[Late onset of a chronic septic granulomatous disease]

Chronic septic granulomatosis is a disease characterized by an impaired bactericidal potential of the neutrophilic polynuclear. The cutaneous manifestations rarely reveal the disease, but are of considerable interest in the diagnosis, notably during the late onset forms. We report such a case.

A 15 year-old girl, born of consanguine parents, had a history of visceral leishmaniasis and hepatic hydatidosis. For the past 3 years she had developed dermatitis lesion on the face and skin folds, chronic folliculitis and suppurating axillary and inguinal lymphoadenitis. The absence of a reduction in tetrazolium nitro blue led to the diagnosis of chronic septic granulomatosis. Prophylactic treatment stabilized the cutaneous lesions.

Chronic septic granulomatosis regroups various severe and recurrent manifestations. Its transmission is usually X-linked recessive or, on rare occasions, autosomal recessive. The clinical manifestations leading to the diagnosis are often of very early onset. They are principally pneumonia due to apergillus fumigatus and lymphoadenitis. Cutaneous involvement, although less common, must not be neglected because it can lead to the diagnosis of late onset forms, as in our patient.