Background, Congenital self healing histiocytosis, described for the first
time in 1973 by Hashimoto and Pritzker, is characterized by multiple violac
eous brown cutaneous papulonodules present at birth and disappearing sponta
neously in 2 to 4 months, There are classically no systemic manifestations
and the prognosis is always good. We present a case, particular by the inte
nsity of hemosiderinic deposits.
Case-report. The patient was a 2-month-old female infant. Physical examinat
ion at birth revealed a healthy-appearing full-term newborn, with live skin
lesions: firm violaceous-reddish pigmented papules and nodules distributed
over the head (scalp and forehead), right ear, right forearm and the flexu
ral aspect of the left thigh. There was no hepatosplenomegaly or lymphadeno
pathy, and the patient's general condition was excellent. Histologic examin
ation confirm the diagnosis by showing a dense polymorphous infiltrate comp
osed of histiocytes associated with giant cells and erythrocytes extravasat
ion. Perls stain was strongly positive. Staining with S100 Protein was posi
tive. Outcome was good with involution of all lesions with atrophic and pig
mented scan.
Discussion. The patient's excellent general condition, the absence of visce
ral locations and the spontaneous self healing allows us to classify this f
orm as a benign self healing congenital histiocytosis. This patient present
ed intensely hemosiderinic deposits giving the lesions a strongly pigmented
aspect.