Vulvar Lesions in an 8-Year-Old Girl: Cutaneous Manifestations of Multisystem Langerhans Cell Histiocytosis

Jorgensen, Eugéne C.; Chen, Peter P.; Cron, Julia

doi:10.1016/j.jpag.2017.03.065

Cited by 1 publication

(2 citation statements)

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“…The forms of presentation include pure genital lesions, subsequent multi-organ involvement, oral and cutaneous involvement with subsequent genital lesions, and diabetes insipidus with organic involvement [6]. When lesions are found in high-risk organs (bone marrow, lung, liver, spleen or central nervous system) the response to treatment decreases significantly [1]. The involvement of the pituitary gland can give rise to panhypopituitarism with important deficiencies of growth hormone, thyroid stimulating hormone, antidiuretic hormone and gonadotropins, this type of commitment is typically manifested with diabetes insipidus and amenorrhea.…”

Section: Case Reportmentioning

confidence: 99%

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Vulvar Biopsy as a Key for the Diagnosis of Langerhans Cell Histiocytosis with Multifocal Involvement: Case Report and Review of Literature

M¹,

Morante²,

Hernández³

et al. 2022

Int J Oncol Res

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Langerhans cell histiocytosis is a rare disease and due to its heterogeneous presentation represents a diagnostic and therapeutic challenge. We present the case of a patient with diabetes insipid us and panhypopituitarism, with the appearance of painful vulvar ulcers. 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.A systematic review of the literature was carried out in search of all articles in English or Spanish, published until September 2018, related to the diagnosis and treatment of Langerhans cells histiocytosis in the vulva. Two authors independently searched the PUBMED, EMBASE, COCHRANE, LILACS and SCIELO databases with the keywords "Vulva" AND "Langerhans cells Histiocytosis" OR "Vulva" AND "Histiocytosis X". The information obtained in the review was organized through a data collection instrument that included the following variables: year of publication, study design, age, symptoms, history, morphology of the lesions, histopathological examination, management, follow-up time after treatment and presence of relapse. A total of 131 articles were found with full text available. 55 articles met the eligibility criteria reporting a total of 61 cases. The main symptoms reported were pruritus, vulvar pain and dyspareunia. At the vulvar physical exam, the main findings were papules, nodules, vesicles, ulcers and erythematous plaques in the labia majora and minora. Nowadays, there are no clearly established protocols for the management of these patients.

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Section: Case Reportmentioning

confidence: 99%

“…Langerhans cell histiocytosis, also known as Histiocytosis X, is a disease characterized by clonal proliferation of myeloid-derived dendritic cells [1]. These cells reside in the skin and can migrate to the lymph nodes to present antigens to T and B lymphocytes [2].…”

Section: Introductionmentioning

confidence: 99%