A RARE OFUJI PAPULO-ERYTHRODERMA REVEALING A LANGERHANS CELL HISTIOCYTOSIS INVOLVING ONLY LYMPH NODES

Ofuji Papulo-erythroderma is a rare entity. Its origin is most often paraneoplasic: visceral neoplasias and blood diseases are the most frequently found. We report a case of Ofuji papuloerythroderma revealing langerhans cell histiocytosis involving only lymph nodes.We report the case of a 48-year-old man who presented a pruriginous papuloerythroderma, respecting skin folds, having progressed for 8 years in a context of deterioration of the general state, associated with bilateral inguinal lymphadenopathies. The patient had already had several skin biopsies that were inconclusive. The biological assessment revealed an inflammatory syndrome and a discreet hyper-eosinophilia. Lymph node biopsy confirmed the diagnosis of Langerhans cell histiocytosis. Clinical and imaging studies did not reveal abnormalities in other organs, concluding to isolated lymph node involvement by Langerhans cell histiocytosis. The patient received chemotherapy by vinblastine and corticosteroids with very good clinical progress. Lymph node involvement by Langerhans cell histiocytosis is rarely described in the literature, it is most often located in the drainage area of a cutaneous or bone lesion, and it is rarely isolated. Ofujis papulo-erythroderma is a rare entity, most often paraneoplastic and exceptionally associated with Langerhans cell histiocytosis, which needs a very thorough etiological assessment to determine the underlying etiology.