Battistella Maxime, Fraitag Sylvie, Teillac Dominique Hamel, Brousse Nicole, de Prost Yves, Bodemer Christine
Department of Pediatric Dermatology, Hôpital Necker-Enfants Malades, Assistance Publique-Hôpitaux de Paris, 149 Rue de Sèvres, 75015 Paris, France.
Arch Dermatol. 2010 Feb;146(2):149-56. doi: 10.1001/archdermatol.2009.360.
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.
Observational retrospective survey from July 15, 1989, to April 30, 2007.
Referral center in pediatric dermatology.
Thirty-one patients with a diagnosis of cutaneous LCH in the first 3 months of life and no previous visceral LCH.
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.
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.
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.
描述出生后前3个月起病的皮肤朗格汉斯细胞组织细胞增多症（LCH）患者的临床和免疫组化表现，并确定疾病演变的预测因素。
1989年7月15日至2007年4月30日的观察性回顾性调查。
儿科皮肤病转诊中心。
31例出生后前3个月诊断为皮肤LCH且既往无内脏LCH的患者。
评估皮肤病变特征、调节性T淋巴细胞密度和E-钙黏蛋白表达。比较皮肤LCH自回归型与非自回归型患者组的数据。病理分析在对患者组不知情的情况下进行。
21例患者为自回归型皮肤LCH，10例患者为非自回归型皮肤LCH。单病灶形式、坏死性病变、初发时色素减退斑以及肢体病变的远端分布仅见于自回归型皮肤LCH患者。调节性T淋巴细胞密度与病变中白细胞介素10表达相关（r = 0.77，P = 0.003），但不能预测疾病演变。7例疾病局限于皮肤的患者，无论是否为自回归型，其朗格汉斯细胞均有E-钙黏蛋白表达。1例继发播散性疾病的患者，其朗格汉斯细胞E-钙黏蛋白表达缺失。
皮肤病变的一些形态学特征可将诊断指向自回归型皮肤LCH。调节性T淋巴细胞密度似乎不能预测疾病演变。E-钙黏蛋白表达似乎是皮肤疾病局限的指标，但不是疾病消退的指标。需要更多的免疫组化研究来证实这些数据。
