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).


METHODS
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.


CONCLUSIONS
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.
Pavlović
Milos D
MD
Military Medical Academy, Clinic of Dermatology, Center of Pathology and Forensic Medicine, Belgrade. mdpavlovic2004@yahoo.com
Minić
Aleksandra
A
Zolotarevski
Lidija
L
Vesić
Sonja
S
D005260:Female; D006646:Histiocytosis, Langerhans-Cell / Q000151:congenital* / Q000473:pathology*; D006801:Humans; D007231:Infant, Newborn; D012075:Remission, Spontaneous; D012871:Skin Diseases / Q000151:congenital* / Q000473:pathology*