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Notes: A boy with an ectodermal dysplasia is presented, together with clinical and laboratory findings. Description of members of the family is included and comparison made with other ectodermal dysplasia syndromes now known to exist. This family closely resembles the recently delineated ‘tooth and nail’ syndrome.

Notes: Three possible mechanisms of the hair shaft abnormality in cheveux incoiffables have been investigated in nine patients. Cheveux incoiffables hairs were found to exhibit a normal distribution of cystine-rich protein within both the cuticle and the cortex, as determined by silver methenamine ultrastructural cytochemistry. Resistance to stretching, at 65% relative humidity and constant temperature, was similar to normal controls. Resistance to bending forces was also assessed, and was equivalent in both groups. On scanning electron microscopy, all patients with cheveux incoiffables demonstrated longitudinal grooving of the hair shaft, and the hairs were triangular or heart-shaped in cross-section. In addition, there appeared to be minimal cuticular weathering. Even hairs of 20–30 cm length showed only minimal cuticular and cortical weathering, compared with normal hair shafts of similar length and diameter. It is possible that the reduced progressive weathering renders the hair shaft more rigid, and could serve to explain the ‘stand on end’ appearance of the hair which is typical of this condition. However, the characteristic cross-sectional shape of the hair shaft in cheveux incoiffables may render it more rigid and resistant to bending forces. This latter theory has yet to be fully investigated.

Notes: The anatomy of the epidermis, dermis and subcutaneous tissues of the nail apparatus is distinct from that of non-appendageal skin. Apart from the demonstration of the longitudinal configuration of the dermal-epidermal junction of the nail bed, there have been no studies of the composition of the basement membrane zone of the nail apparatus. We obtained three human accessory digits, including one thumb, all of which had been amputated for cosmetic reasons, and were without known pathology. Specimens were stained with a battery of monoclonal and polyclonal antibodies which target normal basement membrane zone antigens, and studied by indirect immunoflourescence.This study demonstrated that the four distinct regions of the nail, namely the proximal nail fold, the nail matrix, the nail bed and the hyponychium expressed all the target antigens found in the normal non-appendageal basement membrane. In particular, there was normal expression of the epidermal-associated antigens, the 220-and 180-kDa bullous pemphigoid antigens, and the α6β4 integrin. There was also normal expression of the lamina lucida antigens LH39, GB3 and laminin. It is of interest that the dermal-associated components, namely the 285-kDa linear IgA antigen, the extracellular matrix glycoproteins chondroitin sulphate, type VII collagen and its closely associated proteins, and the poorly characterized antigen for LH24 and LH39 were all normally expressed.Fibronectin, which is not a normal basement membrane zone component, was diffusely expressed in the extracellular matrix, but did not accentuate the basement membrane zone. LHF2, an antibody raised against tenascin, which shows weak positive staining at the tips of dermal papillae in normal skin (unpublished data) diffusely stained the extracellular matrix and the tips of the dermal papillae of the nail matrix and hyponychium, but did not stain the basement membrane of the nail bed.These findings show that despite the numerous distinct features of epidermis, dermis and subcutis of the nail apparatus, the basement membrane zone has virtually identical antigen expression to that of the dermal-epidermal junction elsewhere. The difference in LHF2 expression in the nail bed most likely reflects the different shape of the nail bed basement membrane, which is arranged in longitudinal folds.

Notes: We have studied 10 cases of scarring alopecia, and investigated tbe diagnostic reliability of immunofluorescence and bistopatbology in licben planopilaris and pseudopelade. In tbe ligbt of our findings, we discuss tbe possible patbomechanisms of botb disease processes.

Notes: The pathogenesis of Darier's disease was investigated by immunohistochemical staining of skin biopsies from involved and uninvolved skin in 14 patients, using monoclonal antibodies specific for keratins expressed in simple epithelia, stratified squamous epithelia and during skin specific differentiation as well as keratins expressed in mucosa and some benign epidermal hyperproliferative states.Uninvolved perilesional skin from Darier's patients showed a normal profile of keratin expression, whereas in lesional skin there was apparent delay in the expression of the suprabasal skin specific keratins. Suprabasal keratins were not detected in basal cells, thus there was no true premature keratinization. The presence of hyperproliferative keratins was restricted to suprabasal cells in lesional skin. Four patients were receiving treatment with ctretinate at the time of biopsy, but results in these patients did not differ from patients using topical treatments. Etretinate did not influence the profile of keratin expression in uninvolved or involved skin. The expression of type VII collagen was examined and was normal throughout uninvolved and iL-sHiiitil skin in Darier's disease.

Notes: A case is reported of cutaneous T-cell lymphoma in an elderly woman. Dystrophy of all 20 nails was caused by a lymphomatous infiltrate of the nail apparatus.

Notes: Using a radiation source from which the output is maximal in the near infra-red range, we have successfully treated thirty-six tattoos in twenty-one patients. This technique is described and its place in practice is discussed.

Notes: In order to examine whether anti-androgen therapy was effective in hidradenitis suppurativa (HS), ethinyloestradiol 50μg/cyproterone acetate 50 mg in a reverse sequential regimen was compared with ethinyloestradiol 50μg/norgestrel 500μg (Eugynon 50) in 24 female patients. Both treatments produced substantial improvement in disease activity. Seven patients cleared and have remained free of disease for 18 months, five patients improved, four remained unchanged, while two deteriorated. Cyproterone acetate was not clinically significantly more effective than E50, and both gave a similar reduction in free androgen index. Anti-androgen therapy appears to be beneficial in the treatment of hidradenitis suppurativa.