[Parapemphigus and variants; clinical and immunological aspects]

The clinical, histopathological and immunological findings and therapeutic data of 29 patients with different variants of pemphigoid seen by us in the period 1981-1988 are summarized. All 29 patients: disseminated or bullous pemphigoid (20 cases), localized pemphigoid (3 cases), cicatricial pemphigoid (2 cases), juvenile pemphigoid (2 cases), papular pemphigoid (1 case) and pemphigus vegetans (1 case) showed as common denominators subepidermal blister formation and linear deposition of Ig (IgG) and complement in the basement membrane zone. In nearly all the cases with disseminated pemphigoid treatment with immunosuppressive drugs orally was indicated. With particular reference to the age of patients in this subgroup (mean age greater than 70 years) aspects inherent to the treatment are briefly discussed. In contrast to the (relatively) benign course of the disease in localized pemphigoid and in juvenile pemphigoid, cicatricial pemphigoid with mucosal localization appears to be a variant within the spectrum of pemphigoid with severe complications due to tissue retraction and loss of local function.