Title: Pure Vulvar Langerhans Cell Histiocytosis: A Case Report and Literature Review

Welcome to our comprehensive exploration of pure vulvar Langerhans Cell Histiocytosis (LCH). In
this article, we delve into a detailed case report along with an extensive literature review, shedding
light on this rare and intriguing medical condition.

Langerhans Cell Histiocytosis (LCH) is a group of disorders characterized by the abnormal

accumulation and proliferation of Langerhans cells. While LCH can affect various parts of the body,
its occurrence in the vulvar region is exceedingly rare, making each documented case a significant
contribution to medical literature.

Crafting a thorough literature review on such a niche topic is a challenging endeavor. It requires
meticulous research, precise analysis, and a deep understanding of the subject matter. The scarcity of
available literature adds another layer of complexity, demanding exhaustive searches across diverse
academic databases and medical journals.

Our case report provides a detailed examination of a specific instance of pure vulvar LCH, offering
valuable insights into its clinical presentation, diagnostic challenges, and treatment modalities.
Complemented by a comprehensive literature review, this article serves as a valuable resource for
healthcare professionals and researchers alike.

For those seeking assistance in navigating the intricacies of literature review writing or in crafting
their own academic papers on medical topics, we recommend availing the services of ⇒
StudyHub.vip ⇔. With a team of experienced writers and researchers specializing in diverse fields,
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In conclusion, our exploration of pure vulvar Langerhans Cell Histiocytosis stands as a testament to
the ongoing pursuit of knowledge in the medical field. Through diligent research and collaboration,
we aim to contribute to the collective understanding of rare medical conditions and pave the way for
improved diagnosis and treatment strategies.
Cancer 1995;76(12): 2471-2484. PubMed 28. Howarth DM, Gilchrist GS, Mullan BP, Wiseman GA,
Edmonson JH, Schomberg PJ. The information obtained in the review was organized through a data
collection instrument that included the following variables and outcomes: year of publication, study
design, age, symptoms, history, morphology of the lesions, histopathological examination,
management, follow-up time after treatment and presence of relapse (Flow chart). Since 50-80
percent of cases manifest cutaneous involvement, a skin biopsy provides a rapid and accessible
means to secure. Endocrine abnormalities are relatively common in LCH multisystem disease. All
presented with superficial lymph node enlargement. Histiocytosis X: Histogenic arguments for a
Langerhans cell origin. Corresponding author: Dr. Chandra Sekhar Sirka, Department of
Dermatology and Venereology, AIIMS, Sijua, Patrapada, Bhubaneswar - 751 019, Odisha, India.
Although a predilection for gnathic bones was reported in 20% of the cases, in this patient, the
syndrome primarily involved the skull, femur and iliac bones as well as skin and uterus. This is an
open-access article distributed under the terms of the Creative Commons Attribution License, which
permits unrestricted use, distribution, and reproduction in any medium, provided the original author
and source are credited. Reading helps your vocabulary According to researchers, children who
regularly read books at an early age develop large vocabularies in the long run. Positivity for protein
S100 (x4) A 2-month-old male was observed in our department because of a 3-week-history of
asymptomatic papules on the trunk. Her physical examination was significant for bilateral peripheral
visual field loss. The main aim of the research is to analyze the role of information technologies in the
formation of leadership qualities of young people, to study the relationship between the students’
academic performance and their le. Treatment was started with chemotherapy with cytarabine and
pegfiligrastin with the disappearance of skin and vulvar lesions. On hematoxylin and eosin stain, the
biopsy classically consists of reniform Langerhans cells associated with an eosinophil-rich
inflammatory infiltrate. In this report, a 10 year old boy presented who afflicted with LCH 3 years
after diagnosis and treatment of mixed cellularity Hodgkin lymphoma. Add 'Search on Flickriver' to
your browser's search box. In the present case report, the patient presented not only lytic bone
lesions but also skin and uterus lesions. The patient presented with painful symptoms at age of 20-
years. Langerhans cell histiocytosis presenting in the neonatal period: A retrospective case series.
Langerhans cell histiocytosis: the case for conservative treatment. The later methodology is based
upon obtaining a score derived from three primary parameters including: clinical examination. The
provisional radiologic diagnosis of Langerhans Cell Histiocytosis of the clavicle was confirmed by an
incisional biopsy of the left mid-clavicle lesion. Although LCH is more common and best
characterized in children, the disorder also presents in adults. Higher radiotherapy doses are easily
achievable in this region using modern radiotherapy techniques that maintain minimal risk of
radiation-related toxicities. Medical College, Chandigarh, India 2 Departments of Dermatology,
Govt. To browse Academia.edu and the wider internet faster and more securely, please take a few
seconds to upgrade your browser. To date, the exact underlying pathogenesis of this condition in one
or more bones of the human body remains unknown. Childhood histiocytosis: A review of twenty
two cases.
Although a handful of cases involving LCH limited to the hypothalamic region have been reported,
this one represents only the second example of recurrence after radiation therapy. Vocabulary growth
is an inevitable consequence of reading. J Pediatr 1975; 87(2):184-189. PubMed 21. Arceci RJ,
Brenner MK, Pritchard J. The images revealed bone rarefaction in the lower limbs. Between
December 2008 and June 2011 2090 consecutive HIV-negative adults (?18 years of age) with new
smear positive pulmonary TB presenting at participating district TB units in Ho Chi Minh City were
recruited. Incidence and survival of childhood Langerhans cell. We thank the SCC-WUSM and
Barnes-Jewish Hospital in St. Louis, Mo., for the use of the Clinical Trials Core, which provided
regulatory service. Case presentation A 50-year-old Caucasian woman who had headaches, memory
difficulties, and diabetes insipidus was found to have a 2.5cm suprasellar mass. Cancer 1995;76(12):
2471-2484. PubMed 28. Howarth DM, Gilchrist GS, Mullan BP, Wiseman GA, Edmonson JH,
Schomberg PJ. Its presentation can differ from a mild bone lesion to a multi-systemic evolved
malignant neoplasm; however, the latter outcome is almost rare. Systemic involvement occurs mainly
in children and local involvement in adults. Although some studies found that the younger the patient
is at the time of diagnosis, the worse the prognosis, this only. Patients with multisystemic
involvement did not show any systemic symptoms but had lymphadenopathy and enlargement of the
liver and spleen. According to different management and treatment of these conditions, exact
histopathologic evaluation and even using immunohistochemistery (IHC) can prevent misdiagnosis.
The two conditions have similarities both clinically and histopathologically. A possible hypothesis is
that the systemic changes impacted the patient's quality of life, and that the symptoms were
debilitating enough for the patient to neglect her oral health. Clinically, LCH is a polymorphic
disease that can affect one or several organs. Taking into account the biopsy, diabetes insipidus, and
secondary amenorrhea, the diagnosis of Langerhans cell histiocytosis with multisystem involvement
were considered. His recurrence was successfully controlled with vinblastine, cyclophosphamide and
prednisone. Many lymphocytes are admixed with the Langerhans histiocytes. According to the
degree of commitment, the proposed treatment can vary between only local resection and the
application of topical corticosteroids or nitrogen mustard, up to the requirement of radical
vulvectomy associated with adjuvant management with chemotherapy or radiotherapy. She
underwent CT simulation for radiation therapy planning. Methods: Clinical and histopathological
findings of five cases of cutaneous LCH were reviewed based on the hospital records. Commonly
affected organs include the lungs, skin, and bone; however, virtually any tissue may be involved,
including the stomach, central nervous system, thyroid, and liver. The main aim of the research is to
analyze the role of information technologies in the formation of leadership qualities of young people,
to study the relationship between the students’ academic performance and their le. Hematoxylin and
eosin stained sections ( A and B ) show gliotic brain parenchyma with diffuse involvement by plasma
cells, lymphocytes, and foci of histiocytes ( A ); high magnification of histiocytic aggregate shows
cells with pale granular eosinophilic cytoplasm and grooved nuclei, indicated by arrows ( B ). In the
present clinical case, the patient was intravenously administered monthly doses of bisphosphonates,
which resulted in adequate control of bone activity. Mucositis and neutropenia developed early in the
course of his. Case presentation A 50-year-old right-handed previously healthy Caucasian woman
presented with a several month history of daily headaches, blurry vision, worsening daytime
somnolence, and progressive memory difficulties. Patients who respond to chemotherapy during the
first 6 weeks.
Initial radiation treatment succeeded in shrinking the tumor and relieving clinical symptoms
temporarily; however, growth and recurrence of clinical symptoms was noted at 9 months. Methode
yang digunakan dalam perancangan ini adalah methode waterfall, dimana metode waterfall
menggambarkan pendekatan yang sistematis dan juga berurutan pada pengembangan perangkat
lunak. The prognosis is related to the age at onset, the progression of disease, and the presence of
systemic involvement. Organ. Hemogram, liver function tests and radiographs of the chest and skull
were normal in all the cases. While viewing any Flickr photos page, click on this button to open the
same view on Flickriver. The benefits of reading books include exposing the reader to new words
directly from the read context and increasing your soft skills. Commonly affected organs include the
lungs, skin, and bone; however, virtually any tissue may be involved, including the stomach, central
nervous system, thyroid, and liver. Vocabulary growth is an inevitable consequence of reading. While
viewing any Flickr photos page, click on the bookmarklet to open the same view on Flickriver. Based
on the initial good response of the tumor to fractionated therapy and the lack of any systemic
disease, a second course of radiation was prescribed. Conclusion Isolated hypophyseal involvement
by Langerhans cell histiocytosis in adults is a unique presentation of a rare disease. With modern
imaging and radiotherapy techniques higher doses should offer little increased more durable risk to
surrounding critical structures. The follow-up varied between 1.5 and 130 months with an average of
23.7 months. There were 6 relapses (16.2%). Descriptions of findings in the physical exam were not
specific, and therefore do not allow to determine any characteristic in the presentation that is
associated with a higher risk for relapse. Patients with more abnormal findings receive a higher score,
and therefore. Epidemiologic study of Langerhans cell histiocytosis. This case report might
contribute to a better understanding of the pathogenesis of LCH and will help to expand the
knowledge of health professionals, especially dental surgeons, about this condition. However, with
the advent of immunohistochemical stains, the Histocyte Society proposed reclassification. Within
the female genital tract, the vulva is the most frequent site of involvement. The treatment is
determined according to the severity, size of the lesions, and systemic involvement. It generally does
not present cutaneous, pulmonary, or pituitary manifestations. Nguy?n Th? Qu?nh Nhu 2018, BMC
infectious diseases Drug resistant tuberculosis (TB) is increasing in prevalence worldwide. The aim of
this study was to determine the optimal duration of planned delay before surgery in patients who
have had SARS?CoV?2 infection. This case report is unique in that it presented with involvement of
temporal bone wherein it can be confused with inflammatory pathologies like Cholesteatoma and
tumours like Rhabdomyosarcoma. The patient, a 31-year-old woman, leucoderma, presented with a
more than 10-year history of pain in the lower limbs. Clinical symptoms are related to the site of
disease. In the oral cavity, the treatment is restricted to classic periodontal therapy with scaling and
root planning, or a surgical approach. Scattered lymphocytes and rare neutrophils are seen in this
portal tract. The epidermis was focally ulcerated adjacent to areas in which there was
epidermotropism of. The LCs in LCH manifest an activated immunophenotype, resulting in their
increased proliferation and migration. Aberrant. Nowadays, there are no clearly established protocols
for the management of these patients.
The mass appeared to be separate from the pituitary on both sagittal and coronal imaging (Figure 1 ).
Very rarely, such cells were binucleated and exhibited folds in the nuclear membrane. To link to this
page, Copy and Paste the following HTML code. Langerhans cell histiocytosis: malignancy or
inflammatory disorder doing a great job of imitating. The patient was treated with chemotherapy and
autologous stem cell transplantation, and experienced no further symptoms. Further, no evidence of
recurrence was observed on follow-up imaging. Reading helps your vocabulary According to
researchers, children who regularly read books at an early age develop large vocabularies in the long
run. The second skin biopsy showed the same features as the first one, compatible with Langerhans
cell. Fluid attenuated inversion recovery ( E ) and T2-weighted imaging ( F ) demonstrate mild
edema in the surrounding deep thalamic nuclei and brainstem. Erythema and maceration of inguinal
folds Figure 7 Figure 8 Figure 7. Pediatr Dermatol 1998;5(3):145-150. PubMed 24. Esterly NB,
Mauer HS, Gonzalez-Crussi F. During this time, an extensive cutaneous eruption also became
apparent. No atypical lymphocytes, no multinucleated giant cells, no infectious organisms or viral
inclusions, and only rare eosinophils were identified. Hemogram, liver function tests and radiographs
of the chest and skull were normal in all the cases. Methods: Clinical and histopathological findings
of five cases of cutaneous LCH were reviewed based on the hospital records. Further
characterization of the histiocytes showed strong reactivity for S100, CD163, and CD1a (Figure 3 ),
supporting the diagnosis of LCH. Figure 2. MRI at this time demonstrated an interval increase in
lesion size from 8mm to 14mm and increased edema in the surrounding tissues. The spectrum of
manifestations varies from isolated lesions to a disseminated disease with multiorgan involvement.
We excluded articles that reported cases of non-vulvar Langerhans histiocytosis and that did not
have histological confirmation. The patient was started on oral prednisolone 40 mg daily and
methotrexate 15 mg once weekly. Progressive weight loss had been noticed over the last year, which
was. As more information has become available, a revised classification schema was proposed and
includes: dendritic cell disorders. Pediatr Blood Cancer 2007 48(5):555-560. PubMed 6. Schmitz L,
Favara BE. Taking into account the biopsy, diabetes insipidus, and secondary amenorrhea, the
diagnosis of Langerhans cell histiocytosis with multisystem involvement were considered. Report
from the International Registry of the Histiocyte Society. Proposals by the Histiocyte Society have
assisted in the unification of LCH. However, with the advent of immunohistochemical stains, the
Histocyte Society proposed reclassification. Download Free PDF View PDF Free PDF Langerhans
cell histiocytosis following Hodgkin lymphoma: a case report from Iran Parvin Rajabi 2010, Journal
of research in medical sciences: the official journal of Isfahan University of Medical Sciences The
occurrence of Langerhans Cell Histiocytosis (LCH) in a patient with lymphoma is an indication of a
probable relationship between them. All patients showed a response; complete response (CR) was
observed in five patients. Twenty-four months after completion of the treatment protocol, he is
without evidence. To date, the exact underlying pathogenesis of this condition in one or more bones
of the human body remains unknown.
The infiltrate usually lies close to the epidermis and may result in ulceration and crusting. Included in
this category, are conditions that were previously designated as eosinophilic. In the present clinical
case, the patient was intravenously administered monthly doses of bisphosphonates, which resulted
in adequate control of bone activity. Treatment of multi-organ disease is controversial, with some
advocating high-dose prednisone as the first-line therapy. See Full PDF Download PDF Free
Related PDFs Langerhans cell histiocytosis followed by Hodgkin's lymphoma Jahanbanoo Shahryari
2012, The Korean journal of internal medicine A 22-year-old man was referred to our institution due
to lower back pain and was diagnosed with Langerhans cell histiocytosis of the thoracic and lumbar
spine. The anti-core factor (FAN) and rheumatoid factors, C3 and C4, showed normal values. There
were some multinucleated giant cells, with frequent lymphoplasmocytes intermingled with groups of
eosinophilic polymorphs (Figure 2). Histopathological examination reveals the presence of abnormal
Langerhans cells in an inflammatory background of eosinophils, histiocytes, neutrophils and
lymphocytes. Louis, MO, 63110, USA John J Stephens Department of Radiation Oncology, Case
Western University School of Medicine, 11100 Euclid Avenue, Cleveland, OH, 44106-6068, USA
David B Mansur Department of Neurological Surgery, Washington University Medical School, 660
S. Some of the lesions had an overlying yellow crust, whereas others were petechial or. To link to
this page, Copy and Paste the following HTML code. Nowadays, there are no clearly established
protocols for the management of these patients. Childhood histiocytosis: A review of twenty two
cases. Controversies and new approaches to treatment of Langerhans cell histiocytosis. Hematol. The
use of the Internet for educational purposes is seen as the key to facilitating the learning process, the
formation of general and special competences of graduates, their ability to self-discipline,
responsibility, autonomy and leadership. All the biopsies received were routinely processed and
stained with routine H and E stain and S-100 immunostain. More recently, several large cooperative
studies have shown that multi-agent chemotherapy that is sustained over a longer. Of the 61 cases
reported, 24 (39.3%) also presented systemic involvement. Although a handful of cases involving
LCH limited to the hypothalamic region have been reported, this one represents only the second
example of recurrence after radiation therapy. Furthermore, long-term complications are not limited
to patients with multi-organ system disease. The benefits of reading books include exposing the
reader to new words directly from the read context and increasing your soft skills. The fact that the
patient was re-treated at the same institution using modern CT simulation techniques mitigated some
technical difficulties and allowed us to calculate composite dose distributions, thus enabling us to
ensure that critical structures were spared. Further characterization of the histiocytes showed strong
reactivity for S100, CD163, and CD1a (Figure 3 ), supporting the diagnosis of LCH. Figure 2. The
treatment is determined according to the severity, size of the lesions, and systemic involvement. In
our case, all other mucosa except genital mucosa were unaffected and there was no clinical evidence
suggestive of involvement of any other organ system. Reclassification Working Group of the
Histiocytic Society. Biopsy of lymph node showed two abnormal cell populations: distended sinus by
large, pale histiocytes with nuclear grooves, and the interfollicular region containing immature-
appearing cells with irregular nuclei slightly larger than that of small lymphocyte, dispersed
chromatin, inconspicuous nucleoli, scant cytoplasm, and scattered mitotic figures. The patient was
started on oral prednisolone 40 mg daily and methotrexate 15 mg once weekly. Report from the
International Registry of the Histiocytic Society. Contemporary Classification of Histiocytic
Disorders.
Also, allows quickly viewing any Flickr photo on black background in large size. The patient
understands that her name and initials will not be published and due efforts will be made to conceal
identity, but anonymity cannot be guaranteed. No atypical lymphocytes, no multinucleated giant
cells, no infectious organisms or viral inclusions, and only rare eosinophils were identified. Fibrosis
Portal and periportal fibrosis is evident in this hepatic Langerhans cell histiocytosis case as
highlighted by trichrome stain. All histology images were captured using the following equipment:
Olympus BX51 microscope, Olympus DP70 camera, and Olympus DP controller software. Flickriver
widget for iGoogle or Netvibes can display almost any Flickriver view - most interesting today, by
user, by group, by tag etc. It rarely presents as an isolated lesion of the hypophysis. Occurrence of
these two conditions in the same patient, particularly not simultaneously, is rare. The QUANTEC
(Quantitative Analyses of Normal Tissue Effects in the Clinic) data analysis on radiation-induced
toxicities was used to evaluate risk to critical structures based on the composite plan. The benefits of
reading books include exposing the reader to new words directly from the read context and
increasing your soft skills. The benefits of reading books include exposing the reader to new words
directly from the read context and increasing your soft skills. The second skin biopsy showed the
same features as the first one, compatible with Langerhans cell. Immunohistochemical staining
showed a mixed population of T-cells (Figure 2 C) and B-cells (Figure 2 D) with a normal 2:1 ratio
of kappa- and lambda-light chain expression (Figure 2 E and 2 F), consistent with a reactive non-
neoplastic infiltrate. Six years after the initial symptom onset, while still under the aforementioned
chemotherapy, the patient presented with intense headache. Authors’ original submitted files for
images Below are the links to the authors’ original submitted files for images. The patient, a 31-year-
old woman, leucoderma, presented with a more than 10-year history of pain in the lower limbs.
Controversies and new approaches to treatment of Langerhans cell histiocytosis. Hematol. The
information obtained in the review was organized through a data collection instrument that included
the following variables and outcomes: year of publication, study design, age, symptoms, history,
morphology of the lesions, histopathological examination, management, follow-up time after
treatment and presence of relapse (Flow chart). From 2014 to 2016, the patient underwent a rigorous
treatment protocol with 4 mg zoledronic acid (Zometa, Novartis, Brazil) administered parenterally.
Langerhans cell histiocytosis: Clinical presentation, pathogenesis, and treatment. In this study, we
present a case of LCH followed by Hodgkin lymphoma (HL). Patients who respond to chemotherapy
during the first 6 weeks. As chemotherapeutic agents commonly used to treat LCH (vinca alkaloids,
antimetabolites, and antifolates) are often associated with significant side effects, it was felt
chemotherapy would be reserved for salvage therapy when either surrounding normal tissues would
not tolerate re-irradiation or if systemic disease were to arise. At the 2-year followup, the patient was
asymptomatic, and the lesion showed complete resolution without recurrence. Due to the fact of its
heterogeneous presentation, it represents a diagnostic and therapeutic challenge. Expression of
Epstein-Barr virus in Langerhans cell histiocytosis. As a matter of fact, morphological identification
of LCH cells and positive immunochemical staining with CD1a and S100 are necessary for a
diagnosis, but the immunostainig with CD207 (Langerin) is essential for a definitive one. This
disease therefore requires a multidisciplinary approach including specialties such as gynecology,
dermatology, endocrinology, neurosurgery and oncology. Corresponding author: Dr. Chandra Sekhar
Sirka, Department of Dermatology and Venereology, AIIMS, Sijua, Patrapada, Bhubaneswar - 751
019, Odisha, India. Very rarely, such cells were binucleated and exhibited folds in the nuclear
membrane.
Nosology and pathology of Langerhans cell histiocytosis. Occurrence of these two conditions in the
same patient, particularly not simultaneously, is rare. Int J Radiat Oncol Biol Phys. 2010, 76 (3
Suppl): S28-S35. Article. Methodology: Immunohistochemistry was used to identify ET-1 and
proteins of interest in paired colonic tissue samples from 8 patients with CRC (normal and cancerous
tissue). Long-term morbidity and health related quality of life after multi-system. All histology
images were captured using the following equipment: Olympus BX51 microscope, Olympus DP70
camera, and Olympus DP controller software. He is referred to the pediatric oncologist for further
workup and possible biopsy for a Langerhans cell histiocytosis. A final histopathological diagnosis of
LCH was established based on these findings. During this process, the medical community often
collectively assigns a label to a condition and consequently to those who would be said to have the
disorder. Historically, the nomenclature regarding LCH has been confusing because the disease had
been sub-categorized. The more you read, the more you expose yourself to new words that slowly
become a part of your usual repertoire. A 32 year-old woman, leucoderma, presented at an
orthopedician's clinic with complaints of pain in the lower limbs, with increased pain in cold weather.
We performed a systematic review of all articles in English or Spanish, published until September
2018, related to the diagnosis and treatment of Langerhans cell histiocytosis in vulva. In 2017, she
presented with pain in the lower abdominal region and underwent another imaging scan, which
revealed the presence of a soft tissue lesion. At the time of recurrence, the use of chemotherapy was
revisited. Reading helps your vocabulary According to researchers, children who regularly read
books at an early age develop large vocabularies in the long run. A Greasemonkey script that adds
Flickriver links to various Flickr photo pages - user photos, favorites, pools etc, allowing to quickly
open the corresponding Flickriver view. The vulvar biopsy confirmed Langerhans cell histiocytosis
with spreading to multiple organs, so it was decided to start chemotherapy, achieving the complete
elimination of all lesions. When you read, you gain information and knowledge and you have a
plethora of other benefits as well. Pediatr Blood Cancer 2007 48(5):555-560. PubMed 6. Schmitz L,
Favara BE. The histological differential diagnosis included: lymphoma, lymphocytic hypophysitis,
and LCH. Dari hasil perancangan sistem menggunakan methode tersebut menghasilkan aplikasi yang
dapat meningkatkan pela. Radiotherapy has been thought to be curative at the relatively low dose of
20Gy. All presented with superficial lymph node enlargement. However, with the advent of
immunohistochemical stains, the Histocyte Society proposed reclassification. The eosinophils were
seen diffusely scattered along with other inflammatory cells in three patients (cases nos. 1, 3 and 4),
while they were seen in clusters in two others (cases nos. 2 and 5). In addition, three patients (cases
nos 1, 4 and 5) showed the presence of multinucleate giant cells. For patients who have localized
bone lesions, curettage is generally sufficient for diagnosis as well as therapy, although. Biology of
Langerhans cells and Langerhans cell histiocytosis. After starting the medication, the patient was
referred to an orthopedician who primarily suggested a bone surgery for prosthesis placement to
stabilize the clinical picture; however, the patient did not consent to this surgery. Sandra Buitrago:
Searched the PUBMED, EMBASE, COCHRANE, LILACS and SCIELO databases and
organization through a data collection instrument.