Cheuk Wah, Lee King-Chung, Chong Lai-Yin, Yuen Siu-Tsan, Chan John K C
Department of Pathology, Queen Elizabeth Hospital Hong Kong, SAR China.
Am J Surg Pathol. 2009 Nov;33(11):1713-9. doi: 10.1097/PAS.0b013e3181b201de.
IgG4-related sclerosing disease is a syndrome characterized by the involvement of a wide variety of tissues by lymphoplasmacytic infiltrates and sclerosis, elevated serum IgG4 titer, and increased IgG4+ plasma cells in tissues. This report describes 2 cases with skin involvement, a feature not well documented in the literature. The patients had plaques or nodules in the skin of the cheek, temporal or periauricular region. Histologically, there was dermal and subcutaneous involvement by a nodular lymphoid infiltration often interspersed with lymphoid follicles and sclerotic stroma. The infiltrate was rich in plasma cells, small lymphocytes, and sometimes plasmablasts. The IgG4+ cell count was high (342 to 425 per high power field), with an IgG4/IgG proportion from 68% to 100%. As the morphology was compatible with pseudolymphoma, 14 cases of cutaneous pseudolymphoma were retrieved from the archives for IgG4 and IgG immunostaining. Two cases exhibited marked increase in IgG4+ cells, and showed many similarities with cutaneous manifestation of IgG4-related sclerosing disease, but the limited available clinical information precluded a conclusion on their nosologic nature. In summary, IgG4-related sclerosing disease can manifest with skin lesions, and is also one of the potential etiologies of cutaneous pseudolymphomas.
IgG4相关性硬化性疾病是一种综合征，其特征为多种组织受到淋巴浆细胞浸润和硬化的影响、血清IgG4滴度升高以及组织中IgG4+浆细胞增多。本报告描述了2例有皮肤受累的病例，这一特征在文献中记载较少。患者在脸颊、颞部或耳周区域的皮肤出现斑块或结节。组织学上，真皮和皮下组织有结节状淋巴样浸润，常夹杂着淋巴滤泡和硬化性间质。浸润细胞富含浆细胞、小淋巴细胞，有时还有成浆细胞。IgG4+细胞计数较高（每高倍视野342至425个），IgG4/IgG比例为68%至100%。由于形态学与假性淋巴瘤相符，从存档病例中检索出14例皮肤假性淋巴瘤进行IgG4和IgG免疫染色。2例显示IgG4+细胞显著增多，并与IgG4相关性硬化性疾病的皮肤表现有许多相似之处，但有限的临床资料无法对其疾病性质作出结论。总之，IgG4相关性硬化性疾病可表现为皮肤病变，也是皮肤假性淋巴瘤潜在病因之一。
