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

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

*المؤلف المقابل لهذا العمل

نتاج البحث: المساهمة في مجلةمراجعة النظراء

ملخص

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.

اللغة الأصليةEnglish
رقم المقال13
دوريةOnline Journal of Health and Allied Sciences
مستوى الصوت16
رقم الإصدار2
حالة النشرPublished - 2017

ASJC Scopus subject areas

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