Polycythemia vera revealed by Wells syndrome

BACKGROUND: Wells syndrome is an eosinophilic dermatosis that is mainly reported in association with infections or insect bites, and more rarely during haematological disorders. CASE REPORT: A 32 year-old woman presented several recurrent episodes of massive swelling and erythema papulo-nodular inflammatory plaques on the buttocks that spontaneously resolved. The biopsy revealed marked infiltration of the dermis with eosinophils suggesting Wells syndrome. Examination found an isolated hepatosplenomegaly with true polycythemia and a myeloproliferative disorder. The diagnosis of Vaquez disease was made. DISCUSSION: Wells syndrome is a distinctive disease entity with a wide polymorphism of clinical and histological features, unspecific, and varying depending on the age of the lesions. Various triggering factors are involved, but this syndrome may also reveal hematological disorders. Cutaneous manifestations often occur before the hematological diagnosis, but are frequently misdiagnosed. Only two other cases of Wells syndrome associated with Vaquez disease have been reported. Our case report underlined the importance of systematic research for an hemopathy in Wells syndrome, especially in young patients.