Bilateral microphthalmia with blepharophimosis, linear lesions of dermal aplasia involving the face, and microcephaly were present in a newborn girl who died at age 9 months from cardiomyopathy resulting in ventricular fibrillation. Autopsy showed an atrial septum defect, persistent gross trabeculation of the left ventricle, and an arteria lusoria. This case represents a further example of a new entity for which we propose the term MIDAS syndrome. The acronym stands for microphthalmia, dermal aplasia, and sclerocornea. Our patient is the second with this syndrome to have a major congenital heart defect. Cytogenetic studies reported in previous cases indicate that the underlying gene defect can be assigned to Xp22.3. This new X-linked male-lethal trait should be distinguished from focal dermal hypoplasia that will be found to map elsewhere on the X-chromosome.

The expression of tenascin, a recently discovered extracellular matrix glycoprotein, was studied by immunohistochemistry in normal human skin and in a number of skin diseases with epidermal hyperproliferation such as psoriasis, basal cell carcinoma, Bowen's disease and solar keratosis. Tenascin expression in the upper dermis of normal skin was found to vary from almost absent to patchy along the basal membrane. Staining was continuous and intense around blood vessels, hair follicles and eccrine sweat ducts. In basal cell carcinoma a marked expression of tenascin was found in the tumour stroma, especially adjacent to the basal membrane surrounding the tumour cell nests. In Bowen's disease and solar keratosis, tenascin expression was found in the dermis next to the keratinocytes. In psoriasis the dermal papillae of clinically involved skin were intensely stained and a continuous band of tenascin was present in the upper dermis along the basal membrane. The distribution of tenascin differed from other known extracellular matrix components.

The new term Becker nevus syndrome is proposed for a phenotype characterized by the presence of a particular type of organoid epithelial nevus showing hyperpigmentation, increased hairiness and hamartomatous augmentation of smooth muscle fibers, and other developmental defects such as ipsilateral hypoplasia of breast and skeletal anomalies including scoliosis, spina bifida occulta, or ipsilateral hypoplasia of a limb. The present review includes 23 cases that can be categorized under this designation. The Becker nevus syndrome usually occurs sporadically. The associated anomalies tend to show a definite regional correspondence, suggesting a common origin from an early postzygotic mutation.

Objectives-To evaluate the usefulness of Gram staining and culture of skin lesions in patients with acute meningococcal infections.Design-Retrospective study.Setting-Community hospital and intensive care unit ofa teaching hospital.Subjects-51 patients admitted from 1989 to 1993 with proved meningococcal infections and microbiological examination of specimens from skin lesions.Interventions-Needle aspiration of a skin lesion before start of antibiotic treatment in 26 patients in the community hospital; punch biopsy of skin lesion after start ofantibiotic treatment in 25 patients in the teaching hospital.Main outcome measures-Detection of meningococci by Gram

Three cases are reported of hypogammaglobulinemic males with recurrent Campylobacter jejuni septicemia and erysipelas-like cellulitis without diarrhoea. In one patient Campylobacter jejuni grew from skin biopsy specimens. The findings in another patient were strongly suggestive of osteomyelitis caused by Campylobacter jejuni. Since the susceptibility of hypogammaglobulinemic patients to infection with Campylobacter jejuni is probably related to a lack of serum bactericidal activity against Campylobacter jejuni due to lack of IgM, two patients in whom previous antimicrobial treatment failed were treated with plasma infusions. This regimen supplemented with imipenem resulted in cure of these relapsing infections. Campylobacter jejuni septicemia must be considered in hypogammaglobulinemic patients who present with periodic fever and cellulitis.