LANGERHANS CELL HISTIOCYTOSIS

  • Fátima Satomi Nishimori Residente do terceiro ano de Dermatologia/Resident of the third year of Dermatology, Hospital Federal da Lagoa, Rio de Janeiro, Brasil
  • Gabriel Monteiro de Castro Chaves Residente do primeiro ano de Dermatologia/Resident of the first year of Dermatology, Instituto de Dermatologia Professor Rubem David Azulay, Rio de Janeiro, Brasil
  • Camila Nemoto de Mendonça Residente do terceiro ano de Dermatologia/Resident of the third year of Dermatology, Hospital Federal da Lagoa, Rio de Janeiro, Brasil
  • Priscila Mara Chaves e Silva Residente do segundo ano de Dermatologia/Resident of the second year of Dermatology, Hospital Federal da Lagoa, Rio de Janeiro, Brasil
  • Calisqui Bradenburg Fernandes Residente do primeiro ano de Endocrinologia/Resident of the first year of Endocrinology, Hospital Federal da Lagoa, Rio de Janeiro, Brasil
  • Leonardo Vieira Neto Professor do Departamento de Clínica Médica e Serviço de Endocrinologia – Faculdade de Medicina e Hospital Universitário Clementino Fraga filho – Universidade Federal do Rio de Janeiro, Brasil/Professor of the Department of Internal Medicine and Endocrine Unit – Medical School and Clementino Fraga Filho University Hospital – Federal University of Rio de Janeiro. Endocrinologista do Serviço de Endocrinologia do Hospital Federal da Lagoa/ Endocrinologist of Endocrine Unit – Hospital Federal da Lagoa, Rio de Janeiro, Brasil
  • João Carlos Regazzi Avelleira Especialista em Dermatologia pela Sociedade Brasileira de Dermatologia/Specialist in Dermatology; Chefe do Serviço de Dermatologia/Head of the Dermatology Department, Hospital Federal da Lagoa, Rio de Janeiro, Brasil
Keywords: Histiocytosis, Langerhans cell

Abstract

The authors report a case of a young male patient who presented with erythematous and descamative lesions, with maceration, in the axillary and inguinal region, associated with erythematous ulcerated plaques in the perianal region. Histopathological examination was compatible with Langerhans cell histiocytosis, with immunohistochemical positivity for CD1a and S100, thus confirming the diagnosis. Systemic investigation revealed an extra-axial mass in supra-sellar region; he underwent chemotherapy, with a significant improvement of the cutaneous lesions. He keeps his follow-up by the Dermatology, Hematology and Endocrinology Units.

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References

Donadieu J, Chalard F, Jeziorski E. Medical management of Langerhans cell Histiocytosis from diagnosis to treatment. Expert Opin Pharmacother. 2012; 13(9):1309-22.

Degar BA, Rollins BJ. Langerhans cell histiocytosis: malignancy or inflammatory disorder doing a great job of imitating one? Dis Model Mech. 2009(2): 436-9.

Badalian-Very G, Fleming M, Vergilio JA, Rollins BJ. Pathogenesis of Langerhans Cell Histiocytosis..Annu Rev Pathol. 2013; 8:1-20.

Minkov M, Grois N, McClain K, Nanduri K, Rodriguez-Galindo C, Simonitsch-Klupp I, et al. Langerhans Cell Histiocytosis: Histiocyte Society Evaluation and treatment guidelines. Boston:International Histiocyte Society; 2009.

Quattrino AL, Briggs MC, Silveira JC, Vilar E, Diniz C. Histiocitose de células de Langerhans: relato de caso e revisão da literatura. An Bras Dermatol. 2007; 82(4):337-41.

Decoster L, De Braekeleer K, Bourgain C, Schallier D. Langerhans Cell Histiocytosis: two case reports in adults and review of the literature. Acta Clin Belg. 2010: 65-75.

Gallo MS, Martínez MP, Abalovich MS, Gutiérrez S, Guitelman MA. Endocrine manifestations of Langerhans cell histiocytosis diagnosed in adults. Pituitary. 2010: 13(4):298-303.

Yuasa M, Fujiwara S, Oh I, Yamaguchi T, Fukushima N, Morimoto A, et al. Rapidly Progressing Fatal

Adult Multi-Organ Langerhans Cell Histiocytosis Complicated with Fatty Liver Disease. J Clin Exp Hematopathol.

; 52(2):121-6.

Park L, Schiltz C, Korman N. Langerhans Cell Histiocytosis. J Cutan Med Surg. 2012; 16(1):45-9.

Imashuku S, Kudo N, Kaneda S, Kuroda H, Shiwa T, Hiraiwa T ,et al. Treatment of patients with hypothalamic-pituitary lesions as adult-onset Langerhans cell Histiocytsis. Int J Hematol. 2011;94(6):556-60.

Published
2014-09-19
How to Cite
Nishimori, F. S., Chaves, G. M. de C., de Mendonça, C. N., Chaves e Silva, P. M., Fernandes, C. B., Neto, L. V., & Avelleira, J. C. R. (2014). LANGERHANS CELL HISTIOCYTOSIS. Journal of the Portuguese Society of Dermatology and Venereology, 72(2), 227-231. https://doi.org/10.29021/spdv.72.2.260
Section
Case Reports