Female patient, 25 years old, from the state of São Paulo.\nWhen she was ten months old, her mother who brought her to our clinic reporting\nthat the child had had absence of hair, eyelashes and eyebrows since birth. An\nanatomopathological examination performed on the scalp revealed alopecia. One\nyear later she presented desquamative lesions on her fingertips and dystrophic\nfingernails. At two years and six months of age, hyperkeratotic and desquamative\nplaques appeared on her palms.\nAt eight years of age the patient presented a clinical picture of total alopecia\nand nail dystrophy. Hyperkeratotic plaques, initially present on her palms,\ninvolved the soles of feet as well (). Besides, she presented a distal tapering of fingers, which at\nradiological examination showed reduction of soft tissues in distal extremities\nof fingers, acquiring a triangular configuration, and bone structures of normal\nappearance (). A biopsy was done\nwhich revealed absence of hair follicles, but sweat glands with normal features.\nThe patient did not present changes in sudoresis, dentition or hearing.\nWhen she was 14 years old, after being evaluated by a psychologist, emotional\ndamage was observed and depression secondary to the pathology was diagnosed.\nMoreover, learning difficulties were noticed and light mental retardation\ndiagnosed. At the time, the patient wore a hairpiece and presented remission of\npalmoplantar keratoderma after the introduction of topical keratolytics.\nPatient treatment continued with multidisciplinary follow-up (dermatology,\npsychology and genetics). The same patient has two children which were not\naffected by the syndrome and are healthy. The patient's parents are\nphenotypically normal with no consanguinity. Genetic study did not reveal\nsimilar cases in the family. Currently at 25 years of age, the patient is\nperiodically monitored and deals with her pathology in a very conscious way,\ncontinuing with the treatment for palmoplantar keratoderma ().
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 ().