Langerhans cell histiocytosis—a case report

A 17-year-old male presented for dermatologic consultation with slightly elevated reddish papules covered by yellowish scales in the scalp for the last two years and reddish and indurated ulcers in the perineum lasting six months. Additional complaints included polyuria, polydipsia, delay in the de...

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Autores principales: Thiago Jeunon, Maria Auxiliadora Jeunon Sousa, Nilton Santos-Rodrigues, Raquel Lopes
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Lenguaje:EN
Publicado: Mattioli1885 2012
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Acceso en línea:https://doaj.org/article/2050214bd11049629cc3339574568635
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spelling oai:doaj.org-article:2050214bd11049629cc33395745686352021-11-17T08:33:17ZLangerhans cell histiocytosis—a case report10.5826/dpc.0201a042160-9381https://doaj.org/article/2050214bd11049629cc33395745686352012-01-01T00:00:00Zhttp://dpcj.org/index.php/dpc/article/view/996https://doaj.org/toc/2160-9381 A 17-year-old male presented for dermatologic consultation with slightly elevated reddish papules covered by yellowish scales in the scalp for the last two years and reddish and indurated ulcers in the perineum lasting six months. Additional complaints included polyuria, polydipsia, delay in the development of secondary sexual characteristics and hearing loss of the right ear secondary to a medium otitis. Lesions from scalp and perineum were sampled for histopathologic examination and revealed a dense cellular infiltrate made up of mononuclear cells with conspicuous eosinophilic cytoplasm and large cleaved vesicular nucleus, some of them with shapes resembling the format of a kidney and others reminiscent of coffee beans. Numerous intermingling eosinophils were present. The diagnosis of Langerhans cell histiocytosis was then rendered and confirmed by positive immunostaining of neoplastic cells for anti-CD1a and anti-S100 protein antibodies. The work-up revealed diabetes insipidus, hypogonadotropic hypogonadism, hiperprolactenemia, growing-hormone deficiency and thickness of the pituitary stalk. The patient was treated with prednisone and vinblastin based chemotherapy regimen for six months with complete remission, but presented recurrence of some lesions in the scalp, which were handled with topical mustard and corticosteroids. After chemotherapy, the endocrinologic disturbances were corrected with hormonal replacement therapy. The patient is currently in good health with a follow-up of five years. Thiago JeunonMaria Auxiliadora Jeunon SousaNilton Santos-RodriguesRaquel LopesMattioli1885articleLangerhans cell histiocytosisLangerhans celldermatologyhistopathologydiabetes insipidushypogonadotropic hypogonadismDermatologyRL1-803ENDermatology Practical & Conceptual (2012)
institution DOAJ
collection DOAJ
language EN
topic Langerhans cell histiocytosis
Langerhans cell
dermatology
histopathology
diabetes insipidus
hypogonadotropic hypogonadism
Dermatology
RL1-803
spellingShingle Langerhans cell histiocytosis
Langerhans cell
dermatology
histopathology
diabetes insipidus
hypogonadotropic hypogonadism
Dermatology
RL1-803
Thiago Jeunon
Maria Auxiliadora Jeunon Sousa
Nilton Santos-Rodrigues
Raquel Lopes
Langerhans cell histiocytosis—a case report
description A 17-year-old male presented for dermatologic consultation with slightly elevated reddish papules covered by yellowish scales in the scalp for the last two years and reddish and indurated ulcers in the perineum lasting six months. Additional complaints included polyuria, polydipsia, delay in the development of secondary sexual characteristics and hearing loss of the right ear secondary to a medium otitis. Lesions from scalp and perineum were sampled for histopathologic examination and revealed a dense cellular infiltrate made up of mononuclear cells with conspicuous eosinophilic cytoplasm and large cleaved vesicular nucleus, some of them with shapes resembling the format of a kidney and others reminiscent of coffee beans. Numerous intermingling eosinophils were present. The diagnosis of Langerhans cell histiocytosis was then rendered and confirmed by positive immunostaining of neoplastic cells for anti-CD1a and anti-S100 protein antibodies. The work-up revealed diabetes insipidus, hypogonadotropic hypogonadism, hiperprolactenemia, growing-hormone deficiency and thickness of the pituitary stalk. The patient was treated with prednisone and vinblastin based chemotherapy regimen for six months with complete remission, but presented recurrence of some lesions in the scalp, which were handled with topical mustard and corticosteroids. After chemotherapy, the endocrinologic disturbances were corrected with hormonal replacement therapy. The patient is currently in good health with a follow-up of five years.
format article
author Thiago Jeunon
Maria Auxiliadora Jeunon Sousa
Nilton Santos-Rodrigues
Raquel Lopes
author_facet Thiago Jeunon
Maria Auxiliadora Jeunon Sousa
Nilton Santos-Rodrigues
Raquel Lopes
author_sort Thiago Jeunon
title Langerhans cell histiocytosis—a case report
title_short Langerhans cell histiocytosis—a case report
title_full Langerhans cell histiocytosis—a case report
title_fullStr Langerhans cell histiocytosis—a case report
title_full_unstemmed Langerhans cell histiocytosis—a case report
title_sort langerhans cell histiocytosis—a case report
publisher Mattioli1885
publishDate 2012
url https://doaj.org/article/2050214bd11049629cc3339574568635
work_keys_str_mv AT thiagojeunon langerhanscellhistiocytosisacasereport
AT mariaauxiliadorajeunonsousa langerhanscellhistiocytosisacasereport
AT niltonsantosrodrigues langerhanscellhistiocytosisacasereport
AT raquellopes langerhanscellhistiocytosisacasereport
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