Chronic Bullous Disease of Childhood in A Resource-Limited Setting: A Diagnostic Challenge

Abstract

Chronic Bullous Disease of Childhood (CBDC) is a rare autoimmune blistering disease that predominantly affects young children. Its diagnosis is often challenging because of clinical overlap with infectious and other autoimmune blistering disorders, particularly in resource-limited settings where direct immunofluorescence (DIF), the diagnostic gold standard, is unavailable. Objective: To describe the diagnostic approach and management of a pediatric patient with CBDC using clinicopathological correlation and systematic exclusion of differential diagnoses in the absence of DIF. This case report was based on comprehensive history taking, physical examination, medical record review, laboratory investigations, histopathological examination, treatment evaluation, and clinical follow-up of a 4 year old girl presenting with generalized blistering lesions. The clinical and laboratory findings were analyzed descriptively through clinicopathological correlation, systematic comparison with relevant differential diagnoses, and assessment of the patient's clinical response to therapy. The patient presented with a one-month history of generalized tense bullae involving the forehead and extremities, with some lesions demonstrating the characteristic cluster of jewels pattern. Histopathological examination revealed subepidermal and intraepidermal blister formation with neutrophilic and eosinophilic inflammatory infiltrates. Although DIF could not be performed because of limited institutional resources, the diagnosis was established through clinicopathological correlation, systematic exclusion of differential diagnoses, and a favorable response to dapsone therapy. Marked clinical improvement was observed within two weeks after dapsone. A structured clinicopathological approach, combined with careful exclusion of differential diagnoses and therapeutic response, can facilitate timely diagnosis and appropriate management of CBDC in resource-constrained settings where immunopathological confirmation is unavailable.