Female patient, 41 years old, of mixed race, from Tremedal, Bahia, came to the\ndermatology ambulatory reporting that since birth she had not had hair anywhere in\nher body besides nail dystrophy and palmoplantar hyperkeratosis ( and ). She denied previous history of cutaneous neoplasms, dental\nabnormalities, intolerance to heat or other significant medical problems and also\ninformed that two of her siblings presented similar symptoms.\nShe reports that for 10 years coalescent papules and nodules of pinkish color,\nverrucous aspect appeared on her feet and hands, growing in number and size with\ntime. She denied pain, burning sensation or bleeding at the site of the cutaneous\nlesions.\nDuring physical examination the presence of coalescent pinkish papules was noticed,\nforming plaques with cobblestone aspect on the feet, besides alopecia and nail\ndystrophy. Laboratory exams were normal. The possible differential diagnoses of\ncutaneous lesions included diffuse eccrine poromatosis, plane warts, Clouston\nSyndrome, pachyonychia congenita and other forms of ectodermal dysplasias ().\nAn incisional biopsy of the right foot lesion was performed, which revealed thin\ncords of anastomosed epithelial cells in continuity with the epidermis. The cords\nwere composed of round and oval nucleated cells. Luminal structures which varied in\nsize were also found, whose walls contained a single layer of cells. These findings\nwere compatible with the diagnosis of ESFA (. PCR for HPV 10 was negative.
A 81-year-old white female patient had had a painful tumor at the lateral malleolus of\nthe right ankle for three years. Physical examination revealed an erythematous tumor\nwith a small central ulceration on the right lateral malleolus ( and ). The\nfollowing hypotheses were formulated: cutaneous annex tumor, fibrosarcoma, schwannoma,\ncutaneous lymphoma, basal cell carcinoma and squamous cell carcinoma. Next, a lesion\nbiopsy was performed.\nHistopathology showed epidermis-derived, intertwined epithelial cords, ending in thin\nprojections. Some of these projections had a typical 'crab claw' appearance. Between\nthese cords, a rich fibrovascular stroma is present (). Light formations of the eccrine ducts were identified within some\nof the cords ().\nThe patient was referred to the plastic surgery service, where surgical excision and\nreconstruction by a reverse-flow supramalleolar flap were done (). Three months postoperatively, the patient was completely\nrecovered ().