Neonatal and early infantile cutaneous langerhans cell histiocytosis: comparison of self-regressive and non-self-regressive forms.
OBJECTIVE
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.


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


METHODS
Referral center in pediatric dermatology.


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


METHODS
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.
Department of Pediatric Dermatology, Hôpital Necker-Enfants Malades, Assistance Publique-Hôpitaux de Paris, 149 Rue de Sèvres, 75015 Paris, France. maximebattistella@hotmail.com
Maxime
M
Battistella
Sylvie
S
Fraitag
Dominique Hamel
DH
Teillac
Nicole
N
Brousse
Yves
Y
de Prost
Christine
C
Bodemer
D000367:Age Factors; D015820:Cadherins / Q000378:metabolism; D015331:Cohort Studies; D005260:Female; D051858:Forkhead Transcription Factors / Q000378:metabolism; D006646:Histiocytosis, Langerhans-Cell / Q000276:immunology* / Q000378:metabolism / Q000473:pathology*; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D016753:Interleukin-10 / Q000378:metabolism; D053645:Interleukin-2 Receptor alpha Subunit / Q000378:metabolism; D019394:Ki-67 Antigen / Q000378:metabolism; D018655:Lymphocyte Count; D008297:Male; D012189:Retrospective Studies; D012871:Skin Diseases / Q000276:immunology* / Q000378:metabolism / Q000473:pathology*; D050378:T-Lymphocytes, Regulatory / Q000502:physiology
D015820:Cadherins; C418974:FOXP3 protein, human; D051858:Forkhead Transcription Factors; C508017:IL2RA protein, human; D053645:Interleukin-2 Receptor alpha Subunit; D019394:Ki-67 Antigen; D016753:Interleukin-10