De Quatrebarbes J, Cordel N, Bravard P, Lenormand B, Joly P
Clinique dermatologique, CHU Rouen.
Ann Dermatol Venereol. 2004 Jan;131(1 Pt 1):55-7. doi: 10.1016/s0151-9638(04)93543-0.
Melkersson Rosenthal's syndrome is a rare disease that classically combines: orofacial edema, peripheral facial paralysis and a plicated tongue. Miescher's cheilitis represents the monosymptomatic form of the disease. Its etiopathogenesis is unknown. We report 2 cases of Miescher's cheilitis during which the discovery of a monoclonal lymphocyte expansion raised the question of an eventual link between these two diseases.
CASE No 1. A 30 Year-old man, without medical past history, had been followed up for 3 Years for Miescher's cheilitis. The supplementary examinations permitted elimination of an infectious cause, Crohn's disease, sarcoidosis or a contact allergy. A serum monoclonal IgG kappa was discovered fortuitously. An X-ray of the skeleton and the myelogram were normal. There was no detectable monoclonal rearrangement of the genes of the blood or bone marrow T or B-cell lymphocyte receptor. In the absence of progression towards a malignant blood disease three Years later, we concluded in a benign monoclonal gammapathy. CASE No 2. A 36 Year-old Algerian man, without past medical history, had been followed-up for 8 Years for a granulomatous macrocheilitis. The search for Crohn's disease, sarcoidosis or a contact allergy was negative and the diagnosis of an incomplete Melkersson Rosenthal syndrome was retained. The blood count revealed persisting hyperlymphocytosis in the blood. The etiological search for a hyperlymphocytosis showed a monoclonal rearrangement of the T-cell lymphocyte receptor genes in the blood lymphocytes. The myelogram was normal.
Melkersson Rosenthal's syndrome is a rare granulomatous disease of the mucosa of the mouth. The etiopathogenesis of this affection is unknown and controversial, several case reports suggest that it could be a disease of immunological origin. A clonal T-cell lymphocyte population was revealed in the labial lesions of a 12 Year-old patient presenting with Melkersson Rosenthal's syndrome during a control visit, without the role of this lymphocyte population having been determined. We report two other cases associating blood lymphocyte proliferation and Melkersson Rosenthal' syndrome. This association is not necessarily fortuitous because of the rarity of the syndrome on the one hand and the uncommon nature of the detection of lymphocyte clones in young patients on the other. The presence of a clonal population can be interpreted in two manners: it can demonstrate chronic antigen stimulation, which with a super-antigenic effect leads to the expansion of a lymphocyte population making it detectable. The other hypothesis would be an increased secretion of cytokines by the lymphocyte clone provoking a granulomatous organization, as during granulomatous lymphomas.
梅尔克森 - 罗森塔尔综合征是一种罕见疾病，典型表现为口面部水肿、周围性面瘫和皱襞舌。米舍尔唇炎是该疾病的单症状形式。其病因发病机制尚不清楚。我们报告2例米舍尔唇炎病例，在此过程中发现单克隆淋巴细胞增殖引发了这两种疾病之间可能存在关联的问题。
病例1。一名30岁男性，无既往病史，因米舍尔唇炎已接受随访3年。进一步检查排除了感染性病因、克罗恩病、结节病或接触性过敏。偶然发现血清单克隆IgG κ。骨骼X线检查和脊髓造影正常。血液或骨髓T或B细胞淋巴细胞受体基因未检测到单克隆重排。3年后无向恶性血液病进展，我们诊断为良性单克隆丙种球蛋白病。病例2。一名36岁阿尔及利亚男性，无既往病史，因肉芽肿性巨唇炎已接受随访8年。克罗恩病、结节病或接触性过敏检查均为阴性，诊断为不完全性梅尔克森 - 罗森塔尔综合征。血常规显示血液中淋巴细胞持续增多。淋巴细胞增多症的病因检查显示血液淋巴细胞中T细胞淋巴细胞受体基因单克隆重排。脊髓造影正常。
梅尔克森 - 罗森塔尔综合征是一种罕见的口腔黏膜肉芽肿性疾病。这种疾病的病因发病机制尚不清楚且存在争议，一些病例报告表明它可能是一种免疫源性疾病。在一名12岁患有梅尔克森 - 罗森塔尔综合征的患者复诊时，其唇部病变中发现了克隆性T细胞淋巴细胞群，但该淋巴细胞群的作用尚未确定。我们报告另外两例血液淋巴细胞增殖与梅尔克森 - 罗森塔尔综合征相关的病例。这种关联不一定是偶然的，一方面是因为该综合征罕见，另一方面是因为在年轻患者中检测到淋巴细胞克隆的情况不常见。克隆性群体的存在可以有两种解释：它可以证明慢性抗原刺激，这种刺激具有超抗原效应，导致淋巴细胞群扩张从而可被检测到。另一种假设是淋巴细胞克隆分泌细胞因子增加，引发肉芽肿形成，如同肉芽肿性淋巴瘤。
