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A 12-year-old girl suffering from this disease since birth is described. The clinical picture of the condition is peculiar: hyperemia and infiltration, vast areas of dry skin, marked exfoliation, involvement of large natural folds, erosions after opened vesicles.

Clinical, morphological, ultrastructural and immunological studies were performed in a case of congenital self-healing non-Langerhans cell histiocytosis. The patient showed several aspects that have not been published before: a large nodule in the vulvar region, vesiculobullous elements and pneumonia (asymptomatic). The relationship of the vesicles and pneumonia to the histiocytic disorder is not clear. Ultrastructurally, worm-like (comma-shaped) particles, dense bodies and Birbeck granules were not found. Histiocytes were Leu-6 negative, and S100 (partly), Leu M3 and HLA-DR positive. Positive reactions were also obtained with anti-lysozyme and non-specific esterase. Several aspects of this case and of others described previously are discussed.

We describe two children with extensive erosions and vesicles at birth that healed with reticulate scarring. These add to the three other cases reported previously. Our two patients also have extensive neurologic deficits, which may represent another feature of this syndrome. Possible causes are discussed.

Basal membranes are an ubiquitous component of all human organs and fulfil a large variety of functions. They separate epithelial from mesenchymal tissue and control the passage of substances, of inflammatory as well as tumor cells. They form the extracellular cytoskeleton, regulate growth processes, and play an important part in wound healing. Ultrastructurally, the lamina densa can be distinguished from the lamina lucida. Anchoring fibrils connect the basal membrane of the dermoepidermal junction with the underlying dermis, while anchoring plates connect it with the epidermal cells. During the last few years, various components of the basal membrane have been biochemically analyzed. Different proteins were characterized, and their corresponding morphological structure could be identified. The growing knowledge regarding the structure and composition of the dermo-epidermal junction has led to a better understanding of many diseases involving this structure, in particular inborn and acquired bullous diseases, but also that of the role of the basal membrane in metastasis and tumor invasions. The characterization of antigens in bullous diseases with circulating antibodies has led to exact diagnostic criteria concerning the often overlapping disorders and allows, e.g., the differentiation between bullous pemphigoid and epidermolysis bullosa acquisita.

A 39 years old female patient with congenital erythroderma is reported. The condition was considered as the non bullous type of Brocq's congenital ichthyosiform erythroderma until recently. The patient was admitted as she had generalized erythema, desquamation and oedema, pyrexia and oliguria. The scales were fine and small, quite different from those of lamellar ichthyosis. The patient developed a recidivant pustular eruption on palms, face and trunk. So the diagnosis was changed to congenital psoriasiform erythroderma and this was supported by histopathological examination of both scaling and pustular lesions. There was a remarkable improvement on amethopterin therapy. The case is discussed and the relevant litterature is reviewed.