Nofal Ahmad, Amer Hala, Alakad Rania, Nofal Eman, Desouky Fatma El, Yosef Ayman, Albalat Waleed, Gharib Khaled, Mostafa Inass, Fathy Sahar, Waked Monira, Ragheb Eman, Gammaz Hanan
Department of Dermatology, Faculty of Medicine, Zagazig University, Zagazig, Egypt.
Al-Haud Al-Marsoud Dermatology Hospital, Cairo, Egypt.
Int J Dermatol. 2017 Mar;56(3):284-290. doi: 10.1111/ijd.13437. Epub 2016 Sep 25.
Lichen myxedematosus (LM) is a rare, chronic idiopathic disorder characterized clinically by waxy, closely set papules and histopathologically by diffuse dermal mucin deposition and fibroblast proliferation. The most recent classification of LM was proposed in 2001; however, it seems to be complex, confusing, and imprecise. Herein, we present seven cases of LM to evaluate the validity of the current classification, to propose new diagnostic criteria and classification, and to suggest a clinically relevant severity grading system for this rare disorder.
The study included seven patients with different presentations and severities of LM. All patients were subjected to thorough dermatological and systemic examination, routine laboratory tests, evaluation of thyroid function, protein electrophoresis, and detailed investigations to detect systemic involvement.
The current classification does not meet the requirements of proper diagnosis of different presentations of LM. Subtyping of the studied patients differs greatly according to the old classification and the newly proposed one. New diagnostic criteria, classification, and grading are consequently suggested.
We propose two sets of diagnostic criteria to define the disease more precisely and to avoid confusion associated with the other classification. The first set comprises constant clinical and histopathological features that are always present in every case, and the second set includes associated features that were variably reported in some patients. LM is then subclassified according to the presence or absence of systemic manifestations into a systemic severe form (scleromyxedema) and a non-disabling, pure cutaneous form.
黏液水肿性苔藓（LM）是一种罕见的慢性特发性疾病，临床特征为蜡样、密集分布的丘疹，组织病理学特征为弥漫性真皮黏蛋白沉积和成纤维细胞增殖。黏液水肿性苔藓的最新分类于2001年提出；然而，它似乎复杂、令人困惑且不精确。在此，我们报告7例黏液水肿性苔藓病例，以评估当前分类的有效性，提出新的诊断标准和分类，并为这种罕见疾病提出一个与临床相关的严重程度分级系统。
该研究纳入了7例具有不同表现和严重程度的黏液水肿性苔藓患者。所有患者均接受了全面的皮肤科和全身检查、常规实验室检查、甲状腺功能评估、蛋白电泳以及检测全身受累情况的详细检查。
当前分类不符合准确诊断黏液水肿性苔藓不同表现的要求。根据旧分类和新提出的分类，所研究患者的亚型差异很大。因此，提出了新的诊断标准、分类和分级。
我们提出两组诊断标准，以更精确地定义该疾病，并避免与其他分类相关的混淆。第一组包括每个病例中始终存在的恒定临床和组织病理学特征，第二组包括在一些患者中不同程度报告的相关特征。然后根据是否存在全身表现将黏液水肿性苔藓分为全身严重型（硬化性黏液水肿）和非致残性、单纯皮肤型。
