Rogers R S
Department of Dermatology, Mayo Clinic, Rochester, Minnesota, USA.
Dermatol Clin. 1996 Apr;14(2):371-9. doi: 10.1016/s0733-8635(05)70363-6.
The Melkersson-Rosenthal syndrome is a rare disorder of unknown etiology characterized by a triad of recurrent orofacial swelling, relapsing facial paralysis, and fissured tongue. Exacerbations and recurrences are common. The orofacial swelling is characterized by fissured, reddish-brown, swollen, nonpruritic lips or firm edema of the face. The facial palsy is indistinguishable from Bell's palsy. The fissured tongue is seen in one third to one half of patients and, although the least common manifestation, its presence assists in diagnosis. The classic triad is not seen frequently in its complete form; therefore, diagnosis is difficult. This is particularly true because monosymptomatic and oligosymptomatic variants are seen more commonly. Cheilitis granulomatosa of Miescher is an example of a monosymptomatic variant of the Melkersson-Rosenthal syndrome. The histologic findings of noncaseating, sarcoidal granulomas support the diagnosis. These granulomas are not invariably present, and their absence does not exclude the diagnosis of the Melkersson-Rosenthal syndrome. Thus, the Melkersson-Rosenthal syndrome is a disease with elements of orofacial granulomatosis. Orofacial granulomatosis is a clinicopathologic entity describing oral lesions with noncaseating granulomas. The spectrum of this entity includes patients with oral Crohn's disease, patients with oral lesions who will develop typical bowel symptoms of Crohn's disease in the ensuing months to years, patients with tooth-associated infections, patients with sarcoidosis, and patients with food or contact allergies. The value of the clinicopathologic construct of orofacial granulomatosis is to provoke the careful search for provocative causes for the reactive symptom complex of the Melkersson-Rosenthal syndrome.
梅尔克森 - 罗森塔尔综合征是一种病因不明的罕见疾病，其特征为复发性口面部肿胀、复发性面瘫和沟纹舌三联征。病情加重和复发很常见。口面部肿胀的特征为嘴唇出现裂隙、红棕色、肿胀且无瘙痒感，或面部出现坚实性水肿。面瘫与贝尔面瘫难以区分。三分之一至一半的患者可见沟纹舌，虽然这是最不常见的表现，但其存在有助于诊断。完整形式的经典三联征并不常见；因此，诊断困难。尤其因为单症状和少症状变体更为常见，情况更是如此。米舍尔的唇炎肉芽肿是梅尔克森 - 罗森塔尔综合征单症状变体的一个例子。非干酪样、结节病样肉芽肿的组织学发现支持诊断。这些肉芽肿并非总是存在，其不存在也不能排除梅尔克森 - 罗森塔尔综合征的诊断。因此，梅尔克森 - 罗森塔尔综合征是一种具有口面部肉芽肿病成分的疾病。口面部肉芽肿病是一种临床病理实体，描述伴有非干酪样肉芽肿的口腔病变。该实体的范围包括患有口腔克罗恩病的患者、口腔病变患者（这些患者在接下来的数月至数年中将出现典型的克罗恩病肠道症状）、与牙齿相关感染的患者、结节病患者以及食物或接触性过敏患者。口面部肉芽肿病的临床病理结构的价值在于促使人们仔细寻找梅尔克森 - 罗森塔尔综合征反应性症状复合体的激发原因。
