Progressive symmetric erythrokeratoderma: Report of a Chinese family

Abstract

A large pedigree of progressive symmetric erythrokeratoderma is reported. The proband was a 22-year-old male with generalized asymptomatic lesions characterized by symmetrical well-demarcated erythematous hyperkeratotic plaques mainly distributed on the extremities. The proband's parents were also affected, and they were first cousins. Thus, a case of familial progressive symmetric erythrokeratoderma is described.

Progressive symmetric erythrokeratoderma with nephrotic syndrome: Coincidence or new association?

“…Although some cases have been identified to have loricrin and few to have connexin mutations in PSEK, EKV, or both, recent reports show that the same gene mutations were not found in some pedigree of PSEK. [5]…”

Progressive symmetrical erythrokeratodermia - Case report*

“…It affects both sexes equally during the first months or years of their lives. 9 Genetic penetration, however, can be incomplete which generates a variable clinical expressivity. Spontaneous remission does not usually occur, although cases have been described in the literature.…”

Progressive Symmetric Erythrokeratoderma. First case reported in the Dominican Republic

“…www.odermatol.com Also, the symmetry of the lesions in PSEK is more striking than in EKV [5]. These overlapping characteristics have led some authors to propose the alternate term "EKV et progressiva" [7].…”

“…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 also the face, palms and soles [4,5]. Despite that molecular basis of PSEK has not yet been established, there are reports of mutations in the loricrin gene [4,6].…”