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Case Reports
. 2013 Jan-Feb;88(1):109-12.
doi: 10.1590/s0365-05962013000100016.

Progressive symmetrical erythrokeratodermia -- case report

Bianca de Mello Guaraldi  1 , Thaís Jerez Jaime, Rafael de Mello Guaraldi, Daniel Fernandes Melo, Osvania Maris Nogueira, Nilton Rodrigues
Affiliations
Case Reports

Progressive symmetrical erythrokeratodermia -- case report

Bianca de Mello Guaraldi et al. An Bras Dermatol. 2013 Jan-Feb.

Abstract
in English, Portuguese

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.

Eritroqueratodermia simétrica progressiva é uma genodermatose rara de herança autossômica dominante, com penetrância variável, descrita por Darier em 1911. E caracterizada por placas eritematosas e queratósicas, bem delimitadas, simetricamente distribuídas ao longo das extremidades, nádegas e mais raramente face. Os autores relatam o caso de uma paciente de 55 anos apresentando lesões no dorso das mãos, coxins interfalangeanos, punhos, região inguinal e dorso dos pés. O caso demonstra raridade, diagnóstico tardio, exuberância clínica e acometimento familial.

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Conflict of interest statement

Financial Support: none

Conflict of Interests: none

Figures

FIGURE 1
FIGURE 1
Clinical aspect on the dorsum of the hands. Fixed and well-defined symmetrically distributed erythematous-scaly plaques
FIGURE 2
FIGURE 2
Clinical aspect of the elbows. Fixed and well-defined symmetrically distributed erythematous-scaly plaques
FIGURE 3
FIGURE 3
Clinical aspect on dorsum of the feet. Fixed and well-defined symmetrically distributed erythematousscaly plaques
FIGURE 4
FIGURE 4
Clinical aspect of the inguinal area. Erythematous plaques, with a brownish-colored halo delimiting the affected region
FIGURE 5
FIGURE 5
Histopathology stained by Hematoxilin Eosin. HE, 100x. An orthokeratotic hyperkeratosis in a basket-weave pattern. Presence of moderate acanthosis and focus of thickened granular layer
See this image and copyright information in PMC

References

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