Perianal ulcer and diabetes insipidus: A rare presentation of langerhans cell histiocytosis in an adult male

Ng Chong Beng, Nik Ritza Kosai Nik Mahmood, Lim Shyang Yee, Srijit Das, A. Subasri, Reynu Rajan

Research output: Contribution to journalArticle

Abstract

Langerhans cell histiocytosis (LCH), previously known as Histiocystosis X, is a rare idiopathic disease caused by a build-up of langerhan cells in the body. LCH has a heterogenous form of clinical presentation and presents with varying degree of severity. The severity often depends on the disease burden. LCH can manifest as a unifocal disease, multifocal-unisystem or multifocal-multisystem disease with the latter being more fatal than the rest. Risk organ involvement confers a grim prognosis. Perianal lesions are extremely rare skin manifestation of LCH with only few cases reported in the past. Vinca-alkaloids and steroid therapy have been shown to be effective in a large percentage of those presenting with LCH. This case report highlights the diagnosis and management of LCH presenting as a painful perianal ulcer in the background of newly diagnosed diabetes insipidus.

Original languageEnglish
Article number13
JournalOnline Journal of Health and Allied Sciences
Volume16
Issue number2
Publication statusPublished - 2017

Fingerprint

Langerhans Cell Histiocytosis
Diabetes Insipidus
Ulcer
Vinca Alkaloids
Skin Manifestations
Langerhans Cells
Rare Diseases
Steroids

Keywords

  • Anus
  • Diabetes insipidus
  • Endocrine
  • Histopathology
  • Langerhans cell
  • Pituitary
  • Skull
  • Ulcer

ASJC Scopus subject areas

  • Medicine(all)

Cite this

Perianal ulcer and diabetes insipidus : A rare presentation of langerhans cell histiocytosis in an adult male. / Beng, Ng Chong; Nik Mahmood, Nik Ritza Kosai; Yee, Lim Shyang; Das, Srijit; Subasri, A.; Rajan, Reynu.

In: Online Journal of Health and Allied Sciences, Vol. 16, No. 2, 13, 2017.

Research output: Contribution to journalArticle

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