Disseminated crusted papules in a newborn.

BACKGROUND: Congenital self-healing Langerhans cell histiocytosis (Hashimoto-Pritzker disease) is the rarest form of Langerhans cell histiocytosis, usually confined to the skin and/or mucous membranes. Cutaneous eruption is mostly generalized, papular, nodular or vesicular. Despite impressive clinical presentation in a newborn it infrequently spreads to internal organs (which then portends a grave prognosis, indistinguishable from Letterer-Siwe disease).

CASE REPORT: We presented a full-term newborn, female, 3.3 kg who had a multitude of erythematous and crusted papules, nodules and pseudovesicles distributed all over the body, except for the mucous membranes. A biopsy and haematoxylin--eosin stain revealed dermal infiltration of pleomorphic histiocytes with eosinophilic ground-glass cytoplasm and round to bean-shaped nuclei. Over the next six weeks the eruption gradually subsided leaving no residues, or a few atrophic scars.

CONCLUSION: There is no need for specific treatment of congenital self-healing Langerhans cell-histiocytosis in the absence of multiorgan involvement. However, a close and regular follow-up is necessary to evaluate the children for systemic symptoms and signs.