Congenital self-healing histiocytosis (Hashimoto-Pritzker)

Background Congenital self-healing Langerhans cell histiocytosis (CSHLCH) i s a rare condition, initially seen at birth or in the neonatal period, with generalized papules, vesicles, or nodules, Affected infants are otherwise well and the skin lesions tend to involute spontaneously within weeks to mo nths. Methods Twelve patients with CSHLCH were seen from 1989 to 1998. Results Eight patients were girls and four were boys and all presented with lesions at birth which disappeared 1-3 months later. The lesions consisted of numerous brownish-red papules, papulovesicles, crusts, and nodules dist ributed on the face, limbs, palms, and soles. Two patients had oral mucosal lesions, and one had ulcerated lesions that evolved leaving hypochromic ma cules, Light microscopy showed a histiocytic infiltrate in the papillary de rmis with epidermotrophism, Two cases were studied by electron microscopy: the Langerhans cells showed Birbeck granules and laminated corpus in their cytoplasm, Immunomarking with S100 protein was performed in all 12 patients and was positive. CD1 was also tested in four cases and was positive. Conclusions Because CSHLCH is a rare condition, we emphasize that, although it is usually a benign, self-limited entity, careful evaluation for system ic disease must be performed and long-term follow-up must be carried out to detect evidence of relapse or progression of the disease; this is essentia l when treating these patients.