Open AccessCANDLE Syndrome: Case Report of a Rare Type of Auto- Inflammatory Disease
Author(s) -
Asif Ali,
Mohammad Imnul Islam,
Shahana A Rahman
Publication year - 2021
Publication title -
bangladesh journal of child health
Language(s) - English
Resource type - Journals
eISSN - 2408-8315
pISSN - 0257-3490
DOI - 10.3329/bjch.v44i3.52711
Subject(s) - failure to thrive , medicine , lipodystrophy , organomegaly , dermatology , skin biopsy , girl , biopsy , neutrophilic dermatosis , disease , pathology , pediatrics , pyoderma gangrenosum , immunology , human immunodeficiency virus (hiv) , viral load , antiretroviral therapy , psychology , developmental psychology
CANDLE syndrome (chronic atypical neutophilic dermatosis with lipodystrophy and elevated temperature) is an autoinflammatory disease/syndrome characterized by recurrent fever, skin lesions, and multisystem inflammatory manifestations. Most of the patients have shown mutation in PSMB8 gene. Here, we report a 9-year-old girl with recurrent fever, atypical facies, widespread skin lesions, generalized lymphadenopathy, hepato-splenomegaly, lipodystrophy, and failure to thrive. Considering the clinical features and laboratory investigations including skin biopsy findings, diagnosis was consistent with CANDLE syndrome. Therefore, it is recommended to consider CANDLE syndrome in a young child who presents with recurrent fever, characteristics rashes, organomegaly and failure to thrive.
Bangladesh J Child Health 2020; VOL 44 (3) :174-177