Sporadic Progressive Symmetric Erythrokeratoderma: Classical Presentation of a rare Condition

  • Kompal Agarwal BP Koirala Institute of Health Sciences
  • Sudha Agrawal BP Koirala Institute of Health Sciences, Dharan
  • Anju Pradhan BP Koirala Institute of Health Sciences, Dharan
Keywords: Genetic Heterogeneity, Keratoderma, palmoplantar, Nepal, Retinoids

Abstract

Progressive symmetrical erythrokeratodermia (PSEK) is a rare autosomal dominant genodermatosis presenting in infancy or early childhood. An 11-year-old male presented with a history of pruritic, erythematous, scaly, hyperkeratotic plaques first noted at 5 years of age, with no history of similar lesions in the family. Cutaneous examination revealed multiple, irregularly shaped, erythematous plaques with fine, shiny white, adherent scaling distributed symmetrically over the face, trunk, inguinal area, bilateral axillae and extensor surfaces of limbs. Palmoplantar keratoderma was present with thickened, yellowish discolored nails. No systemic abnormality was found. The histopathological findings were consistent with PSEK. The case is being reported to increase the awareness about this rare disease.

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Author Biographies

Kompal Agarwal, BP Koirala Institute of Health Sciences

Junior Resident, Department of Dermatology, Venereology and Leprology

Sudha Agrawal, BP Koirala Institute of Health Sciences, Dharan

Professor, Department of Dermatology

Anju Pradhan, BP Koirala Institute of Health Sciences, Dharan

Additional Professor, Department of Pathology

Published
2019-03-31
How to Cite
Agarwal, K., Agrawal, S., & Pradhan, A. (2019). Sporadic Progressive Symmetric Erythrokeratoderma: Classical Presentation of a rare Condition. Nepal Journal of Dermatology, Venereology & Leprology, 17(1), 69-72. https://doi.org/10.3126/njdvl.v17i1.23293
Section
Case Reports