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 presen...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 butoocks 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.展开更多
Background. "Agranular CD4+CD56+hematodermic neoplasm"are rare hematologic neoplasms which were recently shown to correspond to the plasmocytoid dendritic cells. Case report. A 83-year-old presented isolated...Background. "Agranular CD4+CD56+hematodermic neoplasm"are rare hematologic neoplasms which were recently shown to correspond to the plasmocytoid dendritic cells. Case report. A 83-year-old presented isolated skin lesions purple, infiltrating the dermis. The biopsy has shown a dense dermal infiltration with malignant cells CD4+CD56+CD43+. There were no bone marrow involvement and no circulating blood cells. A chemotherapy permitted a clinical remission after six courses. Unfortunately, skin and blood relapses appear four months later. After a short success of chemotherapy by DHAP, the patient died three month later. Discussion. "Agranular CD4+CD56+hematodermic neoplasm"is a distinct entity from the cutaneous primary lymphomas. Recently plasmocytoid monocyte cells have been identified as the precursor of the malignant population with the high expression of CD123, IL-3 receptor. It is a distinct clinicopathologic entity by its clinical presentation with skin tropism, bone marrow involvement with or without leukemic phase and poor prognosis independent of the kind of treatment and its particular phenotype CD4+CD56+CD43+. It would be interesting to use antibodies linked to CD123 in therapeutic because any treatment have efficacity in this disease.展开更多
文摘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 butoocks 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.
文摘Background. "Agranular CD4+CD56+hematodermic neoplasm"are rare hematologic neoplasms which were recently shown to correspond to the plasmocytoid dendritic cells. Case report. A 83-year-old presented isolated skin lesions purple, infiltrating the dermis. The biopsy has shown a dense dermal infiltration with malignant cells CD4+CD56+CD43+. There were no bone marrow involvement and no circulating blood cells. A chemotherapy permitted a clinical remission after six courses. Unfortunately, skin and blood relapses appear four months later. After a short success of chemotherapy by DHAP, the patient died three month later. Discussion. "Agranular CD4+CD56+hematodermic neoplasm"is a distinct entity from the cutaneous primary lymphomas. Recently plasmocytoid monocyte cells have been identified as the precursor of the malignant population with the high expression of CD123, IL-3 receptor. It is a distinct clinicopathologic entity by its clinical presentation with skin tropism, bone marrow involvement with or without leukemic phase and poor prognosis independent of the kind of treatment and its particular phenotype CD4+CD56+CD43+. It would be interesting to use antibodies linked to CD123 in therapeutic because any treatment have efficacity in this disease.
