[Intense hemosiderin deposits in a case of self-healing congenital histiocytosis]. / Dépôts hémosidériniques importants au cours d'une histiocytose congénitale auto-involutive.

ABSTRACT

BACKGROUND: Congenital self healing histiocytosis, described for the first time in 1973 by Hashimoto and Pritzker, is characterized by multiple violaceous brown cutaneous papulonodules present at birth and disappearing spontaneously in 2 to 4 months. There are classically no systemic manifestations and the prognosis is always good. We present a case, particular by the intensity of hemosiderinic deposits. CASE-REPORT The patient was a 2-month-old female infant. Physical examination at birth revealed a healthy-appearing full-term newborn, with five skin lesions firm violaceous-reddish pigmented papules and nodules distributed over the head (scalp and forehead), right ear, right forearm and the flexural aspect of the left thigh. There was no hepatosplenomegaly or lymphadenopathy, and the patient's general condition was excellent. Histologic examination confirm the diagnosis by showing a dense polymorphous infiltrate composed of histiocytes associated with giant cells and erythrocytes extravasation. Perls stain was strongly positive. Staining with S100 Protein was positive. Outcome was good with involution of all lesions with atrophic and pigmented scars. DISCUSSION: The patient's excellent general condition, the absence of visceral locations and the spontaneous self healing allows us to classify this form as a benign self healing congenital histiocytosis. This patient presented intensely hemosiderinic deposits giving the lesions a strongly pigmented aspect.