Pustaka Ilmiah Universitas Padjadjaran

Abstrak

Atypical Pemphigus Foliaceus with Clinical Manifestation Resembling Subcorneal Pustular Dermatosis

Introduction Pemphigus foliaceus (PF) is a chronic bullous autoithmune disease characterized by erosions, scales. and crusts on erythematous base, with initial lesion of flaccid bullae. Some atypical PF had been reported with clinical features of scales, crusts, erosions with pustules on erythematous base, Subcorneal pustular dermatosis (SCPD) is a chronic recurrent disease with manifestation of pustular eruption accompanied by half-half blisters. Case A case of pemphigus foliaceus with initial pustular lesions resembling SCPD in an 18-yearold girl was reported. The lesions presented as pustules, erythematous plaques, haemorrhagio crust, pustulous crust, and scales.. There were also half-half blisters. Patient was diagnosed as SCPD based on histopathological examination revealing subcorncal pustules and treated with dapson. However, due to absence of clinical improvement, the second biopsy was performed for a direct immunofluorescence (DIF) examination. Result of (DIF) examination revealed intercellular irnrnunoglobulin r deposit in the epidermis and review of histopatological examination supported pemphigus foliaceus then given 100 mg of cyclophosphmide daily, because there was history or no clinical improvement with systemic corticosteroids Significant clinical improvement was obtained at day 10th of cyclophosphamide therapy. Discussion Atypical PF in four patients was reported, which clinically mimicked closely those of SCPD, Histopathological examination showed subcorneal pustules in the subcorneal regions of the epidermis, DIP showed deposition of IgG on the keratinocyte cell surface, therefore the diagnosis SCPD can be excluded. Diagnosis of pemphigus was based on patient’s clinical features and histopathologic examination confirmed by imunolluoresens examination. For pemphigus that does not respond well to corticosteroids, adjuvant therapy can be an alternative treatment of choice.