos clínicos
 MMII de paciente de meia idade ou velhos; varizes
 lesão eritematosa bem demarcada com colarette descamativo
 associação com psoríase (reacional, inflamatória)
R. Degos, J. Delort, J. Civatte, A. Poires Baptista: Tumeur épidermique d'aspect particulier:
acanthome à cellules claires. Annales de dermat.et syphilig., Paris, 1962, 9: 361-371
6-ACANTOMA DE CÉLULAS CLARAS
http://dermoscopymadesimple.blogspot.com/2010/09/clear-
cell-acanthoma.html
http://www.dermoscopyatlas.com/index.cfm
 .
7-COMPLEXO DE CARNEY
Carney complex (also known as "LAMB syndrome," and "NAME syndrome") is an
autosomal dominant condition comprising myxomas of the heart and skin,
hyperpigmentation of the skin (lentiginosis), and endocrine overactivity (Carney et al.,
1985; McCarthy et al., 1986)
LAMB: lentigines, atrial myxomas, mucocutaneous myxomas, and blue nevi
NAME: nevi, atrial myxoma, myxoid neurofibroma, and ephelides
it must be differentiated from Carney triad – coexistence of several neoplasms,
including: gastric epithelioid leiomyosarcoma, pulmonary chondroma, and extra-adrenal
paraganglioma
approximately 7% of all cardiac myxomas are associated with Carney complex
the majority of cases are caused by mutations in the PRKAR1-α gene on
chromosome 17q24, which has been suggested to function as a tumor-suppressor
gene
the spotty skin pigmentation and lentigines occur most commonly on the face,
especially on the lips, eyelids, conjunctiva, and oral mucosa (McCarthy et al., 1986)
cardiac myxomas may lead to embolic strokes and heart failure (Reynen, 1995)
and may present with fever, joint pain, shortness of breath, diastolic rumble, and tumor
plop; myxomas may also occur in the skin and breast
endocrine tumors may manifest as disorders such as Cushing syndrome
http://emedicine.medscape.com/article/160000-overview
7-COMPLEXO DE CARNEY
8-DERMATOPATIA FIBROSANTE NEFROGÊNICA
Nephrogenic systemic fibrosis (NSF) or nephrogenic fibrosing dermopathy is a rare and
serious syndrome that involves fibrosis of skin, joints, eyes, and internal organs. Its cause is not
fully understood. However, there is much evidence to suggest that it is associated with exposure to
gadolinium (which is frequently used as a contrast agent for MRIs) in patients with severe kidney
failure
epidemiological studies suggest that the incidence of NSF is unrelated to gender, race, or age
and it is not thought to have a genetic basis.
patients develop large areas of hardened skin with fibrotic nodules and plaques. NSF may
also cause joint contractures resulting in joint pain and limitation in range of motion. In its most
severe form, NSF may cause severe systemic fibrosis affecting internal organs including the lungs,
heart and liver
at the microscopic level, NSF resembles scleromyxedema – proliferation of dermal fibroblasts
and dendritic cells, thickened collagen bundles, increased elastic fibers, and deposits of mucin;
recent case reports have described the presence of sclerotic bodies (also known as
elastocollagenous balls) in skin biopsies from NSF patients. While not universally present, this
finding is believed to be unique to NSF
most patients with NSF have undergone hemodialysis for renal failure, some have never
undergone dialysis and others have received only peritoneal dialysis. Many patients have taken
immunosuppressive medications and have other diseases, such as hepatitis C. Four of the seven
gadolinium contrast agents approved by the FDA have been principally implicated in NSF, including
Omniscan, Multihance, Magnevist, and OptiMARK.
the first cases of NSF were identified in 1997, but NSF was first described as an independent
disease entity in 2000 – Cowper SE, Robin HS, Steinberg SM, Su LD, Gupta S, LeBoit PE (2000).
"Scleromyxoedema-like cutaneous diseases in renal-dialysis patients". Lancet 356 (9234):
1000–1
8-DERMATOPATIA FIBROSANTE NEFROGÊNICA
Am Fam Physician. 2009 Oct 1;80(7):711-4. Schlaudecker JD, Bernheisel CR.
Gadolinium-associated nephrogenic systemic fibrosis.
Abstract – Nephrogenic systemic fibrosis is a progressive, potentially fatal multiorgan
system fibrosing disease related to exposure of patients with renal failure to the
gadolinium-based contrast agents used in magnetic resonance imaging. Because of
this relationship between nephrogenic systemic fibrosis and gadolinium-based contrast
agents, the U.S. FDA currently warns against using gadolinium-based contrast agents in
patients with a glomerular filtration rate less than 30 mL per minute per 1.73 m2, or any
acute renal insufficiency related to the hepatorenal syndrome or perioperative liver
transplantation. There have been reports of nephrogenic systemic fibrosis developing in
patients not exposed to gadolinium-based contrast agents, but most patients have the
triad of gadolinium exposure through contrast-enhanced magnetic resonance imaging,
renal failure, and a proinflammatory state, such as recent surgery, endovascular injury,
or sepsis. Development of nephrogenic systemic fibrosis among patients with severe
renal insufficiency following exposure to gadolinium-based contrast agents is
approximately 4 percent, and mortality can approach 31 percent. The mechanism for
nephrogenic systemic fibrosis is unclear, and current treatments are disappointing.
Prevention with hemodialysis immediately following gadolinium-based contrast agents
has been recommended, but no studies have shown this to be effective. Because of the
large number of patients with clinically silent renal impairment and the serious
consequences of nephrogenic systemic fibrosis related to gadolinium exposure,
physicians should use alternative imaging modalities for patients who are at risk.
8-DERMATOPATIA FIBROSANTE NEFROGÊNICA
J Am Acad Dermatol. 2010 Sep;63(3):389-99.
Revisiting nephrogenic systemic fibrosis in 6 kidney transplant recipients: a single-
center experience.
Lemy AA, del Marmol V, Kolivras A, High WA, Matos C, Laporte M, Nortier JL.
Abstract – nephrogenic systemic fibrosis (NSF) is a fibrotic disorder occurring in patients with
renal dysfunction. Exposure to gadolinium (Gd)-based contrast agents (GBCAs) during
renal impairment is associated with development of NSF. METHODS: A cross-referenced
search of kidney transplantation and radiology databases at a single institution revealed the
prevalence of NSF in the transplant population. Clinical records and skin biopsy specimens
from 6 patients with kidney transplant given a diagnosis of NSF were reviewed to identify
contributing factors. RESULTS: Between January 1999 and December 2006, NSF was
diagnosed in 6 of 705 patients with kidney transplant (0.9%). Renal function was impaired
in all patients. Of 33 patients with kidney transplant exposed to GBCAs, 5 (15.2%)
developed NSF. Disease onset ranged from 7 days to 11 months after exposure to GBCAs.
All 5 patients exposed to GBCAs who developed NSF were also treated with a beta-blocker
and clinical improvement was observed with discontinuation. The sixth case NSF appeared
unrelated to Gd, without a known exposure, and testing of tissue via mass spectrometry
revealed no Gd. Symptoms of NSF in this patient disappeared after administration of
darbepoetin was switched from subcutaneous to intravenous injection. One patient with NSF
who manifested the highest Gd level in tissue died 22 months after disease onset.
LIMITATIONS: The study represents the retrospective experience of only a single center.
CONCLUSIONS: NSF can develop in kidney transplant recipients with altered graft function.
In these patients, exposure to GBCAs appears associated with development of NSF. The role
of beta-blockers in the course of the disease merits further investigation
9-MF variantes – Woringer-Kolopp
Semin Cutan Med Surg. 2000 Jun;19(2):91-9.
Mycosis fungoides: classic disease and variant presentations.
Howard MS, Smoller BR.
Abstract – mycosis fungoides is a peripheral non-Hodgkin's T-cell
neoplastic process, representing the most common type of primary
cutaneous malignant lymphoma. Neoplastic lesions classically show
skin predilection and characteristic clinical and histologic features in
patch, plaque, and tumor stages. In addition, several clinicopathologic
variants of mycosis fungoides have been delineated, including
poikiloderma atrophicans vasculare (parapsoriasis variegata),
Sézary syndrome, granulomatous mycosis fungoides,
hypopigmented mycosis fungoides, folliculocentric mycosis
fungoides, syringotropic mycosis fungoides, and Woringer Kolopp
disease. We will review the salient features of patch, plaque, and tumor
stage mycosis fungoides in this article and follow with a discussion of
these variant clinicopathologic presentations and of therapeutic
modalities.
9-MF variantes – Woringer-Kolopp
Am J Dermatopathol. 2005 Feb;27(1):68-85. Steffen C
Ketron-Goodman disease, Woringer-Kolopp disease, and pagetoid
reticulosis.
Abstract – in this historical review I will synopsize the original articles by
Lloyd W. Ketron and M.H. Goodman who described Ketron-Goodman
disease, by Frederic Woringer and Pierre Kolopp who described
Woringer-Kolopp disease, and by Otto Braun-Falco and colleagues
who described pagetoid reticulosis. In their publications, each of these
authors reported on one patient. I will review the clinical picture of the
three patients, their histopathology, and the pathogenesis of each
disease as suggested by the above authors. Then the views of others
that have written on the subject recently will be reviewed particularly as
to their conception of the diseases. Publications that describe the
histopathology of the patch (early) stage of mycosis fungoides will be
redacted to compare it to the histopathology of Ketron-Goodman
disease, Woringer-Kolopp disease, and pagetoid reticulosis. I will discuss
whether any or all of them are diseases sui generis, whether they are
one, two, or three entities, or whether any or all are but forms of mycosis
fungoides.
Segundo caso observado por Frédéric Woringer em 1958 – Kollop
(urologista) em 1938 mandou uma biópsia do antebraço E de um rapaz de
13 anos pensando em tuberculose cutânea.
Woringer-Kollop – CD3+
Woringer-Kollop – CD3+
Ketron-Goodman
Ketron-Goodman
9-Granulomatous slack skin
Dermatology. 1998;196(4):382-91. van Haselen CW
Granulomatous slack skin. Report of three patients with an updated review of the literature.
Abstract - PURPOSE: Granulomatous slack skin (GSS) is a rare cutaneous disorder characterized
clinically by the evolution of circumscribed erythematous lax skin masses, especially in the body
folds, and histologically by a granulomatous T-cell infiltrate and loss of elastic fibers. GSS is often
associated with preceding or subsequent lymphoproliferative malignancies, especially mycosis
fungoides (MF) and Hodgkin's disease (HD). No effective treatment is known yet. Whether this entity is a
benign disorder, a peculiar host reaction to a malignant lymphoma, a precursor of malignant lymphoma or
an indolent cutaneous T-cell lymphoma (CTCL) in itself is still a matter of debate. PATIENTS AND
METHODS: The results of the patients with GSS from the Netherlands are compared with the cases
reported in the world literature. RESULTS: A female patient had had GSS for 8 years without developing
a secondary malignancy. In a second female patient with a histologically confirmed diagnosis of MF, GSS
developed 18 years later in the axillary and inguinal folds which had previously been affected by plaque-
stage MF lesions. A third male patient with a 6-year history of erythematosquamous skin disease
diagnosed as CTCL developed GSS. Moreover, granuloma formation was also found in a facial basal cell
carcinoma, in a cervical lymph node and the spleen. Clonal rearrangements of the T-cell receptor beta
genes were found in the 2 female patients; the male patient could not be tested. CONCLUSION: GSS is a
rare clinicopathological entity. Only 34 patients have been described so far. The development of GSS
within plaque MF lesions has not been reported before. Our third case developed very extensive skin
lesions and showed a strong propensity to develop granulomas as compared to cases reported before.
The presence of a clonal T-cell population was demonstrated in all cases tested. Our cases support the
idea that GSS is a very rare and rather indolent type of CTCL. Apparently, the disease is associated with
a peculiar immune response, characterized by granuloma formation and disappearance of elastic
fibers resulting in the lax skin. The relationship between GSS and other preexisting or subsequent
lymphoproliferative diseases (diagnosed in approximately 50% of the cases) warrants a life-long follow-
up.
9-Granulomatous slack skin
Acta Derm Venereol. 2001 Jan-Feb;81(1):42-4. Topar G
Granulomatous slack skin: a distinct disorder or a variant of
mycosis fungoides?
About 75% of cutaneous lymphomas belong to the group of
T-cell lymphomas . Mycosis fungoides is the most common
entity in this group. Granulomatous slack skin is a rare form of
cutaneous T-cell lymphoma closely related to mycosis fungoides.
We present here a patient with areas of lax skin for
several years who developed a generalized erythroderma with
associated immunoactivation and a deterioration in his general
condition. This report discusses clinically and histologically the
differential diagnoses, namely granulomatous slack skin and
granulomatous mycosis fungoides, and suggests that these
2 disorders are only variants in the broad spectrum of a single
disease.
An. Bras. Dermatol. vol.82 no.5 Rio de Janeiro Sept./Oct. 2007
9-MUCINOSE FOLICULAR
An. Bras. Dermatol. vol.77 no.6 Rio de Janeiro Nov./Dec. 2002
Mucinose Folicular: revisão da literatura e relato de um caso
Em 1957, Pinkus3 descreveu uma nova entidade dermatológica, sob a
denominação de alopecia mucinosa, apresentando a observação de seis
pacientes portadores da nova enfermidade. Porém, como a alopecia só é
clinicamente evidente quando se encontram acometidas áreas cutâneas com
pêlo terminal, parece ser mais adequada a designação MuF, proposta em
1959 por Jablonska Chorzelski e Lancucky e adotada pela maioria dos autores
que se ocuparam do assunto. A MuF tem sido observada em todas as raças,
em todas as idades e igualmente em ambos os sexos. Sua causa permanece
desconhecida mas a tendência atual é considerá-la um padrão de reação do
epitélio folicular a diversas noxas
A classificação da MuF foi ampliada recentemente para incluir três formas:
1.primária, de curta evolução
2. primária, de curso prolongado
3. secundária, associada a outros processos
A terceira variedade, que habitualmente incide entre os 40 e os 70 anos de
idade, apresenta-se como múltiplas placas generalizadas, infiltradas,
associadas a linfomas de células T, especialmente micose fungóide – 11 a
32%
9-MUCINOSE FOLICULAR
aspectos histopatológicos
1. infiltrado folicular, perifolicular ou perivascular,
predominantemente linfocitário, mas pode haver
eosinófilos ou plasmócitos
2. na micose fungoide, geralmente não há infiltrado
linfocitário folicular relevante
3. acomete vários folículos ao mesmo tempo,
especialmente na metade superior
4. mucinose intrafolicular, semelhante a edema, mas
cora para mucina (PAS+AB, ferro coloidal)
MF hipopigmentada pediátrica
10-ATLL – LINFOMA DE CÉLULAS T
Int J Dermatol. 2008 Apr;47(4):359-62. Pezeshkpoor F
Specific cutaneous manifestations in adult T-cell leukemia/lymphoma.
Abstract – BACKGROUND: Adult T-cell leukemia/lymphoma (ATLL) is an
aggressive malignancy which may occur in individuals infected with human T-cell
lymphotropic virus type-I (HTLV-I). HTLV-I is endemic in Khorasan, with a frequency
of 2.3% in the general population. As specific cutaneous manifestations of lymphoma
may occur in a significant number of patients, we studied these manifestations in
ATLL patients admitted to the Hematology and Dermatology Departments of Ghaem
Hospital, Mashhad, Iran, during 1995-2004. METHODS: In this descriptive study,
demographic and clinical information was obtained from 23 patients suffering from
ATLL with specific cutaneous lesions (atypical lymphocytes on histopathology of
cutaneous lesions), and was analyzed statistically. RESULTS: Of the 23 patients, 11
were male and 12 were female. The mean age was 48.17 +/- 14.1 years. The birth
place in over 85% of cases was the north of Khorasan. The most common type of
specific skin lesion was a maculopapular eruption (11 cases; 47.8%); papular
lesions were seen in four cases (17.4%). Other lesions included plaques,
ichthyosis-like lesions, erythroderma, tumors, papules, and nodular lesions. In
most patients (56.5%), the skin lesions were generalized. CONCLUSION: The most
common type of specific skin lesion in ATLL was maculopapular eruption, especially
with a generalized distribution. Other types of specific skin lesion, in order of
frequency, were papules, plaques, ichthyosis-like skin lesions, nodules, tumors, and
erythroderma.
10-ATLL – LINFOMA DE CÉLULAS T
Int J Dermatol. 2010 Oct;49(10):1099-110.
Cutaneous manifestations associated with HTLV-1 infection.
Bittencourt AL. Laboratory of Pathology, Complexo Hospitalar Universitário
Prof. Edgard Santos, Federal University of Bahia, Salvador, Bahia, Brazil.
achilea@uol.com.br
Abstract - Skin lesions are frequent in HTLV-1 infection and may constitute an
alert for the diagnosis of this condition. The most severe skin diseases related
to this virus are adult T-cell leukemia/lymphoma (ATLL), an aggressive form
of leukemia/lymphoma that fails to respond to chemotherapy, and infective
dermatitis associated with HTLV-1 (IDH), a severe and recurrent form of
eczema occurring in childhood. ATLL affects the skin in 43-72% of cases. In
this review, the clinical, histopathological and immunohistochemical aspects of
ATLL and IDH will be discussed, as well as the differential diagnoses, giving
particular focus to the primary cutaneous ATLL. IDH may progress to HTLV-1-
associated myelopathy/tropical spastic paraparesis (HAM/TSP) and to ATLL.
Adult onset IDH and reactional and inflammatory dermatoses found in carriers
and also in patients with HAM/TSP will be considered. Other dermatological
diseases that occur more frequently in HTLV-1-infected individuals such as
xerosis, acquired ichthyosis, seborrheic dermatitis and infectious and parasitic
dermatoses will also be discussed.
Human T-cell leukemia virus type I (HTLV-I) infection and the onset of adult T-cell
leukemia (ATL); Matsuoka, Masao; Retrovirology  Vol.  2  Issue  1, 2005
flower cells
flower cells
11-BIRT-HOGG-DUBÉ
Birt–Hogg–Dubé syndrome (BHD; 1977) is a human genetic disorder that involves
susceptibility to renal cancer, renal and pulmonary cysts, and tumors of the hair
follicles. The disorder has been reported in more than 100 families worldwide, and it is
inherited in an autosomal dominant pattern. Mutations in the FLCN gene, located on the
short arm of chromosome 17 (17p11.2), that makes a protein called folliculin.
Clinical triad
1. The cutaneous manifestations of BHD were originally described as
fibrofolliculomas, trichodiscomas, and acrochordons. The dermatologic
diagnosis of BHD can be made in an individual five or more skin lesions, at least one
of which must be confirmed as a fibrofolliculoma by biopsy.
2. Most individuals (89%) with BHD are found to have multiple cysts in both lungs, and
24% have had one or more episodes of pneumothorax. The cysts can be detected
by chest CT scan.
3. Renal tumors can manifest as multiple types of renal cell carcinoma, but certain
pathological subtypes (including chromophobe, oncocytoma, and oncocytic hybrid
tumors) are more commonly seen in BHD.
Other, less commonly associated features include a large connective-tissue nevus,
parathyroid adenomas, flecked chorioretinopathy, bullous emphysema, lipomas,
angiolipomas, parotid oncocytomas, multiple oral mucosal papules, neural tissue tumors,
and multiple facial angiofibromas.
http://emedicine.medscape.com/article/1060579-overview
A- TRICODISCOMA B- FIBROFOLICULOMA
12-BROOKE-SPIEGLER
Brooke-Spiegler syndrome is an uncommon disease with a predisposition to
develop cutaneous adnexal neoplasms such as cylindromas, trichoepitheliomas,
spiradenomas, trichoblastomas, basal-cell carcinomas, follicular cysts,
organoid nevi, and malignant transformation of pre-existing tumors in the affected
individuals
Brooke-Spiegler syndrome is inherited in autosomal-dominant fashion, although
expression and penetrance are variable. Lesions usually begin to appear in the
second or third decades and gradually increase in number and size throughout
adult life
women are affected more frequently than are men
mutations in the CYLD tumor-suppressor gene, located at 16q12-q13, have
been implicated in the phenotype diversity
Cylindromas, trichoepitheliomas, and spiradenomas, which are the most
commonly observed tumors, are typically located on the head and neck
multiple trichoepitheliomas may be seen also in two other rare syndromes:
Rombo syndrome (vermicular atrophoderma, milia, hypotrichosis, basal-cell
carcinomas, trichoepitheliomas, and peripheral vasodilatation with cyanosis) and
Bazex syndrome (follicular atrophoderma, hypotrichosis, occasional
trichoepitheliomas, basal-cell carcinomas, and localized or generalized
hypohidrosis)
http://dermatology.cdlib.org/131/cases/NYUcases/111505_7.html
12-BROOKE-SPIEGLER
Am J Dermatopathol. 2011 Mar 8. Michal M
Multiple (Familial) Trichoepitheliomas: A Clinicopathological and Molecular Biological Study,
Including CYLD and PTCH Gene Analysis, of a Series of 16 Patients.
Abstract – Multiple familial trichoepitheliomas (MFT) constitute an autosomally inherited syndrome possibly
related to Brooke-Spiegler syndrome (BSS). Although some early studies suggested a role for the PTCH gene
on chromosome 9q22.3 in the etiopathogenesis of MFT, recent studies of occasional patients with the MFT
clinical phenotype identified mutations in the CYLD gene on chromosome 16q12-q13, a gene responsible for
BSS. A systematic investigation of PTCH and CYLD mutations in patients with MFT has never been performed.
Our main objective was to collect a reasonably large series of patients with MFT to (1) study the
clinicopathological spectrum of the disease, (2) determine whether the PTCH gene is implicated in the
pathogenesis of MFT, and if so (3) determine the relative frequency of CYLD and PTCH mutations, (4) establish
if there may be any possible genotype-phenotype correlations, and (5) study the spectrum of somatic mutations.
Clinical analysis including family histories, histopathological investigations, and molecular genetic studies were
performed. There were 9 female and 7 male patients ranging in age from 11 to 63 years. They presented with
multiple, small, discrete and sometimes confluent, skin-colored to pink, asymptomatic nodules preferentially
located on the face, being especially prominent and confluent in the nasolabial folds and inner aspects of the
eyebrows. A total of 66 conventional trichoepitheliomas (TEs) were studied microscopically. Aside from typical
features of TE, some also exhibited variant morphological patterns including areas reminiscent of other benign
adnexal neoplasms and melanocytic hyperplasia. In none of the 9 patients tested was a germline mutation of the
PTCH gene identified. Germline CYLD mutations were detected in 6 of 13 patients tested (identical in 2 unrelated
patients) including 2 novel mutations, whereas the remaining 7 individuals showed wild-type alleles. Two patients
with germline wild-type CYLD showed, however, a somatic mutation in the gene (1 duplication, 1 substitution
mutation). Neither CYLD nor PTCH germline mutations were found in the 5 patients in whom both genes were
analyzed. MFT seems to be a phenotypic variant of BSS. The PTCH gene is rarely, if ever, involved in the
pathogenesis of MFT. Absence of a germline mutation of the CYLD gene in cases harboring a somatic mutation
may be explained by large deletions in the gene or by mutation in intronic sequences or in the promoter region.
Considering our 5 patients with no mutation in either gene, the final possibility is that another, as yet undescribed
gene (neither CYLD nor PTCH) is implicated in the pathogenesis of some patients with MFT.
13-HEMANGIOMA GLOMERULOIDE – POEMS
polineuropatia, organomegalia, endocrinopatia, proteína M e alterações cutâneas
(skin changes) – Bardwick, 1980
mieloma osteoesclerótico, síndrome de Crow-Fukase e síndrome de Takatsuki
síndrome de POEMS é uma entidade clínica única definida pela presença concomitante
de doença monoclonal de plasmócitos, polineuropatia periférica e outras manifestações
paraneoplásicas tais como organomegalias, endocrinopatias, alterações cutâneas,
papiledema, lesões osteoescleróticas e sobrecarga de volume extravascular.
a sobrevida média dos pacientes com síndrome de POEMS é significativamente superior
àquela esperada para os portadores de mieloma múltiplo (165 e 38 meses,
respectivamente), independente do número de manifestações presentes ao diagnóstico e da
intensidade de infiltração da medula óssea por plasmócitos
 outros achados comuns relacionados à síndrome incluem lesões osteoescleróticas,
doença de Castleman, derrame pleural, ascite, edema periférico, papiledema, eritrocitose,
plaquetose e baqueteamento dos dedos. O quadro clínico peculiar e a maior sobrevida
média dos pacientes distinguem claramente a síndrome de POEMS do mieloma múltiplo.
pelo menos dois terços dos portadores de síndrome de Poems apresentam uma ou mais
das seguintes anormalidades endócrinas detectadas através da avaliação clínica ou
laboratorial: hipogonadismo, hipotiroidismo, diabetes melllitus, insuficiência adrenal ou
hipoparatiroidismo
alterações cutâneas: hiperpigmentação, hipertricose, acrocianose, pletora,
hemangiomata/telangectasia
Rev. Bras. Hematol. Hemoter. vol.29 no.1 São José do Rio Preto Jan./Mar. 2007
http://emedicine.medscape.com/article/1097031-overview
14-ESTRONGILOIDIASE
Strongyloidiasis is caused by the soil-dwelling nematode Strongyloides stercoralis. This parasite is
endemic to tropical and subtropical regions throughout the world including areas of the southeastern United
States and Appalachia and affects 100 million people worldwide. Humans are infected transcutaneously
by filariform larvae that subsequently travel via the venous system to the lungs. There they eventually enter
the pharynx and are swallowed. In the small intestine adult female worms produce eggs that hatch
rhabditiform larvae. These larvae pass in the stool to continue the free-living external life cycle or develop
into filariform larvae that can reinfect the host by penetration of the perianal skin or intestinal wall. This
latter process is termed "autoinfection" and can occur at low levels throughout the course of infection.
Strongyloidiasis has a wide range of manifestations from asymptomatic disease to disseminated
infection. Acute and chronic infection, hyperinfection syndrome, and disseminated infection are described.
Cutaneous manifestations of acute strongyloidiasis are not well characterized, but local urticarial
reaction at the site of larval entry may occur. Pulmonary and gastrointestinal symptoms have been
described, with larvae detectable in stool within three to four weeks. Chronic infection is most frequently
asymptomatic. Dermatological manifestations include chronic urticarial lesions of the buttocks and
waistline that last one to two days. Larva currens is pathognomonic of chronic Strongyloides infection
and is caused by filiariform larval migration in the skin. This manifests as intensely pruritic
serpiginous urticarial wheals that move at a rate of 5-15 cm/hour and occur mainly on the buttocks,
groin, and trunk. Biopsies have not revealed larvae. Hyperinfection occurs with accelerated
autoinfection, usually as a result of immunosuppression that leads to increased multiplication and migration
of larvae. This increased larval burden produces exacerbated gastrointestinal and pulmonary symptoms.
When larvae migrate beyond the pulmonary and intestinal systems, the term "disseminated infection" is
used. Cutaneous manifestations of disseminated disease, as seen in our patient, include progressive
petechial and purpuric eruptions on the abdomen and proximal extremities. As we have shown,
larvae can be demonstrated on routine hematoxylin-eosin stains. The larvae migrate through blood
vessel walls into the dermis causing hemorrhage and the petechial appearance of this eruption.
http://anagen.ucdavis.edu/1412/case_reports/strongyloidiasis/arch.html
15-DOENÇA DE FLEGEL
Flegel originally described hyperkeratosis lenticularis perstans (HLP) in 1958-
Flegel H. [Hyperkeratosis lenticularis perstans.]. Hautarzt. Aug 1958;9(8):363-4
occurs most commonly in mid-to-older age groups; however, reports exist of
occurring in patients as young as 13 years
all reports have been in white patients
small, red-brown, hyperkeratotic, 1-5 mm papules begin to develop
symmetrically on the lower extremities are the most frequent and characteristic
presentation of hyperkeratosis lenticularis perstans; involvement of the ear pinnae,
arms, palms, soles, and the oral mucosa has been reported, although these reports
are rare; involvement of the trunk has been reported but remains an unusual variant
removal of the scale reveals a bright red base, often with pinpoint bleeding
DD: acrokeratosis verruciformis of Hopf, porokeratosis, actinic keratosis, Stucco
keratosis, Darier disease, and Kyrle Disease
biopsy: a discrete area of hyperkeratosis occurs (with areas of parakeratosis)
overlying a thinned stratum malpighii and thinned-to-absent granular layer; irregular
acanthosis and some vascular dilatation are peripheral; a lymphoid infiltrate with
occasional histiocytes in a bandlike pattern in the papillary dermis typically is seen –
pode ter padrão de DIVa muito discreto
http://emedicine.medscape.com/article/1107012-diagnosis
16-PYOSTOMATITIS VEGETANS
Coll Antropol. 2010 Apr;34 Suppl 2:279-82.
Pyostomatitis vegetans associated with inflammatory bowel disease--
report of two cases.
Mijandrusić-Sincić B, Licul V, Gorup L, Brncić N, Glazar I, Lucin K.
Abstract – Pyostomatitis vegetans (PV) is a rare, chronic mucocutaneous
disorder associated with inflammatory bowel disease (IBD). Oral lesions of
PV are distinct and present as multiple white or yellow pustules with an
erythematous base that coalesce and undergo necrosis to form a typical
"snail tracks" appearance. Two cases of PV associated with IBD--one with
Crohn's disease (CD) and the other with ulcerative colitis (UC) are reported. In
the first case, adalimumab therapy brought the oral and gastrointestinal
manifestations to complete remission. In the second case, the remission was
achieved with systemic steroid therapy, but the disease relapsed after
therapy discontinuation. Azathioprine was added leading to sustained
remission of PV. Because of persistent active intestinal manifestation of UC, in
spite of immunosuppressive therapy, infliximab was introduced. With the
therapy remission of intestinal manifestation of UC was achieved as well. Our
cases confirm previously reported good experience with immunomodulators
and biologics in the treatment of PV. But, before using them we have to exclude
an infectious etiology of oral lesions.
16-PYOSTOMATITIS VEGETANS
Am J Dermatopathol. 2011 Feb;33(1):e1-6.
All that glitters is not pemphigus: Pyodermatitis-pyostomatitis vegetans
misdiagnosed as IgA pemphigus for 8 years.
Abellaneda C, Mascaró JM Jr, Vázquez MG, Pablo IM, Iranzo P.
Abstract
Pyodermatitis-pyostomatitis vegetans is a rare mucocutaneous dermatosis
often associated with gastrointestinal disorders, especially with inflammatory
bowel disease. It is clinically characterized by erythematous lesions with
multiple pustules and erosions affecting the mucosal surfaces. Cutaneous
lesions are characterized by exudative and vegetating plaques affecting
frequently the axillae and groins. The clinical diagnosis is supported by
histologic findings, whereas immunofluorescence studies (negative) are
useful to rule out other entities such as pemphigus. Herein we report the
case of a young man who was misdiagnosed as having IgA pemphigus for 8
years due to positive immunofluorescence findings. The clue for the final
diagnosis was the diagnosis of a concomitant ulcerative colitis, which
prompted us to reconsider his cutaneous disease.
16-PYOSTOMATITIS VEGETANS
J Am Acad Dermatol. 1994 Aug;31(2 Pt 2):336-41.
Pyodermatitis-pyostomatitis vegetans: a specific marker for inflammatory
bowel disease.
Storwick GS, Prihoda MB, Fulton RJ, Wood WS.
Abstract
In pyodermatitis-pyostomatitis vegetans annular pustular cutaneous lesions
may precede, accompany, or follow the usually extensive vegetating oral
disease. Sometimes only cutaneous or only oral lesions occur and previously
have been described as separate entities. Clinical, histopathologic, and
immunopathologic evidence clearly indicates this is one disease and
suggests that it is distinct from pemphigus vegetans. The association
between pyodermatitis-pyostomatitis vegetans and inflammatory bowel
disease, most commonly ulcerative colitis, has been amply confirmed.
Pyodermatitis-pyostomatitis vegetans should be considered a marker for
inflammatory bowel disease.
17-AMALGAMA TATTOO
Amalgam tattoos are common oral pigmented lesions that clinically present as
isolated, blue, grey, or black macules on the gingivae, the buccal and alveolar
mucosae, the palate, and/or the tongue. They are due to deposition of a mixture of
silver, tin, mercury, copper, and zinc, which are components of an amalgam
filling, into the oral soft tissues. The deposition occurs after a number of different dental
procedures that include diffusion through soft tissues from root-end fillings, accidental
deposition of fine metallic particles into the gingiva by high-speed drills, accidental
abrasion of the mucosa by high-speed rotary instruments, or deposition of amalgam
scraps left behind during extraction.
These tattoos do not represent a health hazard since the mercury present in
amalgam is not in a free state. However, owing to its clinical appearance, amalgam
tattoos can be mistaken for a number of different conditions of concern, such as
melanoma, pigment-cell nevi, melanotic macules, melanoacanthoma, Kaposi's
sarcoma, and physiologic pigmentation. The diagnosis is more easily determined if
the lesion is in the vicinity of a large silver amalgam restoration or a gold crown. If not, a
biopsy may be performed. Histopathologic features include discrete, fine, dark
granules and irregular, solid fragments. They can be found along collagen bundles
and vessels and also are found within macrophages, mulinucleated giant cells,
fibroblasts, and endothelial cells.
Treatment for amalgam tattoos was originally limited to surgery with grafting of
mucosa or gingiva over the previous site of the tattoo. Advances in laser technology now
allow amalgam tattoos to be removed by the Q-switched ruby laser.
http://dermatology.cdlib.org/145/nyu/cases/032007_6.html
17-AMALGAMA TATTOO
Hell Stomatol Chron. 1989 Apr-Jun;33(2):113-20.
Differential diagnosis of bluish and pigmented lesions of the oral
mucosa
Tsiklakis K, Patsakas A.
Abstract – the clinical features of the most common bluish and
pigmented lesions of the oral mucosa are discussed in this paper.
Considerable attention is given to the findings from the medical and
dental history of the patient, in the methodology of the clinical
examination (inspection, palpation, digital pressure, aspiration) in the
clinical characteristics of the lesions (location, size duration,
consistency, prognosis) in the laboratory findings (radiographs and other
supplementary examinations) and in the differential diagnosis. The
bluish and pigmented lesions which are discussed include: melanoma,
Albright's syndrome, Addison's diseases, Peutz-Jeghers's
syndrome, arsening poisoning, hemangioma, hematoma, petechia
and ecchymosis, Sturge-Weber syndrome, amalgam tatoo, heavy
metal lines, mucocele and eruption cyst.
http://emedicine.medscape.com/article/1078143-media
18-SÍNDROME DE SNEDDON
Sneddon's syndrome, also known as "Idiopathic livedo reticularis with
cerebrovascular accidents“, is a form of characterized by several
symptoms, including: cerebrovascular disease, livedoid vasculitis, and
hypertension
it is named for Ian Bruce Snnedon in 1965 – Sneddon IB (April 1965).
"Cerebrovascular lesions and livedo reticularis“; Br. J. Dermatol. 77: 180–5
J Am Acad Dermatol. 2005 Jun;52(6):1009-19.
Livedo reticularis: an update.
Gibbs MB, English JC 3rd, Zirwas MJ.
Abstract
Livedo reticularis (LR) is a well-known, relatively common physical finding
consisting of macular, violaceous, connecting rings that form a netlike
pattern (Fig 1). In most cases, it is a completely benign finding related to
cold exposure. However, there are many potential causes (Table I), and
this can make the evaluation of a patient presenting with this finding very
difficult. An excellent review of the topic by Fleischer and Resnick was
published in 1990. We have endeavored to update the literature and provide
clinicians with guidance regarding the evaluation and treatment of patients
presenting with LR.
18-SÍNDROME DE SNEDDON
J Neurol. 2005 Oct;252(10):1155-66. Epub 2005 Aug 26.
The spectrum of differential diagnosis in neurological patients with livedo
reticularis and livedo racemosa. A literature review.
Kraemer M, Linden D, Berlit P.
Abstract
Livedo is a cutaneous sign of striking violaceous netlike patterned erythema of
the skin. This dermatological phenomenon is of special interest in the
differential diagnosis in neurological patients. In 1907 Ehrmann distinguished
two different patterns of livedo: the pathological livedo racemosa and the
physiological livedo reticularis. Despite important clinical differences, in the
English language literature the heading livedo reticularis is still used for all
types of livedo. A literature review about the spectrum of differential diagnosis
in patients with livedo reticularis (especially cutis marmorata and
amantadine-induced livedo reticularis) and livedo racemosa (especially
Sneddon's syndrome, Divry-van Bogaert syndrome, systemic lupus
erythematosus, antiphospholipid antibody syndrome, polyarteritis
nodosa, cholesterol embolization syndrome, livedoid vasculopathy and
haematological diseases) is provided.
19-MALACOPLAQUIA
Malakoplakia is an inflammatory condition presenting as a
plaque or a nodule that usually affects the genitourinary tract but
may rarely involve the skin. Malakoplakia was first described in
the early 1900s as yellow soft plaques that were seen on the
mucosa of the urinary bladder. Microscopically, malakoplakia is
characterized by the presence of foamy histiocytes with
distinctive basophilic inclusions, which are known as
Michaelis-Gutmann bodies
Malakoplakia is a treatable inflammatory reaction to bacteria,
mycobacteria, or fungi that may occur in immunocompromised
patients – a number of cases caused by Rhodococcus equi have
been reported in AIDS patients
also characteristic of lesions of malakoplakia are rounded intra-
or extracellular inclusions representing giant lysosomes. They
contain organisms and often calcify. These inclusions are known
as Michaelis-Gutman bodies
http://emedicine.medscape.com/article/1055606-overview
19-MALACOPLAQUIA
Arch Pathol Lab Med. 2008 Jan;132(1):113-7.
Cutaneous malakoplakia.
Kohl SK, Hans CP.
Abstract – malakoplakia is an acquired granulomatous disorder first
described by Michaelis and Gutmann in 1902. The pathogenesis of
malakoplakia is poorly understood, but it is thought to be secondary to
an acquired bacteriocidal defect in macrophages occurring mostly
in immunosuppressed patients or in the setting of autoimmune
disease. Malakoplakia has been described in numerous anatomic
locations, most commonly in the genitourinary tract. Microscopically,
malakoplakia consists predominantly of sheets of macrophages known
as von Hansemann cells with scattered targetoid intracytoplasmic
inclusions known as Michaelis-Gutmann bodies. Cutaneous
malakoplakia is a rare entity with less than 50 cases reported in the
literature. In this article, we review cutaneous malakoplakia including the
clinical, gross, and microscopic features as well as the treatment and
prognosis of 40 cases of cutaneous malakoplakia identified in the
literature.
Michaelis-Gutmann bodies –von Kossa
Michaelis-Gutmann bodies are positive for calcium (von Kossa stain,
original magnification, x 200).
20-GRANULOMA 2ND PREENCHEDOR DÉRMICO
Plast Reconstr Surg. 2008 May;121(5):1811-20. Salles AG, Ferreira MC.
Complications after polymethylmethacrylate injections: report of 32 cases.
Abstract – During the past 15 years, polymethylmethacrylate has been used as a
synthetic permanent filler for soft-tissue augmentation.
METHODS: This article reports 32 cases of complications seen at Hospital das Clínicas,
Faculty of Medicine, University of São Paulo, for procedures performed elsewhere.
RESULTS: The average age of the patients was 43.6 years (range, 22 to 70 years).
Twenty-five patients were women. Sixteen injection procedures were performed by
certified plastic surgeons, nine by dermatologists, two by urologists, and one by a
nonphysician. Complications were classified into five groups according to main
presentation as follows: tissue necrosis (five cases), an acute complication that can be
related to technical mistakes but that can also be dependent on patient factors or caused
by local infection; granuloma (10 cases), which usually presents as a subacute
complication 6 to 12 months after the procedure; chronic inflammatory reactions (10
cases), which usually occur years later and can be related to a triggering event, such as
another operation or infection in the area that was injected (these reactions are
immunogenic in origin and may have cyclic periods of activation and remission); chronic
inflammatory reaction in the lips (six cases), which may be present with severe
symptoms, especially with lymphedema, because of mobility of the lip; and infections
(one case), which are rare but possible complications after filling procedures.
CONCLUSIONS: Polymethylmethacrylate filler complications, despite being rare, are
often permanent and difficult or even impossible to treat. Safety guidelines should be
observed when considering use of polymethylmethacrylate for augmentation.
20-GRANULOMA 2ND PREENCHEDOR DÉRMICO
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http://www.ispub.com/journal/the_internet_journal_of_dermatology/volume_3_number_1_11/
article/dermalive_granuloma_a_lesion_with_distinctive_histological_features.html
http://emedicine.medscape.com/article/1125066-overview