Progressive symmetric erythrokeratoderma (PSEK) is a rare genetic skin disease, characterized by fixed erythematous and hyperkeratotic plaques, symmetrically distributed over the extremities. The first description of the disease was made by Darier in 1911, though since then there have been few related publications. The authors report the case of a 9-year-old girl with PSEK, presenting localized lesions over elbows and knees, of typical aspect. The histologic findings were nonspecific and topical treatment unsatisfactory. The description of this case intends to correlate the clinical and histologic findings, as well as the clinical course of the patient, with the current knowledge regarding this disease.
Keratosis; Skin diseases, genetic
