Sisaipho: a new form of presentation of alopecia areata.
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Biomedical subjects
Publications and source records attributed to F M Camacho.
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Granulomatous slack skin is an uncommon cutaneous T-helper cell lymphoma closely related to mycosis fungoides. To the best of our knowledge this disease has not been previously described in children. We report on an 11-year-old boy who presented with painless slack skin masses in the neck, right axilla and arm, anterior wall of the abdomen, both inguinal regions, and the malleolar and dorsal aspects of the feet. The disease started 3 years earlier with erythematous lesions on the neck and wrists. Histologic examination of a specimen from the abdominal mass revealed an extensive lymphoid infiltrate with scattered multinucleated giant cells extending from the papillary dermis to the subcutis. The lymphoid cells showed the following immunophenotype: CD43+ (MT1), CD45+, CD45RO+, CD20-. The phenotype of the giant cells was lysozyme positive, CD68+ and Mac387-. The tumoral lymphoid cells had clonal rearrangement for the gene of the beta chain of the T-cell receptor (C beta TCR). The disease could be controlled with systemic glucocorticoids. Due to the presence of many histiocytes arranged in aggregates in the papillary and mid-dermis, this case was initially considered to be a cutaneous form of histiocytosis. We recommend deep and extensive biopsies in patients with slack skin disease.
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Pili bifurcati is an uncommon hair shaft dysplasia characterized by bifurcation of the hair shaft. The two characteristics that define the dysplasia are: 1. Each bifurcation produces two separate parallel branches which fuse again to form a single shaft. 2. Each branch of the successive bifurcations is covered with its own cuticle. Currently, there is confusion between the terms "pili bifurcati" and "pili gemini". The name "pili gemini" is used to define a kinetic papilla that splits at the upper end from single to double-tipped during the anagen phase and consequently the same follicular matrix produces two different-sized hair shafts having separate cuticles that emerge through a single pilary canal. Pili gemini maintains the double tipped papilla and consequently the hair shaft does not fuse again. Papillar tips that divide into several tips will produce several hair shafts, that characteristically do not fuse again. When the same papilla changes its shape repeatedly during the anagen phase, it can produce hair shafts with bifurcations at irregular intervals: pili multibifurcati. And, as it is also possible for one of the new papillae to split again in two, the hair shaft may be doubly bifurcated: pili bi, bifurcati. Pili bifurcati should be distinguished from acquired splitting of hair shafts that do not represent true bifurcations because the two split parts are never surrounded by a complete cuticle. Such acquired splitting may be called "central trichoptilosis".
We describe a new variant of congenital hypertrichosis, primary multifocal localized hypertrichosis in a mother and her son. They presented with long vellus hair on the trunk and arms in foci from birth. They also associated some forms of hair dysplasia.
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