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A patient with atypical Nekam's disease is described. Although the histological appearances were lichenoid, the presence of porokeratosis and amyloid deposition, previously reported in this condition, argue against the view that Nekam's disease is a subset of lichen planus.
Five patients in the same family showed small keratotic plugs of the palmar creases. Transmission in lineage was consistent with an autosomal dominant pattern of inheritance. The syndrome was highly associated with ichthyosis vulgaris in several members of the family. The histopathology in two patients revealed a close relation between the plugs and the acrosyringium.
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BACKGROUND: Solar/actinic keratoses (AKS) are premalignant lesions, usually less than 1 cm in diameter, that appear on chronically sun-damaged skin. METHODS: We describe four patients with a form of AK that enlarged and recurred despite standard treatment. Histologic examination revealed a single and multilayered sheet of anaplastic cells along the undersurface of the epidermis extending down hair follicles and present over a large area. RESULTS: Three of the patients developed either a squamous cell carcinoma (SCC) or basal cell carcinoma (BCC) within the area encompassed by the AK. CONCLUSIONS: These cases represent an insidious, proliferative form of AK with an increased tendency to develop into skin cancer.
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