Open AccessCross-sectional study on clinical features and histopathology of systemic sclerosis
Author(s) -
Deepthi N Sureshan,
Najeeba Riyaz,
Lekha Thumbayil
Publication year - 2019
Publication title -
journal of skin and sexually transmitted diseases./journal of skin and sexually transmitted diseases
Language(s) - English
Resource type - Journals
eISSN - 2994-6026
pISSN - 2582-3175
DOI - 10.25259/jsstd_29_2019
Subject(s) - medicine , fibrosis , scleroderma (fungus) , histology , pathology , systemic disease , histopathology , skin biopsy , population , disease , trichrome , biopsy , dermatology , gastroenterology , immunohistochemistry , h&e stain , environmental health , inoculation
Objectives: To study the cutaneous and systemic manifestations of systemic sclerosis (SSc) and correlate the severity of cutaneous disease with extent of fibrosis on histology. Methods and Materials: Patients were evaluated for cutaneous and systemic manifestations of SSc. Cutaneous disease was assessed using modified Rodnan Skin Score (mRSS) with mRSS ≥14 indicating severe disease. Masson’s trichrome stained skin biopsy specimens were graded semi-quantitatively according to severity and extent of dermal fibrosis. Subsequently, the clinicohistological correlation was assessed. Results: Thirty-two patients were studied. Eighteen patients had diffuse cutaneous SSc while 14 had limited cutaneous SSc. Ten patients had mRSS <14 while 22 had mRSS ≥14 with a mean mRSS of 15.8. Gastrointestinal system was involved in 56.3%, respiratory system in 53.1%, musculoskeletal system in 31.3%, renal in 6.3%, and cardiovascular in 3.1%. Anti-centromere antibodies were positive in six patients and Anti-Scl-70 in 12. In the histopathological analysis of fibrosis, 40.6% of patients had moderate fibrosis while 59.4% had severe fibrosis. While patients with higher mRSS also had a higher grade of fibrosis histologically, the clinicohistological correlation was not found to be statistically significant. Limitations: The prognostic significance of mRSS could not be assessed as this was a cross-sectional study. Conclusion: The cutaneous and systemic involvement observed in this study was comparable to the findings in other studies. The changes observed in frequency of specific manifestations in different population groups point to the role of genetic and environmental factors in the disease process.