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Uncombable hair syndrome (pili trianguli et canaliculi) is a rare genetic disease characterized by unruly hair, whose diagnosis could be made through an easy test such as hair cross-sectioning typically showing triangular and oval shapes.

Two native Yanomami children from the Venezuelan Amazonia with erythroderma were hospitalized on our service. Clinical, histologic, and immunofluorescence studies diagnosed endemic pemphigus foliaceous. Human leukocyte antigen class II showed DRB1*04 subtype *0411, which has not been previously associated with this disease. However, it shares a common epitope with all the human leukocyte antigen DRB1 alleles that have been involved in this disease among Brazilian populations. Although this condition is endemic in Brazil, our patients are the first two reported in Venezuela.

We describe an 11-year-old girl with a persistent pruritic papular eruption on the vulva. Clinically, the lesions consisted of whitish papules and erosions located on the inner aspect of the labia majora. There was no familial history of skin diseases. Histologically, a biopsy specimen showed difuse hyperkeratosis, parakeratosis, acantholysis throughout the thickness of the epidermis, and the presence of corps ronds. Those findings were consistent with a diagnosis of acantholytic dyskeratosis. At 3 years follow-up, only isolated hyperkeratotic, asymptomatic papules on the same location remained. The occurrence of this focal and sporadic, localized form of acantholytic dyskeratosis seems to be rare in the pediatric population, as we could find no other child with this entity reported in the literature.

We report a 10-month-old girl who, at 4 months, developed a small, reddish, plaquelike lesion on her tongue. This lesion began to enlarge rapidly, resulting in difficulty in swallowing and breathing. On physical examination, there was a large, red, friable, hard in consistency, irregular tumor over the distal portion of her tongue. The histopathology and immunohistochemical findings were consistent with an embryonal rhabdomyosarcoma. The patient was assigned clinical stage I, group III, and began treatment according to the Intergroup Rhabdomyosarcoma Study guidelines, on triple-agent chemotherapy consisting of vincristine, actinomycin D, and cyclophosphamide. She only received four cycles because her parents then refused this treatment. After the tumor size was reduced by chemotherapy, a partial anterior glossectomy was performed. After 30 months of follow-up she has had no recurrences.