Sporadic Progressive Symmetric Erythrokeratoderma: Classical Presentation of a rare Condition
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 le...
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Society of Dermatologists, Venereologists and Leprologists of Nepal (SODVELON)
2019-03-01
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| Series: | Nepal Journal of Dermatology, Venereology & Leprology |
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| Online Access: | https://www.nepjol.info/index.php/NJDVL/article/view/23293 |
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| _version_ | 1849227469962346496 |
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| author | Kompal Agarwal Sudha Agrawal Anju Pradhan |
| author_facet | Kompal Agarwal Sudha Agrawal Anju Pradhan |
| author_sort | Kompal Agarwal |
| collection | DOAJ |
| description |
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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| format | Article |
| id | doaj-art-e76b293ee6e4459f844fd9c5cf73fc7b |
| institution | Kabale University |
| issn | 2091-0231 2091-167X |
| language | English |
| publishDate | 2019-03-01 |
| publisher | Society of Dermatologists, Venereologists and Leprologists of Nepal (SODVELON) |
| record_format | Article |
| series | Nepal Journal of Dermatology, Venereology & Leprology |
| spelling | doaj-art-e76b293ee6e4459f844fd9c5cf73fc7b2025-08-23T10:04:30ZengSociety of Dermatologists, Venereologists and Leprologists of Nepal (SODVELON)Nepal Journal of Dermatology, Venereology & Leprology2091-02312091-167X2019-03-0117110.3126/njdvl.v17i1.23293Sporadic Progressive Symmetric Erythrokeratoderma: Classical Presentation of a rare ConditionKompal Agarwal0Sudha Agrawal1Anju Pradhan2BP Koirala Institute of Health SciencesBP Koirala Institute of Health Sciences, DharanBP Koirala Institute of Health Sciences, Dharan 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. https://www.nepjol.info/index.php/NJDVL/article/view/23293Genetic HeterogeneityKeratoderma, palmoplantarNepalRetinoids |
| spellingShingle | Kompal Agarwal Sudha Agrawal Anju Pradhan Sporadic Progressive Symmetric Erythrokeratoderma: Classical Presentation of a rare Condition Nepal Journal of Dermatology, Venereology & Leprology Genetic Heterogeneity Keratoderma, palmoplantar Nepal Retinoids |
| title | Sporadic Progressive Symmetric Erythrokeratoderma: Classical Presentation of a rare Condition |
| title_full | Sporadic Progressive Symmetric Erythrokeratoderma: Classical Presentation of a rare Condition |
| title_fullStr | Sporadic Progressive Symmetric Erythrokeratoderma: Classical Presentation of a rare Condition |
| title_full_unstemmed | Sporadic Progressive Symmetric Erythrokeratoderma: Classical Presentation of a rare Condition |
| title_short | Sporadic Progressive Symmetric Erythrokeratoderma: Classical Presentation of a rare Condition |
| title_sort | sporadic progressive symmetric erythrokeratoderma classical presentation of a rare condition |
| topic | Genetic Heterogeneity Keratoderma, palmoplantar Nepal Retinoids |
| url | https://www.nepjol.info/index.php/NJDVL/article/view/23293 |
| work_keys_str_mv | AT kompalagarwal sporadicprogressivesymmetricerythrokeratodermaclassicalpresentationofararecondition AT sudhaagrawal sporadicprogressivesymmetricerythrokeratodermaclassicalpresentationofararecondition AT anjupradhan sporadicprogressivesymmetricerythrokeratodermaclassicalpresentationofararecondition |