UNLABELLED: Congenital 'self-healing' Langerhans' cell histiocytosis of Hashimoto-Pritzker is a rare disease occurring mainly in the neonatal period. CASE REPORT: We report on the case of a newborn with widespread eruption since birth, consisting of nodules, papulonodules, sometimes with ulcerations and scabs, concerning all the body, with a predilection for the cephalic area and the scalp, without general abnormalities. The clinical examination, histopathological data, immunohistochemistry, and the benign evolution in nine-, 18- and 24-month periods without particular treatment define the diagnosis of congenital self-healing Langerhans' cell histiocytosis of Hashimoto-Pritzker. CONCLUSION: The position of this disease among the Langherans' cell histiocytoses is probably situated at the benign pole. This is a benign self-healing disease restricted to the skin and the prognosis is good (self-involution). It is important to eliminate a malignant form of other Langerhans' cell histiocytosis such as Letterer-Siwe disease by a checkup searching for a visceral disease. The good prognosis should not lead to forget the possibility of error or forms of relapses; it is therefore imperative to have a rigorous, regular and especially long-term follow-up.
UNLABELLED: Congenital 'self-healing' Langerhans' cell histiocytosis of Hashimoto-Pritzker is a rare disease occurring mainly in the neonatal period. CASE REPORT: We report on the case of a newborn with widespread eruption since birth, consisting of nodules, papulonodules, sometimes with ulcerations and scabs, concerning all the body, with a predilection for the cephalic area and the scalp, without general abnormalities. The clinical examination, histopathological data, immunohistochemistry, and the benign evolution in nine-, 18- and 24-month periods without particular treatment define the diagnosis of congenital self-healing Langerhans' cell histiocytosis of Hashimoto-Pritzker. CONCLUSION: The position of this disease among the Langherans' cell histiocytoses is probably situated at the benign pole. This is a benign self-healing disease restricted to the skin and the prognosis is good (self-involution). It is important to eliminate a malignant form of other Langerhans' cell histiocytosis such as Letterer-Siwe disease by a checkup searching for a visceral disease. The good prognosis should not lead to forget the possibility of error or forms of relapses; it is therefore imperative to have a rigorous, regular and especially long-term follow-up.