THE OCCURRENCE OF IGA PEMPHIGUS-FOLIACEUS WITHOUT NEUTROPHILIC INFILTRATION

A 70-year-old woman with pemphigus foliaceus is reported. Direct immun ofluorescence performed on perilesional skin revealed deposits of IgA, C3 and lambda chains in the intercellular substance of the upper stra tum spinosum. Indirect immunofluorescence demonstrated serum antibodie s of the IgA class against the intercellular region of the upper epide rmis at an initial titre of 1 : 2560. Histological studies, performed together with immunofluorescence, revealed an absence of a distinct ne utrophil infiltrate either in the epidermis or dermis, contrary to the findings in cases with intra-epidermal IgA deposits reported previous ly. In recent years there have been several reports of dermatoses with intra-epidermal IgA deposits.(1-11) These observations differ, result ing in controversy about the classification of the disease and its aet iology.(12) Zillikens et al.(13) proposed a classification of these pa tients into three subgroups: (a) IgA pemphigus foliaceus showing inter cellular IgA deposits and subcorneal acantholysis and pustules; (b) Ig A pemphigus of the intra-epidermal neutrophil type showing intercellul ar IgA deposits and intra-epidermal pustules; (c) neutrophil dermatose s with subcorneal IgA deposits showing linear subcorneal IgA deposits and subcorneal pustules. In all such cases reported to date, a neutrop hil infiltrate was evident. We report a case of pemphigus foliaceus wi th intercellular IgA deposits and high titres of circulating IgA inter cellular antibodies with virtual absence of a distinct neutrophil infi ltrate either in the epidermis or dermis.