[Weber-Christian's disease: a preleukemic disorder?]

Autor: Saghir S; Département d'Hépato-gastroentérologie et de Nutrition-P III, Université Mohammed V, Rabat, Maroc., Meskini T; Département d'Hépato-gastroentérologie et de Nutrition-P III, Université Mohammed V, Rabat, Maroc., Ettair S; Département d'Hépato-gastroentérologie et de Nutrition-P III, Université Mohammed V, Rabat, Maroc., Erreimi N; Département d'Hépato-gastroentérologie et de Nutrition-P III, Université Mohammed V, Rabat, Maroc., Mouane N; Département d'Hépato-gastroentérologie et de Nutrition-P III, Université Mohammed V, Rabat, Maroc.
Jazyk: francouzština
Zdroj: The Pan African medical journal [Pan Afr Med J] 2019 Mar 18; Vol. 32, pp. 127. Date of Electronic Publication: 2019 Mar 18 (Print Publication: 2019).
DOI: 10.11604/pamj.2019.32.127.16106
Abstrakt: Weber christian's disease or idiopathic panniculitis is a rare condition characterized by an inflammation of the subcutaneous adipose tissue; it is a non-specific pathological condition that remains a central issue of debate and whose outcome is unpredictable. We here report the case of a 9-month-old female infant admitted in the hospital with sepsis preceded by respiratory symptoms and followed by the occurrence of small, hard, susceptible, asymmetrical erythematous subcutaneous nodes located in the arms and lower limbs. Cutaneous biopsy showed lobular hypodermitis with polymorphonuclear neutrophil infiltrations. The diagnosis of Weber Christian's disease was retained after eliminating other differential diagnoses. The patient was treated with prednisone with good outcome; during the follow-up period and during disease regression the diagnosis of B-cell acute lymphoblastic leukemia was made; this uncommon outcome has never been described in the literature. The aim of this study is to discuss any similar situation where another diagnosis was made in patients initially treated for idiopathic lobular panniculitis. This highlights the need for exhaustive etiological investigations and prolonged monitoring in order to search for a possible associated disorder.
Competing Interests: Les auteurs ne déclarent aucun conflit d'intérêts.
Databáze: MEDLINE
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