한국인의 국소피부경화증에 대한 임상병리학적 고찰

Abstract

Background: Morphea is an autoimmune condition characterized by excessive accumulation of collagen in the skin and underlying tissues. Its exact pathogenesis is poorly understood, and only a few clinical or histopathological studies have been conducted in Asian patients.

Objective: The aim of this study was to identify the clinical and histopathological characteristics of morphea in Korean patients. Methods: We reviewed the medical records of 101 patients retrospectively, whose clinicopathologic findings were compatible with morphea. We analyazed demographic characteristics, number of lesions, disease subtype, and histopathological findings. The density of inflammatory cell infiltrate was classified as absent, mild, moderate, or dense.

Results: The mean age of the 101 patients was 25 years old with a female predominance (female to male ratio 2.3:1.0). Overall, circumscribed morphea (52.5%) was the most common clinical type, followed by linear (28.7%), generalized (13.9%), and mixed (13.9%) types. In the pediatric group, linear morphea was the most common subtype (38.9%). The most frequent location of the circumscribed lesions was the trunk (54.2%) followed by lower extremities (21.9%), head and neck (15.6%), and upper extremities (11.5%). The most common site of the linear lesions was upper extremities (39.2%) followed by lower extremities (29.4), head and neck (27.5%), and trunk (3.9%). Disease duration was positively correlated with increased thickness of the skin in 54 patients (p<0.001). Of 89 biopsy samples, inflammatory cell infiltration was absent in 50, mild in 26, and moderate in 13 patients. Disease duration was not correlated with the density of inflammatory cell infiltration (p=0.110). Antinuclear antibody (ANA) was positive in 52.1% of the patients, and ANA positivity was highest in generalized morphea (72.7%).

Conclusions: The most common disease subtype in Korean patients is circumscribed morphea in adults, and linear morphea in children, which is similar to the pattern reported in non-Asians. The rate of generalized morphea in this study was higher than in a previous Korean study, but comparable with the rate in non-Asian populations. There is an association between skin thickness and disease duration. However, disease duration is not significantly associated with the extent of inflammatory cell infiltration. Many characteristics of morphea remain unexplained, and further research, on a larger sample, would be desirable.