Mucocutaneous and demographic features of systemic sclerosis: A profile of 46 patients from Eastern India

Abstract

Background:Systemic sclerosis (SSc) is a multisystem connective tissue disorder of uncertain etiology. The clinical picture is frequently dominated by prominent cutaneous manifestations that have diagnostic and prognostic significance. The objective of the present study was to find out the demographic profile and the relative frequencies and characteristics of different mucocutaneous features of SSc in a group of patients from eastern India. In addition, we sought to compare the frequency and pattern of the findings in the limited versus the diffuse variety of the disease.Materials and Methods:This was a cross-sectional, clinical observational study. Consecutive patients of SSc attending the dermatology O.P.D. of a tertiary care hospital of eastern India over 3 years were enrolled to the present study.Results:A total of 46 patients (41 females and 5 males; mean age 29.6±12.3 years) of SSc were evaluated. Among mucocutaneous manifestations Raynaud's phenomenon was present in 39 (84.8%) patients. Other cutaneous features included dyspigmentation (40, 86.9%), sclerodactyly (38, 82.6%), inability to open the mouth (38,82.6%), mat-like telangiectasia (11,23.1%), fingertip ulceration and scarring (29,63%), cutaneous calcinosis (1,2.2%), digital gangrene in (2,4.3%), generalized pruritus (4,8.7%), cutaneous small vessel vasculitis (2,4.3%), chronic urticaria (2,4.3%), flexion contractures of the fingers (13,28.3%), and amputation of the digits (3,6.5%). Mucosal changes were observed in 10 (21.7%) patients and nail changes were seen in 13 (28.2%) patients. Diffuse cutaneous SSc was noted in 27 (58.7%) patients and limited cutaneous SSc was seen in the remainder. Thirty-six (78.2%) patients tested positive for ANA.Conclusion:The present study provides a snapshot of the spectrum of the demographic and mucocutaneous manifestations of SSc in the eastern Indian population. We have not observed any statistically significant differences between dcSSc and lcSSc in terms of mucocutaneous manifestations in the studied population.

Clinical profile of cutaneous manifestations of connective tissue diseases in North-East India

“…17,18,[21][22][23][24] Cutaneous sclerosis was found in most patients in the present study and similar findings have been reported by other authors (range: 90-98.5%). [21][22][23][24] Fingertip ulceration was noted in 48% of patients which was also similar to that of previous studies (range: 37-63%). 18,21,22 In this study, "salt and pepper" pigmentation was seen in 44% of patients.…”

“…In a study conducted in Eastern India, 9 out of 46 patients were children. 21 Raynaud's phenomenon was seen in all patients of SSc and majority had sclerodactyly (76%) which was similar to studies from different parts of India. 17,18,[21][22][23][24] Cutaneous sclerosis was found in most patients in the present study and similar findings have been reported by other authors (range: 90-98.5%).…”

“…21 Raynaud's phenomenon was seen in all patients of SSc and majority had sclerodactyly (76%) which was similar to studies from different parts of India. 17,18,[21][22][23][24] Cutaneous sclerosis was found in most patients in the present study and similar findings have been reported by other authors (range: 90-98.5%). [21][22][23][24] Fingertip ulceration was noted in 48% of patients which was also similar to that of previous studies (range: 37-63%).…”

“…13,20 The mean age of presentation was 45.08±19.5 years which was similar to other studies. 21,22 In the present study, only one patient presented at 10 years of age. In a study conducted in Eastern India, 9 out of 46 patients were children.…”

A Study of Mucocutaneous Manifestations in Autoimmune Connective Tissue Disorders at Tertiary Care Centre

“…In SS, we found hidebound skin and decrease mouth opening in all patients followed by Raynaud's phenomenon (90%) and sclerodactyly (70%) similarly noted in study of Sudip Ghosh in eastern India. 12 In MCTD cases, all had given history of muscle weakness and pain followed by history of hair loss, joint pain and Raynaud's phenomenon (Image 6, 7).…”