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Langerhans' Cell Histiocytosis in Infancy

Langerhans' cell histiocytosis (LCH) is a confounding disorder with a wide spectrum of outcomes ranging from spontaneous remission to metastasis and death. The disease commonly presents in infancy or early childhood, often with cutaneous lesions that mimic other conditions. The most straightforward way to establish the diagnosis is by skin biopsy; the diagnostic samples will show an atypical, CD1a-+ cellular infiltrate. Once the diagnosis is confirmed, it is difficult to provide anxious parents with meaningful prognostic information. Researchers from one of the largest pediatric dermatology centers in the U.S. performed a retrospective analysis of the cases of 19 infants with neonatal skin lesions who were eventually diagnosed with LCH.

Patients presented with (in order of frequency): vesiculopustules (crusting); seborrhea-like dermatitis; mucosal lesions (erosions, petechiae, and granulomas); erythematous papules; nodular, ulcerative lesions; and generalized petechiae. They underwent work-ups consisting of complete physical examination, blood counts, chemistry profile, coagulation studies, urine osmolality, and skeletal and chest X-rays. Additional studies (CT, ultrasound, or bone marrow biopsy) were obtained, if indicated. Extracutaneous disease was noted in 12 of 19 infants. Sites of involvement included bone, liver, spleen, lungs, GI tract, and lymph nodes. Four patients later developed diabetes insipidis. Two children died. Both had bone marrow, liver, and spleen involvement. Outcome could not be predicted based on skin lesion morphology.

Comment: This series supports a simpler but more ominous classification system for cutaneous LCH in infancy, because the concept of a separate, self-limited condition -- congenital, self-healing reticulohistiocytosis (a.k.a. Hashimoto-Pritzker disease) -- must now be abandoned. Although a subset of children experience spontaneous remission, prognosis without thorough evaluation is impossible and long-term follow-up is necessary.

— EC Siegfried

Published in Journal Watch Dermatology September 26, 2001

Citation(s):

Stein SL et al. Langerhans cell histiocytosis presenting in the neonatal period: A retrospective case series. Arch Pediatr Adolesc Med 2001 Jul 155 778-783.

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