Fitzpatrick’s

Dermatology
Ninth Edition

EDITORS
SEWON KANG, MD, MPH
MASAYUKI AMAGAI, MD, PhD
ANNA L. BRUCKNER, MD, MSCS
ALEXANDER H. ENK, MD
DAVID J. MARGOLIS, MD, PhD
AMY J. McMICHAEL, MD
JEFFREY S. ORRINGER, MD

VOLUME I

New York Chicago San Francisco Athens London Madrid Mexico City
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Kang_DIGM-FM_Vol-I.indd 3 08/12/18 5:31 pm


Dermal Connective PART
Tissue Disorders
Chapter 70 :: Anetoderma and Other Atrophic
Disorders of the Skin
:: Catherine Maari & Julie Powell
ANETODERMA
AT-A-GLANCE
■ Circumscribed 1- to 2-cm areas of flaccid skin that
may be elevated, macular, or depressed.
■ Often circumscribed sac-like protrusions.
■ Primary or secondary to a preceding dermatosis in
the same location.
■ Association with antiphospholipid syndrome.
■ Pathology consists of loss of elastic tissue in the
dermis.
EPIDEMIOLOGY
The lesions in anetoderma usually occur in young
adults between the ages of 15 and 30 years and more
often in women than men. Anetoderma is rare, and
the incidence is unknown. Several hundred cases have
been reported.1-4
PATHOGENESIS
The pathogenesis of anetoderma is unknown. The key
defect is damage to the dermal elastic fibers. Aneto-
derma may be considered to be unusual scars, because
scars also have decreased elastic tissue. The loss of
dermal elastin could be the result of an impaired turn-
over of elastin caused by either increased destruction
or decreased synthesis of elastic fibers.4,5 Recently, a
decrease in fibulin protein expression was described,
Kang_CH070_p1193-1202.indd 1193
11
suggesting that not only elastolytic overactivity,
but also defective elastic fiber reassembly, may be
involved.6 Immunologic mechanisms may also play a
role, as it can be associated with various systemic con-
ditions, primarily antiphospholipid antibodies.
CLINICAL FEATURES
All types of anetoderma are characterized by a cir-
cumscribed loss of normal skin elasticity. The charac-
teristic lesions are flaccid circumscribed areas of slack
skin with the impression of loss of dermal substance
forming depressions, wrinkling, or sac-like protru-
sions (Fig. 70-1). These atrophic, skin-colored, or blue-
white lesions are 5 to 30 mm in diameter. The number
varies from a few to hundreds. The skin surface can
be wrinkled, thinned, and often depigmented, and a
central depression may be seen. Coalescence of smaller
lesions can give rise to larger herniations. The examin-
ing finger sinks without resistance into a distinct pit
with sharp borders as if into a hernia ring (buttonhole
sign). The protrusion reappears as soon as the pressure
from the finger is removed.4
The most common sites for these asymptomatic
lesions are the chest, back, neck, and upper extremi-
ties. They usually develop in young adults, and new
lesions often continue to form for many years as the
older lesions fail to resolve.
Primary anetoderma occurs when there is no under-
lying associated skin disease (ie, it arises on clinically
normal skin). It is historically subdivided into 2 types:
(a) those with preceding inflammatory lesions, mainly
erythema (the Jadassohn-Pellizzari type), and (b)
those without preceding inflammatory lesions (the
Schweninger-Buzzi type). This classification is only of
historical interest, because the 2 types of lesions can
03/12/18 9:26 am
11
Part 11
::
A ■ Cutaneous B-cell lymphoma
Dermal Connective Tissue Disorders
Figure 70-1 Anetoderma. Primary anetoderma. A, Multi-
ple, sharply defined, depressed lesions that look punched
out in the supraclavicular region. B, Soft, sac-like protru-
sions on the back. When depressed, there is the button-
hole phenomenon. This is the same patient as in A.
coexist in the same patient; the prognosis and the his-
topathology are also the same.4
True secondary anetoderma implies that the charac-
teristic atrophic lesion has appeared in the exact same
site as a previous specific pathology; the most common
causes are probably acne and varicella. Numerous and
heterogeneous dermatoses have been associated with
secondary anetoderma (Table 70-1), namely infec-
tious (syphilis, Lyme disease, leprosy, molluscum
contagiosum), inflammatory (granuloma annulare,
discoid lupus, sarcoidosis, lichen planus) and tumoral
(pilomatricomas, juvenile xanthogranuloma, xantho-
1194 mas, involuted infantile hemangiomas, cutaneous
B-cell lymphoma) to mention only a few. Anetoderma
Kang_CH070_p1193-1202.indd 1194
TABLE 70-1
Conditions Associated with Secondary
Anetoderma
Infectious
■ Syphilis
■ Lyme disease
■ Leprosy
■ Molluscum contagiosum
Inflammatory
■ Granuloma annulare
■ Discoid lupus erythematosus
■ Sarcoidosis
■ Lichen planus
Tumoral
■ Pilomatricomas
■ Juvenile xanthogranuloma
■ Xanthomas
■ Involuted infantile hemangiomas
Other Conditionsa
■ Antiphospholipid syndrome
■ Autoimmune thyroiditis
■ HIV
a
Also associated with primary anetoderma.
also has been described in premature infants, possi-
bly related to the use of cutaneous monitoring leads
or adhesives as well as extreme prematurity.7 Both
primary and secondary types of anetoderma may
be associated with an underlying disease, mainly
antiphospholipid syndrome8 autoimmune thyroiditis
and HIV, in which cases the atrophic lesions do not
necessarily develop in areas of skin inflammation.
Although most cases are sporadic, rare cases of famil-
ial anetoderma have been recently described and are
usually not associated with preexisting lesions.9
PATHOLOGY
In routinely stained sections, the collagen fibers within
the dermis of affected skin appear normal. Perivascu-
lar lymphocytes, in majority T-helper cells, are often
present in all types of anetoderma and do not correlate
with clinical inflammatory findings.10
The predominant defect as revealed by elastic tis-
sue stains is a focal partial or complete loss of elastic
tissue in the papillary and/or midreticular dermis.
There are usually some residual abnormal, irregular,
and fragmented elastic fibers (Fig. 70-2). Presumably,
the weakening of the elastic network leads to flaccidity
and herniation. Direct immunofluorescence sometimes
shows linear or granular deposits of immunoglobulins
and complement along the dermal–epidermal junction
or around the dermal blood vessels in affected skin.11
Electron microscopy demonstrates that the elastic
fibers are fragmented and irregular in shape, occasion-
ally engulfed within macrophages.
03/12/18 9:26 am

Figure 70-2 Anetoderma. Pathology shows decrease of
elastic fibers in the papillary and reticular dermis (Weigert
stain). (Used with permission from Victor Kokta, MD.)
DIFFERENTIAL DIAGNOSIS
Anetoderma must be differentiated from other disor-
ders of elastic tissue as well as atrophies of the connec-
tive tissue (Table 70-2).
Keloids form nodules that are much firmer on pal-
pation. A history of trauma is often elicited, and the
pathology is very distinct.
Glucocorticoid-induced atrophy occurs most com-
monly over the triceps or buttocks at sites where injec-
tions are usually given. Clinically, the lesions resemble
atrophoderma. History is obviously most helpful in
making the diagnosis. On histopathology, polarization
may show the steroid crystals in the dermis.
Nevus lipomatosus superficialis of Hoffman and
Zurhelle presents as a clustered group of soft, skin-
colored to yellow nodules usually on the lower trunk
and buttocks and present since birth. Histology shows
ectopic mature lipocytes located in the dermis.
TABLE 70-2
Differential Diagnosis of Primary Anetoderma
ELEVATED DEPRESSED
Secondary anetoderma Secondary anetoderma
Acne scars Glucocorticoid-induced atrophy
Keloids Acne scars
Nevus lipomatosus superficialis
Papular elastorrhexis
Connective tissue nevi
Kang_CH070_p1193-1202.indd 1195
Papular elastorrhexis is an acquired disorder charac-
terized by white, firm nonfollicular papules measuring
11
1 to 3 mm, evenly scattered on the chest, abdomen, and
back. It usually appears in adolescence or early adult-
hood. The pathology demonstrates focal degeneration
of elastic fibers and normal collagen. There are no asso-
ciated extracutaneous abnormalities. This is believed
by some authors to be a variant of connective tissue
nevi12 or an abortive form of the Buschke-Ollendorff
syndrome,13 whereas others think that these represent
papular acne scars.14 They are differentiated from ane-
toderma by being firm noncompressible lesions.
Middermal elastolysis (MDE) usually consists of
larger areas with diffuse wrinkling without hernia-
Chapter 70 :: Anetoderma and Other Atrophic Disorders of the Skin
tion and with elastolysis limited to the middermis (see
“Middermal Elastolysis” section).
TREATMENT
There is no regularly effective treatment. In secondary
anetoderma, appropriate treatment of the inflamma-
tory underlying condition might prevent new lesions.
Various therapeutic modalities have been tried but with
no improvement of existing atrophic lesions, includ-
ing intralesional injections of triamcinolone, and sys-
temic administration of aspirin, dapsone, phenytoin,
penicillin G (benzylpenicillin), and vitamin E. Some
authors have reported improvement with hydroxy-
chloroquine. In patients with limited lesions that are
cosmetically objectionable, surgical excision may be
useful. Ablative and nonablative fractionated lasers
have shown some improvement in limited cases.15,16
The use of soft-tissue fillers is inconclusive.
OTHER ATROPHIC
DISORDERS OF THE SKIN
MIDDERMAL ELASTOLYSIS
MDE is a rare acquired disorder of elastic tissue. It is
characterized by patches and plaques of diffuse, fine,
wrinkled skin, most often located on the trunk, neck,
and arms. In 1977, Shelley and Wood reported the first
case of “wrinkles due to idiopathic loss of middermal
elastic tissue.”17 Since then, approximately 100 cases
have been reported. The vast majority of patients are
white women between the ages of 30 and 50 years.17-19
PATHOGENESIS
The pathogenesis of this acquired elastic tissue degen-
eration is still unknown. Ultraviolet exposure has
been postulated to be a major contributing factor in
the degeneration of elastic fibers,20 including natural
sunlight and narrowband ultraviolet B phototherapy.21
Other possible mechanisms include defects in the syn-
1195
thesis of elastic fibers, autoimmunity against elastic
03/12/18 9:26 am
11 fibers, and damage to elastic fibers through the release
of elastase by inflammatory cells or fibroblasts. Of
interest, MDE has been reported in a case of immune
reconstitution inflammatory syndrome.22 Some data
suggest that inflammatory processes and an imbalance
between matrix metalloproteinases and tissue inhibi-
tor of metalloproteinases are probably involved in the
pathogenesis of MDE, in addition to CD34+ dendritic
fibroblasts.23 A decrease of lysyl oxidase-like 2 expres-
sion potentially has an effect on elastin renewal.24 A
recent study has shown that dermal fibulin-4 and
fibulin-5 are significantly diminished in MDE compared
with controls. This data indicates that the pathogenesis
is not only secondary to an elastolytic overactivity, but
Part 11
also altered reassembly of elastic fibers.6
CLINICAL FEATURES
::
MDE is characterized by asymptomatic, well-demar-
Dermal Connective Tissue Disorders
cated, or diffuse areas of fine wrinkling (type I),
usually in a symmetric distribution (Fig. 70-3A). Dis-
crete perifollicular papules can be seen in some cases
(type II), leaving the hair follicle itself as an indented
center. More rarely, a reticular pattern (type III) with
erythematous patches and telangiectasia can be seen.
Lesions are typically found on the trunk, neck, and
upper extremities. They are chronic and give the skin
a prematurely aged appearance. There is usually no
history of a preceding inflammatory dermatosis, but
some patients report mild-to-moderate erythema and
more rarely urticarial lesions or granuloma annulare.
There is usually no associated systemic involvement.
Although the diagnosis of MDE is mainly based
on clinical and histopathologic features, noninvasive
diagnostic techniques (optical coherence microscopy
or high-frequency ultrasound) may be helpful.25
A B
Figure 70-3 Middermal elastolysis. A, Well-circumscribed area of fine wrinkling on the neck of a middle-aged woman.
(Used with permission from Richard Dubuc, MD.) B, Histology of middermal elastolysis. Note selective loss of elastic fibers
1196 in the middermis. Normal elastic tissue is preserved in the superficial papillary dermis and in the reticular dermis (Weigert
stain). (Used with permission from Danielle Bouffard, MD.)
Kang_CH070_p1193-1202.indd 1196
PATHOLOGY
Histopathology shows a normal epidermis and, occa-
sionally, a mild perivascular infiltrate in the dermis.
The characteristic features are seen on elastic tissue
stains (such as Verhoeff-van Gieson or Weigert) and
reveal a selective band-like loss of elastic fibers in the
middermis (see Fig. 70-3B). Macrophagic elastophago-
cytosis can occasionally be seen. There is preservation
of normal elastic tissue in the superficial papillary der-
mis above, in the reticular dermis below, and along
adjacent hair follicles. Electron microscopy studies
have shown phagocytosis by macrophages of both
normal and degenerated elastic fiber tissue.26
DIFFERENTIAL DIAGNOSIS
MDE must be differentiated from the other common
disorders of elastic tissue.
Solar elastosis differs by its onset in an older age
group, location in only sun-exposed areas, yellowish
color, and coarser wrinkling, as well as by hyperpla-
sia and abnormalities of elastic fibers and basophilic
degeneration of the collagen in the papillary dermis.
Anetoderma is characterized clinically by smaller
soft macules and papules instead of diffuse wrinkling,
and histologically by elastolysis that can occur in any
layer of the dermis.
Perifollicular elastolysis27 differs by a selective and
almost complete loss of elastic fibers surrounding hair
follicles compared with preservation of elastic fibers
around follicles in MDE. Elastase-producing Staphylo-
coccus epidermidis was found in the hair follicles and is
the presumed etiology of this condition.
Postinflammatory elastolysis and cutis laxa were
originally described in young girls of African descent.
03/12/18 9:26 am
 An inflammatory phase, consisting of indurated
plaques or urticaria, malaise, and fever, preceded the
diffuse wrinkling, atrophy, and severe disfigurement.
Insect bites may be the trigger for the initial inflamma-
tory lesions.28
TREATMENT
There is no known effective treatment for MDE. Sun-
screens, colchicine, chloroquine, vitamin E, and topical
retinoic acid have been tried without good success.17-19
Topical soybean extract and eicosapentaenoic acid
may prove to be interesting options.18
STRIAE
Chapters 105 and 137 provide additional discussion of
striae.
Striae are very common and usually develop
between the ages of 5 and 50 years.29 They occur about
twice as frequently in women as in men. They com-
monly develop during puberty, with an overall inci-
dence of 25% to 35%,30 or during pregnancy, with an
incidence of up to 90%.31
PATHOGENESIS
The factors leading to the development of striae have
not been fully elucidated. Striae distensae are the
results of breaks in the connective tissue, resulting in
dermal atrophy. Many factors, including hormones
(particularly corticosteroids), mechanical stress, and
genetic predisposition, appear to play a role.
CLINICAL FEATURES
Striae are usually multiple, symmetric, well-defined
linear atrophic lesions that follow the lines of cleavage.
Initially, striae appear as red-to-violaceous elevated
lines (striae rubra). Over time, the color gradually
fades, and the lesions become atrophic, with the skin
surface exhibiting a fine, white, wrinkled appearance
(striae alba). The striae can measure several centime-
ters in length and a few millimeters to a few centime-
ters in width.
During puberty, striae appear in areas where there is
a rapid increase in size. In girls, the most common sites
are the breasts, thighs, hips, and buttocks, whereas
in boys, they are seen on the shoulders, lumbosacral
region, and thighs. Other less-common sites include
the abdomen, upper arms, neck, and axillae.
Striae distensae are a common finding on the abdo-
men, and less so on the breasts and thighs, of preg-
nant women, especially during the last trimester. They
are more common in younger primigravidas than in
older pregnant women, and are associated with larger
weight gain and/or with babies of higher birth weight.
Striae gravidarum can be associated with a higher
risk of lacerations during vaginal delivery,32 as well
Kang_CH070_p1193-1202.indd 1197
as the development of pelvic relaxation and clinical
prolapse.33
11
The striae associated with systemic corticosteroid
therapy and Cushing syndrome can be larger and
more widely distributed.
PATHOLOGY
Histologic findings show a decrease in dermal thick-
ness and in collagen in the upper dermis. The collagen
bundles are thinned and lie parallel to the epidermis,
but they are also arranged transversely to the direction
of the striae. Alterations in elastic fibers are variable,
but dermal elastin can be fragmented, and specific
Chapter 70 :: Anetoderma and Other Atrophic Disorders of the Skin
elastin staining can demonstrate a marked reduction
in visible elastin content compared with adjacent nor-
mal dermis.34 There is absence of both hair follicles and
other appendages.
DIFFERENTIAL DIAGNOSIS
The diagnosis of striae distensae is usually straightfor-
ward, but the differential diagnosis does include linear
focal elastosis (elastotic striae) that was first described
by Burket and colleagues in 1989.35 Linear focal elasto-
sis is characterized by rows of yellow palpable striae-
like bands on the lower back. Unlike striae, the lesions
are raised and yellow rather than depressed and white.
Elderly men are most commonly affected, although
cases in teenagers have been described. Linear focal
elastosis is probably not an uncommon condition. His-
tologically, there is a focal increase in the number of
elongated or fragmented elastic fibers and a thickened
dermis. It is postulated that linear focal elastosis may
represent an excessive regenerative process of elastic
fibers and could be thought of as a keloidal repair of
striae distensae.36
TREATMENT37
Striae distensae have no medical consequences, but
they are frequently distressing to those affected. As
stretch marks tend to regress spontaneously to some
degree over time, the usefulness of treatments that have
been tried without case controls is difficult to assess.
Topical treatments that have shown some improve-
ment of early stage striae are tretinoin 0.1% cream,38
a combination of 0.05% tretinoin/20% glycolic acid,
or 10% l-ascorbic acid/20% glycolic acid.39 Several
lasers40 have been used in treating striae: the 585-nm
pulsed-dye laser has been demonstrated to be of some
efficacy in improving the appearance of striae rubra
but has no effect on striae alba. Preliminary data have
shown improvement of striae alba with fractionated
microneedle radiofrequency in combination with frac-
tional carbon dioxide laser.41 Microneedling and com-
bination treatment with radiofrequency and pulsed
magnetic fields also appear promising.42,43 The long-
term future of treatment strategies is encouraging with 1197
the advance in laser technologies.
03/12/18 9:26 am
11 IDIOPATHIC
ATROPHODERMA OF PASINI
AND PIERINI
Idiopathic atrophoderma of Pasini and Pierini is a
form of dermal atrophy that presents as 1 or several
sharply demarcated depressed patches with no out-
pouching, usually on the back of adolescents or young
adults.44 Whether atrophoderma is a nonsclerotic, pri-
marily atrophic variant of morphea or a separate dis-
tinct entity is still debated.45
Part 11

EPIDEMIOLOGY AND
PATHOGENESIS
::

This disorder is more frequently encountered in

Dermal Connective Tissue Disorders

women than in men, with a ratio of 6:1. It usually

starts insidiously in young individuals in the second
or third decades of life. Congenital cases have been
reported.46
Its relationship to morphea is favored by its strik-
ing clinical and histologic similarities to the atrophy
seen at sites of regressing plaques of morphea. Anti-
bodies to Borrelia burgdorferi have been reported.47
Typical lesions of morphea, lichen sclerosus, and
atrophoderma have been observed to occur simul-
taneously in the same patient, but in different
areas, supporting the view that these conditions are
related.48 In a series of 139 patients, 24 (17%) had
white induration in the central portions of their atro-
phic lesions, and 30 (22%) had superficial plaques
of morphea coexisting in areas outside of their atro-
phic foci.49 However, to some, the different course
and outcome of atrophoderma of Pierini and Pasini
as compared with morphea justifies preservation of
a distinct name.
CLINICAL FEATURES
The lesions are well-demarcated depressed patches,
usually occurring on the trunk, especially on the back
and lumbosacral region, followed in frequency by the
chest, arms, and abdomen.47,49 The distribution is often
symmetric and bilateral.
The lesions are single or multiple and usually round
or ovoid, ranging in size from a few centimeters to
patches covering large areas of the trunk (Fig. 70-4).
They are usually asymptomatic and lack inflamma-
tion. When lesions coalesce, they can form large, irreg-
ular, brown patches but can be hypopigmented.49 The
surface of the skin is normal in appearance, and there
is no skin induration or sclerosis.
The borders or edges of these lesions are sharply
defined, and they are usually described as abrupt,
“cliff-drop” borders ranging from 1 to 8 mm in depth,
although they can have a gradual slant.46 These
1198 depressed patches are characteristic and give the
impression of inverted plateaus, or, if multiple lesions
Figure 70-4 Atrophoderma of Pasini and Pierini. Brownish
depressed lesions on the lower back.
are present, they can have the appearance of Swiss
cheese. They are even more apparent when pres-
ent on the back because the dermis is thicker in this
area. The skin surrounding the patches is normal in
appearance, and there is no erythema or lilac ring as
in morphea.
The course of this benign disease is progressive,
and lesions can continue to appear for decades before
reaching a standstill. Transformation to generalized
morphea has not been observed.
PATHOLOGY
The histologic picture is generally not diagnostic.47,49
The epidermis is usually normal. Collagen bundles
in the middermis and reticular dermis show varying
degrees of homogenization and clumping. Dermal
thickness is eventually reduced when compared with
adjacent normal skin. Some irregular clumping and loss
of elastic fibers were described in earlier case reports,
but in most series, no abnormality was seen with elas-
tic tissue stains; consequently, this is not of diagnostic
value. The appendages are usually preserved. If sclero-
dermatous changes appear in preexisting patches, the
histology reveals varying degrees of collagen sclerosis
resembling morphea. A recent study using multipho-
ton microscopy suggests that the atrophic appearance
of atrophoderma lesions reflect changes in the organi-
zation of collagen and elastic fibers and not variation
in their content.50
DIFFERENTIAL DIAGNOSIS
The differential diagnosis is to be made with active
lesions of morphea that usually present as indurated,
often hyperpigmented plaques with a characteristic
peripheral lilac rim.

Kang_CH070_p1193-1202.indd 1198 03/12/18 9:26 am

 TREATMENT 11
No treatment has proven effective. Penicillin and dox-
ycycline have been used with poor results. Dramatic
response to oral hydroxychloroquine was reported in
1 patient.51 Q-switched alexandrite laser can maybe
help decrease hyperpigmentation.52

FOLLICULAR
ATROPHODERMA
Follicular atrophoderma refers to dimple-like depres-
sions at the follicular orifices. It can occur as an iso-

Chapter 70 :: Anetoderma and Other Atrophic Disorders of the Skin

lated defect of limited extent, in association with a
variety of disorders in which hair follicles are plugged
with keratin, or with rare genodermatoses.53, 54
Distinctive ice-pick depressions around hair follicles
can be seen most commonly on the cheeks and on the
back of the hands or feet. These pitted scars can pres-
ent at birth or early in life. A family history may be Figure 70-5 Atrophoderma vermiculatum. Multiple, small,
present. Follicular atrophoderma occurs in the condi- pitted scars on the cheek of a young girl.
tions described in the following sections.
over the malar area and progresses to involve the eye-
brows, scalp, and extremities, with scarring alopecia.
ATROPHODERMA This condition is inherited in an X-linked recessive
fashion in some patients. Ulerythema ophryogenes (or
VERMICULATUM keratosis pilaris atrophicans faciei) differs from atro-
phoderma vermiculatum by affecting primarily the
Atrophoderma vermiculatum is a term that applies lateral portion of the eyebrows (ophryogenes) with
when the lesions are found exclusively on the cheeks.55 erythema, follicular papules, and alopecia (Fig. 70-6).
It is a condition that can either occur sporadically, The underlying pathologic defect in these disorders
be inherited as an autosomal dominant disorder, be appears to be abnormal follicular hyperkeratinization
part of a group of related diseases including kerato- of the upper third of the hair shaft leading to obstruc-
sis pilaris atrophicans, or be associated with various tion of the growing hair and production of chronic
syndromes. inflammation. The end result of this process is scarring
Multiple inflammatory symmetric papules on the below that level. Histopathology is usually not very
cheeks, presumably centered around hair follicles,
may precede the atrophic lesions. These papules then
go on to develop pitted, atrophic, and depressed scars
in a reticulated or honeycomb pattern (Fig. 70-5). These
lesions can extend to the forehead and preauricular
regions. This condition usually has its onset in child-
hood or, less often, around puberty. Men and women
seem to be affected equally. It usually has a slow pro-
gressive course.

KERATOSIS PILARIS
ATROPHICANS
Keratosis pilaris atrophicans55,56 can include atropho-
derma vermiculatum but also a group of closely related
disorders that includes keratosis follicularis spinulosa
decalvans and ulerythema ophryogenes. These condi-
tions are characterized by keratotic follicular papules,
variable degrees of inflammation, and secondary atro-
phic scarring. Keratosis follicularis spinulosa decal- Figure 70-6 Ulerythema ophryogenes. Erythematous fol- 1199
vans begins in infancy with keratotic follicular papules licular papules and scarring alopecia of the eyebrow.

Kang_CH070_p1193-1202.indd 1199 03/12/18 9:26 am

11 helpful and shows dilated follicles, sometimes asso-
ciated with plugging, inflammation, and sclerosis of
dermal collagen.
ASSOCIATED SYNDROMES
The various syndromes that include atrophoderma
vermiculatum are Rombo syndrome (milia, telangiec-
tasias, basal cell carcinomas, hypotrichosis, acral cya-
nosis, and, rarely, trichoepitheliomas), Nicolau-Balus
syndrome (syringomas and milia), Tuzun syndrome
(scrotal tongue), Loeys-Dietz syndrome,57 and finally
Part 11
the Braun-Falco-Marghescu syndrome (palmoplantar
hyperkeratosis and keratosis pilaris).
::
THERAPY
Dermal Connective Tissue Disorders
These disorders are mainly a cosmetic but vexing prob-
lem. Various topical treatments, including emollients,
corticosteroids, tretinoin, and keratolytics, have shown
no consistent benefit. Systemic isotretinoin has been
shown to stop progression and to induce remission
in some cases.55 Dermabrasion as well as carbon diox-
ide and 585-nm pulsed-dye lasers are other options to
improve the appearance of the atrophic scars.58
BAZEX-DUPRÉ-CHRISTOL
SYNDROME (OMIM 301845)
Bazex-Dupré-Christol syndrome is characterized by
follicular atrophoderma, milia, multiple basal cell car-
cinomas, hypotrichosis, and localized hypohidrosis.59
The follicular atrophoderma described as multiple
ice-pick marks or patulous follicles can be found most
commonly on the dorsa of the hands. It is inherited in
an X-linked dominant fashion, and the gene has been
linked to Xq24-q27.60 Additional reported findings
include facial hyperpigmentation, hair shaft dystrophy,
and multiple trichoepitheliomas. This syndrome might
be better considered as an ectodermal dysplasia.61
CONRADI-HÜNERMANN-
HAPPLE SYNDROME
(X-LINKED DOMINANT
CHONDRODYSPLASIA
PUNCTATA, CDPX2,
OMIM 302960)
1200 Conradi-Hünermann-Happle syndrome is an X-linked
dominant disorder that occurs only in girls because
Kang_CH070_p1193-1202.indd 1200
it is usually lethal in hemizygous males. The under-
lying molecular defect consists of mutations in the
emopamil-binding protein gene at Xp11.23-p11.22.62
The clinical manifestations include an ichthyosiform
scaling erythroderma patterned along the lines of
Blaschko that usually resolves during the first year
of life and is replaced by bands of follicular atropho-
derma. Hyperpigmentation, cataracts, scarring alope-
cia, saddle-nose deformity, asymmetric limb reduction
defects, and stippled calcifications of the epiphyses
can be seen. Ichthyosis with keratotic follicular plugs
containing dystrophic calcification in newborns are
distinctive histopathologic features.63
OTHER ATROPHIES OF THE
CONNECTIVE TISSUE
Many systemic conditions (scleroderma [see Chap. 63],
lupus erythematosus [see Chap. 61], dermatomyositis
[see Chap. 62]), and genodermatoses (poikiloderma
congenitale, dyskeratosis congenita, Cockayne syn-
drome, Hallermann-Streiff syndrome) have skin atro-
phy as an associated finding and are described in other
chapters.
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