Clinico-pathological analysis of autoimmune blistering disorders: experience in the central province of Sri Lanka

The knowledge about autoimmune blistering disorders has expanded significantly in the recent past. The management of this group of disorders has become easier with the new diagnostic methods and new treatment modalities which have emerged during the last decade. The studies on autoimmune blistering disorders are very sparse in Sri Lanka. The aim of this study was to analyze the clinicopathological profiles of patients with autoimmune disorders who attended the Dermatology unit, general hospital, Kandy, over a period of 4 years from 1999 to 2003. This seems to be the largest sample of patients studied on the subject in Sri Lanka.

92 cases of autoimmune blistering diseases diagnosed on the basis of clinical, histological, and direct immunofluorescence (DIF) were included in the study. The commonest autoimmune blistering disease appeared to be pemphigus (P) (55.4%) with a mean age of 46 years. The disease was more common in females with a male: female ratio of 1:1.6. Bullous pemphigoid (BP) (25%) was the next common disease, with a mean age of 54% years. In BP, male to female ratio was almost equal. Linear Ig A disease (LAD) (14.1%) was the next common autoimmune disease observed in the study. LAD was the commonest autoimmune blistering disease observed among children. Dermatitis herpetiformis (DH), Epidermolysis bullosa acquisita (EBA), and cicatricial pemphigoid (CP) were rare. A single case each from the above diseases was observed in this 4-year study. There were 2 cases of bullous LE (BSLE) during the study period. There was no cases of Herpes gestationis seen during this period. The majority of pemphigus patients were controlled with moderate doses of systemic steroids when combined with immunosuppressive agents.