A 66-year-old male presented with a pruritic, 3×2 cmsized, erythematous brownish solitary plaque with crusted and verrucous surface on the extensor surface of the right thigh (). The lesion developed 6 to 7 years ago, and had been growing slowly. There was no palpable inguinal lymph node enlargement, and the patient had been otherwise healthy. Biopsy revealed epithelial strands extending down to the dermis, forming anastomoses (). Epithelial strands were composed of 2 areas: the upper area consisting of dysplastic cells with prominent nucleoli and abundant mitoses, and the lower area consisting of oval and round cells, and occasionally small luminal ducts (). Dysplastic cells comprised almost the entire epidermis but did not invade into the dermis (). There was a sharp contrast and demarcation between the upper dysplastic lesion and the benign adenomatous lesion immediately surrounding the dysplastic keratinocytes in the lowermost portion of the epidermis (). In immunohistochemical stains, epithelial membrane antigen (EMA) was positive in the dysplastic cells and weakly positive in the ductal structures in the adenomatous lower portion (). p63 was more intensely positive in the dysplastic cells than in the adenomatous cells (). Carcinoembryonic antigen (CEA) stain was weakly positive in the ductal structures in the adenomatous lower portion (). The lesion was diagnosed as a reactive ESFA associated with Bowen's disease and was completely excised with free margins of the tumor. Histopathology of excised tissues was also consistent with reactive ESFA associated with Bowen's disease ().
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 ().