Background: The CHILD syndrome is characterized by an ichthyosiform and inflammatory nevus showing a strikingly unilateral arrangement. No particular name has so far been given to this nevus, which is why this skin disorder has been described under various inappropriate terms such as epidermal nevus, inflammatory linear verrucous epidermal nevus (ILVEN), unilateral ichthyosiform erythroderma, unilateral ichthyosis or verruciform xanthoma. Objective: In order to avoid such confusion and to make it easier for clinicians to recognize this skin disease, a new name should be given to this disorder, and the diagnostic criteria should be delineated. Method: The term CHILD nevus is proposed and the distinctive clinical, histopathological and ultrastructural features of this disorder are described. Results: A comprehensive clinical and genetic comparison shows that the CHILD nevus can be distinguished from all other types of epidermal nevi by characteristic features such as ptychotropism, waxy yellowish scaling, a unique lateralization pattern showing both diffuse and linear involvement and the presence of foamy histiocytes in the papillae (‘verruciform xanthoma’). Contrasting with all other epithelial nevi, the CHILD nevus is an inherited X-linked dominant, male-lethal trait. Conclusion: This nevus represents a separate cutaneous entity. Future clinical research will probably show that the underlying gene defect often manifests itself as an isolated skin disorder. Such cases should no longer be confused with ILVEN. Recognition of this particular skin disorder is important for genetic counseling because a woman showing an isolated CHILD nevus has an increased risk of giving birth to a daughter suffering from a complex congenital disorder, the CHILD syndrome.

The general ultrastructure of the Malassezia furfur cell envelope is shown in a schematic model. Distinct layers such as the outer lamella, the cell wall and the plasma membrane and special structural elements of the cell wall are described and presented in electronmicrographs. Special attention was paid to the outer lamellar layer that seems to be equivalent to a capsule. It probably contains lipids as well as the cell wall, and it seems to participate in attachment processes.

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A 38-year-old man presented with gingival inflammation together with erosions of the penis. Direct immunofluorescence demonstrated linear deposits of IgA at the basement membrane zone; indirect immunofluorescence and immunoblotting were negative. Linear IgA disease (LAD) was therefore suspected and treatment with dapsone initiated but this was changed to sulfamethoxy-pyridazine and systemic corticosteroids because of methaemoglobinaemia. During 1-year follow-up the lesions continued to wax and wane although they were never as extensive as before. Eighteen months after disease onset there was scarring of the penis together with suspected fibrosis of the inflamed gingival region. In addition the patient was HLA DQ7(3) positive, a haplotype thought to be increased in patients with cicatricial pemphigoid (CP); LAD with scarring or CP with solely linear IgA deposits are possible diagnoses of his condition.

In a case of granulomatous syphilis (transitional state between early and late syphilis), several Langerhans cells (LC) were present in the basal and medial part of the epidermis. Evidence of LC activation was given by the presence of many Langerhans granules, vesicles, a large Golgi apparatus and, sometimes, Golgi complexes. In some LC, signs of degeneration were visible. Langerhans granules surrounded by cytoplasmic material were present in the intercellular space of the epidermis. Apposition of lymphocytes to LC in the dermal space supports the role of LC in immunologic mechanisms. This was further confirmed by signs of phagocytosis in LC. The interaction of LC with intra-epidermal microorganisms (as, for instance, the Treponema pallidum) might be important in the development of the epidermotropic infiltration. This mainly consists of lymphocytes, which sometimes form a pseudolymphoma. A correlation between LC and granuloma formation is suggested.