Abstract
Progressive symmetric erythrokeratoderma (PSEK) is an autosomal dominant genodermatosis with incomplete penetrance and variable expressivity. It belongs to the group of erythrokeratodermas where it can be differentiated from erythrokeratoderma variabilis in the absence of migratory erythematous lesions and in a greater incidence of palmoplantar keratoderma. Molecular basis of PSEK has not yet been established although there are reports of mutations in the loricrin gene. We report a 13-year-old boy with symmetrically distributed hyperkeratotic plaques over the dorsum of the hands and the extensor aspect of the forearms, elbows and knees. As far as we are aware, we report the first case of PSEK in the Dominican Republic.
Highlights
Progressive symmetric erythrokeratoderma (PSEK)was first described by Darier in 1911 in his article entitled “Erythroqueratodermie Verruqueuse en Nappes, Symetrique et Progressive” [1]
As far as we are aware we report the first case of PSEK in the Dominican Republic
Skin biopsies were taken from the lesions near the elbows showing epithelial hyperplasia with hyperkeratosis, regular acanthosis, elongation and anastomosis of rete ridges, pigmentation of the basal layer and perivascular lymphohistiocytic infiltrate (Fig. 4) Based on the clinical and hystopathological findings, the patient was diagnosed with progressive symmetric erythrokeratoderma
Summary
Was first described by Darier in 1911 in his article entitled “Erythroqueratodermie Verruqueuse en Nappes, Symetrique et Progressive” [1]. PSEK describes an autosomal dominant mode of inheritance with incomplete penetrance and variable expressivity It usually develops during early childhood [3] as fixed and slowly progressive erythematous and hyperkeratotic plaques distributed symmetrically over the trunk, knees, elbows, dorsal surfaces of the hands and feet, and sometimes affecting the face, palms and soles [4,5]. Case Report A 13-year-old male that presented to our institution with the onset of symmetrically distributed hyperkeratotic plaques over the dorsum of the hands and the extensor aspect of the forearms, elbows and knees, from the age of 3, asymptomatic. His past medical history was not relevant, and there were no skin complains in any other family members. Skin biopsies were taken from the lesions near the elbows showing epithelial hyperplasia with hyperkeratosis, regular acanthosis, elongation and anastomosis of rete ridges, pigmentation of the basal layer and perivascular lymphohistiocytic infiltrate (Fig. 4) Based on the clinical and hystopathological findings, the patient was diagnosed with progressive symmetric erythrokeratoderma
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