Post-inflammatory elastolysis and cutis laxa (Marshall, Heyl & Weber, 1966) is a skin disease in African infants which appears to be comparatively common in at least two countries. Destruction of elastic tissue and atrophy are preceded by urticarial or by annular erythematous-popular lesions and result in severe disfigurement. The clinical features are intermediate between anetoderma (macular atrophy) and acquired cutis laxa, but sufficiently typical and characteristic to constitute a distinctive syndrome, which might represent an abnormal reaction to the bite of an arthropod.

A mother and her oldest son suffering from acrogeria are described; in the mother the disease was complicated by elastosis perforans serpiginosa. Microscopic and ultramicroscopic details are given.

SUMMARY In this report seven new eases showing unilateral systematized, linear or zosteriform distribution of lesions resembling Darier's disease, but without other findings suggestive of Darier's disease, are presented. It is suggested that the eruption represents an epidermal naevus for which the name acantholytic dyskeratotic epidermal naevus is proposed.

Four patients with the clinical picture of epidermolysis bullosa acquisita were investigated. Biopsies were taken from the involved and uninvolved areas of the skin and the immunohistochemical and microscopic changes were studied. Direct immunofluorescence showed deposition of IgG and C3/4 in a linear or norched pattern along the epidermal basement membrane in both the involved and the uninvolved skin. In addition IgA (3/4), IgM (1/4), C4 (3/4) and properdin (3/4) could be detected. Indirect immunofluorescence revealed the presence of circulating antibodies against inter alia the epithelial basement membrane zone in one patient. Routine electron microscopy showed that the blister was situated in the dermis leaving the basal lamina in the roof of the blister. With immunoelectron microscopy using peroxidase-labelled antibody the in vivo deposition of IgG was observed just beneath the basal lamina in the dermis of both the perilesional and the uninvolved skin. These observations show that epidermolysis bullosa acquisita is a distinct entity, in which autoimmune mechanisms might possibly play a role.

SUMMARY Immunoelectron microscopic studies in a patient with cicatricial pemphigoid revealed the deposition of IgG above the basal lamina. This location is distinctive for pemphigoid and differs from that found in epidermolysis bullosa acquisita.