Neonatal and Early Infantile Cutaneous Langerhans Cell Histiocytosis

Comparison of Self-regressive and Non–Self-regressive Forms

Maxime Battistella, MD; Sylvie Fraitag, MD; Dominique Hamel Teillac, MD; Nicole Brousse, MD, PhD; Yves de Prost, MD, PhD; Christine Bodemer, MD, PhD 

Arch Dermatol. 2010;146(2):149-156.

Objectives  To describe clinical and immunohistochemical findings in patients with cutaneous Langerhans cell histiocytosis (LCH) beginning in the first 3 months of life and to define predictors of disease evolution.

Design  Observational retrospective survey from July 15, 1989, to April 30, 2007.

Setting  Referral center in pediatric dermatology.

Patients  Thirty-one patients with a diagnosis of cutaneous LCH in the first 3 months of life and no previous visceral LCH.

Main Outcome Measures  Cutaneous lesion characteristics, regulatory T-lymphocyte density, and E-cadherin expression were assessed. Data were compared between the patient groups with self-regressive vs non–self-regressive forms of cutaneous LCH. Pathologic analysis was performed blinded to patient group.

Results  Self-regressive cutaneous LCH was found in 21 patients and non–self-regressive cutaneous LCH in 10 patients. Monolesional forms, necrotic lesions, hypopigmented macules at presentation, and distal topography of limb lesions were seen only in patients with self-regressive cutaneous LCH. Regulatory T-lymphocyte density correlated with interleukin 10 expression in lesions (r = 0.77, P = .003) but was not predictive of disease evolution. E-cadherin expression by Langerhans cells was found in 7 patients with disease limited to the skin whether self-regressive or not. One patient with secondary disseminated disease showed loss of E-cadherin expression in Langerhans cells.

Conclusions  Some morphologic traits of skin lesions can orient the diagnosis to a self-regressive form of cutaneous LCH. Regulatory T-lymphocyte density does not seem to be predictive of disease evolution. E-cadherin expression seems to be an indicator of limited skin disease but not of disease regression. Additional immunohistochemical study is required to confirm these data.