Progressive symmetrical erythrokeratodermia - Case report*

Author:

Guaraldi Bianca de Mello1,Jaime Thaís Jerez1,Guaraldi Rafael de Mello2,Melo Daniel Fernandes3,Nogueira Osvania Maris4,Rodrigues Nilton5

Affiliation:

1. Naval Hospital Marcílio Dias (Hospital Naval Marcílio Dias - HNMD), Brazil

2. MD, Fundação Técnico Educacional Souza Marques (FTESM), Brazil

3. MD, Federal University of the State of Rio de Janeiro (Universidade Federal do Estado do Rio de Janeiro - UNIRIO) Graduate degree in Dermatology from the Federal University of the State of Rio de Janeiro (Universidade Federal do Estado do Rio de Janeiro - UNIRIO); Naval Hospital Marcílio Dias (Hospital Naval Marcílio Dias - HNMD), Brazil

4. Biomedical scientist and Biologist, Federal University of the State of Rio de Janeiro (Universidade Federal do Estado do Rio de Janeiro - UNIRIO); Naval Hospital Marcílio Dias (Hospital Naval Marcílio Dias - HNMD); Federal University of the State of Rio de Janeiro (Universidade Federal do Estado do Rio de Janeiro - HUGG-UNIRIO); Federal University of the State of Rio de Janeiro (Hospital Universitário Gaffrée e Guinle- Universidade Federal do Estado do Rio de Janeiro - HUGG-UNIRIO), Brazil

5. Naval Hospital Marcílio Dias (HNMD), Brazil

Abstract

Progressive symmetrical erythrokeratodermia is a rare autosomal dominant genodermatosis with variable penetrance described by Darier in 1911. It is characterized by erythematous and keratotic plaques, sharply defined and symmetrically distributed along the extremities, buttocks and, more rarely, on the face. We report a case of a 55-year-old patient with lesions on the dorsum of the hands, interphalangeal pads, wrists, groin and back feet. This case demonstrates a rare and late diagnosis, clinical profusion and presence of familiar involvement.

Publisher

FapUNIFESP (SciELO)

Subject

Dermatology

Reference9 articles.

1. Eritroqueratodermia simétrica progressiva;Nico MMS;An Bras Dermatol,1995

2. Eritroqueratodermia simétrica progressiva: relato de um caso esporádico e de surgimento tardio;Zanini M;Med Cutan Iber Lat Am,2003

3. Progressive symmetric erythrokeratodermia. Histological and ultrastructural study of patient before and after treatment with etretinate;Nazarro V;Arch Dermatol,1986

4. Progressive symmetric erythrokeratodermia;Rodriguez-Pichardo A;J Am Acad Dermatol,1998

5. Transgenic mice expressing a mutant form of loricrin reveal the molecular basis of the skin diseases, Vohwinkel syndrome and progressive symmetric erythrokeratoderma;Suga Y;J Cell Biol. 200

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