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Case for diagnosis. Erythroderma as manifestation of hypereosinophilic syndrome

Hypereosinophilic syndrome is defined as persistent eosinophilia (>1500/µL for more than six months) associated with organ involvement, excluding secondary causes. It is a rare, potentially lethal disease that should be considered in cutaneous conditions associated with hypereosinophilia. We repo...

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Detalles Bibliográficos
Autores principales: Merlotto, Maira Renata, Cantadori, Lucas Oliveira, Sakabe, Delmo, Miot, Hélio Amante
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Sociedade Brasileira de Dermatologia 2018
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6001083/
https://www.ncbi.nlm.nih.gov/pubmed/29924226
http://dx.doi.org/10.1590/abd1806-4841.20187419
Descripción
Sumario:Hypereosinophilic syndrome is defined as persistent eosinophilia (>1500/µL for more than six months) associated with organ involvement, excluding secondary causes. It is a rare, potentially lethal disease that should be considered in cutaneous conditions associated with hypereosinophilia. We report a case of erythroderma as a manifestation of hypereosinophilic syndrome. A 36-year-old male with no comorbidities presented progressive erythroderma, pruritus, peripheral neuropathy, and eosinophilia in the previous seven months. No mutations were found in FIP1L1/PDGFRA. Patient experienced rapid remission in response to oral prednisone and hydroxyurea. Cutaneous manifestations may be the only evidence of hypereosinophilic syndrome. Genotyping excludes myeloproliferative disease, thereby orienting treatment and prognosis.