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ORIGINAL ARTICLE
Year : 2012  |  Volume : 57  |  Issue : 3  |  Page : 201-205
Mucocutaneous and demographic features of systemic sclerosis: A profile of 46 patients from Eastern India


1 Department of Dermatology, Venereology, and Leprosy, R.G. Kar Medical College, 1, Khudiram Bose Sarani, Kolkata, India
2 Department of Community Medicine, Burdwan Medical College, Burdwan, India

Date of Web Publication16-May-2012

Correspondence Address:
Sudip Kumar Ghosh
Department of Dermatology, Venereology, and Leprosy, R.G.Kar Medical College; 1, Khudiram Bose Sarani, Kolkata-700004, West Bengal
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0019-5154.96193

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   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.


Keywords: Demographic, eastern India, mucocutaneous, systemic sclerosis


How to cite this article:
Ghosh SK, Bandyopadhyay D, Saha I, Barua JK. Mucocutaneous and demographic features of systemic sclerosis: A profile of 46 patients from Eastern India. Indian J Dermatol 2012;57:201-5

How to cite this URL:
Ghosh SK, Bandyopadhyay D, Saha I, Barua JK. Mucocutaneous and demographic features of systemic sclerosis: A profile of 46 patients from Eastern India. Indian J Dermatol [serial online] 2012 [cited 2019 Jul 22];57:201-5. Available from: http://www.e-ijd.org/text.asp?2012/57/3/201/96193



   Introduction Top


Systemic sclerosis (SSc) is a chronic multisystem disorder of unknown etiology characterized clinically by thickening of the skin and by structural and functional abnormalities of different organ-systems. The degree and extent of the cutaneous and internal organ involvement are variable. Vascular structural alterations and tissue fibrosis seem to play an important role in the pathogenesis of SSc. [1] A large number of mucocutaneous features are seen in SSc. Identification of these features is of paramount importance for the early diagnosis of the disease.

Grossly, two subsets of the disease exist, namely, diffuse cutaneous systemic sclerosis (dcSSc) (both proximal and distal extremities involved) and limited cutaneous systemic sclerosis (lcSSc) (proximal extremities spared). [2] It is thought that dcSSc and lcSSc show striking differences in disease evolution kinetics. While in the diffuse cutaneous form visceral involvement may develop within weeks, patients with the limited cutaneous form typically remain stable for many years, but ultimately may develop late visceral complications. Interethnic disparity in terms of clinical manifestations and laboratory findings of SSc has been well documented in the existing literature.

We sought to detect the demographic profile and the relative frequencies and characteristics of different mucocutaneous features of SSc in a group of patients from eastern India. We further wanted to find out the significant difference (if any) between the limited and the diffuse forms of the disease regarding demographic and mucocutaneous features. Paucity of any formal study on these aspects of SSc in the eastern Indian population prompted us to undertake the present study.


   Materials and Methods Top


Patients

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 (August 2007-July 2009) were enrolled to the present study. Informed consent was obtained from all the participants. Detailed history regarding the demographic profile, symptoms, duration, and evolution of the cutaneous lesions, family history, drug intake, and occupation was obtained. The patients were thoroughly examined clinically keeping focus on the mucocutaneous manifestations.

Laboratory evaluation

Routine laboratory investigations including complete hemogram, ESR, C-reactive protein, and biochemical profile were done. Special laboratory tests like estimation of antinuclear antibody (ANA), antitropoisomerase-1, anticentromere antibody, and imaging of hand joints and lungs were done. ANA was analyzed and characterized by indirect immunofluorescence on HEp-2-cells. Lesional histopathology, barium swallow imaging, and other relevant investigations were done whenever needed.

Definition

Patients fulfilling the American College of Rheumatology criteria [1],[2] for classification of SSc were included in this study. The major criterion was sclerosis proximal to the metacarpophalangeal joints. Minor criteria were sclerodactyly, digital pitting scars, and bibasilar pulmonary fibrosis. To fulfill a diagnosis of SSc, either one major or two minor criteria were needed.

Statistical analysis

Categorical data were analyzed with a chi-square test (Yates correction and Fisher's exact P value was calculated in appropriate cases). Continuous variables were compared by unpaired student t test. All these tests were performed by using SPSS software (version 17.0). A P value <0.05 was considered statistically significant.


   Results Top


A total of 46 patients (female: male=8.2:1; mean age= 29.6±12.3 years) of SSc were evaluated. Thirty-two (69.6%) patients were housewives, 9 (19.6%) were students, 2 (4.3%) were clerical workers, 2 (4.3%) manual laborer, and one (2.2%) was unemployed. Family history of SSc was not present in any of the patients. The mean duration of SSc before presenting to us was 23 months.

Thirty (71.4%) patients gave history of hand and feet swelling as the initial symptom. Among the mucocutaneous manifestations [Table 1], Raynaud's phenomenon was present in 39 (84.8%) patients. Out of these 39 patients, it preceded skin tightness in 87.2% (34) of patients and in the remainder (5,12.8% patients), Raynaud's phenomenon and skin tightness appeared simultaneously.
Table 1: Distribution of different clinical manifestations of systemic sclerosis (n=46)

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Dyspigmentation was noted in 40 (86.9%) patients; diffuse hyperpigmentation was seen in 17 (36.9%) patients, localized pigmentation in 4 (8.7%) patients and salt and pepper pigmentation in 25 (54.3%) patients [Figure 1] and [Figure 2]. Sclerodactyly was noticed in 38 (82.6%) patients, difficulty in opening the mouth in 38 (82.6%) patients, and mat-like telangiectasia in 11 (23.1%) patients. Fingertip ulceration and scarring were seen in 29 (63%) patients [Figure 3], cutaneous calcinosis in one (2.2%) patient, digital gangrene in two (4.3%) patients, generalized pruritus in four (8.7%) patients, cutaneous small vessel vasculitis in two (4.3%) patients, and chronic urticaria in two (4.3%) patients. Mucosal changes were observed in 10 (21.7%) patients including seven (15.2%) patients with mucosal hyperpigmentation and two (4.3%) patients with oral candidiasis and one (2.2%) with perleche. Diffuse alopecia affected 10 (21.7%) patients. Nail changes were seen in 13 (28.2%) patients. The findings included shiny nails in nine (19.6%) patients, increased convexities in four (8.7%), clubbing of the fingers in two (4.3%), periungual telangiectasia in one (2.2%), and stunted growth of nails in one (2.2%) patient. Flexion contractures of the fingers were noted in 13 (28.3%) patients and amputation of the digits in three (6.5%) patients [Figure 4] and [Figure 5]. Out of the 46 patients of SSc, dcSSc was noted in 27 (58.7%) patients and lcSSc in the remainder (19, 41.3%).
Figure 1: Salt and pepper dyspigmentation and radial furrowing around the mouth

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Figure 2: Salt and pepper dyspigmentation on chest

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Figure 3: Pitted scar on the finger tips in a young boy

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Figure 4: Shortening of the distal portions of the digits along with sclerodactyly

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Figure 5: X-ray of the hand (lateral view) showing resorption of the terminal phalanges

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ANA was tested in all the patients. Thirty-six (78.2%) patients were positive to ANA test. We could analyze Scl-70 in 24 patients of suspected diffuse SSc and among them 17 (70.8%) patients (n=24) were positive. Anti-centromere antibody was performed in 12 suspected cases of limited SSc and out of them 75% (9/12) patients showed positive results.

On comparing the various features between patients of diffuse versus limited scleroderma [Table 2], there was no statistically significant difference in their age (P=0.15) and gender (P=0.66) distribution.
Table 2: Profile of diffuse and localized systemic sclerosis according to different clinico-demographic parameters (n=46)

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Raynaud's phenomenon was present in 24 (88.9%) and 17 (89.5%) of diffuse and limited SSc patients respectively. Duration of Raynaud's and duration of tightness were more in the diffuse category. However, the difference was not significant statistically (P>0.05). Raynaud's phenomenon appeared earlier than tightness in 19 (79.2%) and 15 (88.2%) of diffuse and limited SSc patients respectively. In five (20.8%) patients of dcSSc Raynaud's appeared simultaneously with tightness. However the interval between the appearance of Raynaud's and the appearance of tightness was comparable in both groups (P=0.90). Difficulty in the opening of the mouth, sclerodactyly, and mat-like telangiectasia were more common in the localized group in comparison to dcSSc but, none of the differences was statistically significant. Finger ulcer and mucosal changes were more predominant in dcSSc, while pigmentary changes and ANA positivity were more in limited SSc. However, these differences were not statistically significant (P>0.05).


   Discussion Top


SSc has a wide array of mucocutaneous manifestations. Despite the heterogeneity of SSc, almost all patients have skin involvement. As such, skin manifestations are crucial in the initial diagnosis of SSc and in the subsequent classification into the different subsets of disease. According to the extent of skin involvement (proximal or distal to elbows and knees) SSc is classified as diffuse cutaneous (dcSSc) or limited cutaneous (lcSSc) disease respectively. Limited cutaneous disease is usually preceded by Raynaud's phenomenon and is characterized by cutaneous sclerosis distal to the elbows, gastrointestinal and pulmonary fibrosis, and anticentromere antibody positivity. On the other hand simultaneous Raynaud's phenomenon, cutaneous skin involvement proximal to the elbow with gastrointestinal, pulmonary, renal and cardiac fibrosis, characterizes dcSSC. [3]

SSc is a relatively rare acquired sporadic disease with a global distribution. Worldwide estimated incidence of SSc is 2.3-10 people per 1 million. [4] No apparent racial predilection exists. [4] SSc can occur in any age group and affect either sex. In consonance with the existing data, there was a female preponderance of SSc in our series (male: female=0.12). Similar results were obtained in a north Indian study [5] (male: female=0.19) and a study from Dakar (male: female=0.19). In a recent study from Sarawak [6] and in another study on Afro-Caribbean population 96% patients were female. [7] In approximately 85% of cases, SSc occurs in individuals aged 20-60 years. Cases are also observed in children and in the elderly population.

The mean age of presentation was 29.6±12.3 years in the present study. Similar findings were obtained from a north Indian study (mean age of presentation was 32.75±11.62 years) [5] and a study from Dakar (33 years). [8] In the present series 19.5% of the patients were below 18 years of age. In a south Indian study 9 out of the 78 patients were children. [9] In another recent study the occurrence of childhood cases was 8.9%. [8]

Although no history of SSc in the first-degree relatives of our patients was found in the present study, familial cases of SSc have rarely been reported in the literature. [10]

The skin is almost universally involved in SSc. The earliest findings may be puffiness, swelling, and decreased flexibility of the joints and tendons. [2] Puffy hand was found in 8.7% patients of the present study. Cutaneous sclerosis was found in all the patients of the present study. More or less similar figures have been reported by the Indian studies (98.5% [5] , 100% [9]), and studies from Iraq (96.5%) [11] and Sarawak (100%). [6]

dcSSc was noted in 58.7% patients and lcSSc in the remainder of the patients in the present study. A study from Sarwak [6] also found dcSSc to be more common (70%) than lcSSc (30%). In another study dcSSC has been reported to be the more common (63%) subset of the disease among the Afro-Caribbean population. [7] On the other hand in Europeans lcSSc is the predominant subset.

Raynaud's phenomenon occurs almost universally in SSc and manifests as episodic blanching followed by cyanosis and then rubor. Raynaud's phenomenon was present in 84.8% of patients of the present series. More or less similar frequency of Raynaud's phenomenon was noted in a north Indian study (91%), [5] among the Afro-Caribbean population (93%), [7] and a study from Iraq (100%). [11] However, a much lower frequency has been reported in a south Indian study (28%) [9] and a study from Dakar (57%). [8] These discrepancies are probably due to the ethnic variation of the study population and climatic difference. Raynaud's phenomenon often predates other manifestations in the limited subtype and is often found concurrently in diffuse SSc. Our study also showed that Raynaud's phenomenon might either precede or appear simultaneously with the skin tightness. We observed fingertip ulceration and scarring in 63% of the patients. The range of frequencies of fingertip ulceration was 52%-70% in previous studies. [5],[6],[7],[11]

Different forms of pigmentary anomalies, namely diffuse hyperpigmentation, localized hyperpigmentation, salt and pepper pigmentation, are common in SSc. Dyspigmentation was a very common and prominent finding (86.9% patients) in our series. Variable incidence of hyperpigmentation ranging from 73.1% to 91% [5],[7],[9] was seen in previous studies. Although no cases of CREST syndrome (calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, telangiectasia) were noted in the present series, cutaneous calcinosis was seen in 2.2% patients. Flexion contractures of the hands were noted in 28.3% patients of the present series while Sharma et al. found a higher incidence (64.6%) in their study. [5]

Telangiectasia was reported in 48% of patients in an Afro-Caribbean study. [7] In a north Indian series 36.8% patients had telangiectasia. [5] in another study 33% patients had telangiectasia. [12] A much lower frequency was noted in the present series (23.1%).

Panniculitis [13] and cutaneous hyperpigmented reticulate scleroderma [14] are some recently described unusual cutaneous manifestation of SSc. We did not find any such cases in the present series. Nail changes were seen in 28.2% and mucosal changes in 21.7% of our patients. Data are sparse regarding the exact incidence of nail and mucosal changes in SSc.

The ANA test was positive in 78.2% patients of the present series. Previous studies have reported a variable percentage (56.8-89%) of ANA positivity. [5],[6],[8],[9],[12] We did not find any statistically significant difference between dcSSc and lcSSc in terms of mean age of presentation, gender distribution, Raynaud's phenomenon, finger ulceration, pigmentery changes, mouth opening, telangiectasia, sclerodactyly, mucosal changes, and ANA positivity.

Interethnic variation in clinical and lab manifestations of SSc has been well documented. [12] Important sociodemographic, clinical, and serologic differences exist between whites, African Americans, and Hispanics, in spite of shared genetic (HLA class II) predisposing factors. [15] Our study further supports this view as different mucocutaneous manifestations of our patients were in conformation with some studies whereas occurrence of some features was grossly different from others as mentioned above.

The present study provides a snapshot of the spectrum of cutaneous manifestations of SSc in the eastern Indian population. Furthermore our study also showed that there are hardly any statistically significant differences between dcSSc and lcSSc in terms of mucocutaneous manifestations in the studied population. A limitation of the present study was the relatively small sample size. A larger study comparing the features of dcSSc and lcSSc may further highlight the differences in the eastern Indian population.

 
   References Top

1.Haustein UF. Systemic sclerosis-scleroderma. Dermatol Online Journal 2002 ;8:3.  Back to cited text no. 1
    
2.Varga J. Systemic Sclerosis (Scleroderma) and related disorders. In: Fauci AS, Braunwald E, Kasper DL, Hauser SL, Longo DL, Jameson JL, Loscazo J, editors. Harrison's Principles of Internal Medicine. 17 ed. New York: McGraw-Hill; 2008. p. 2096-106.   Back to cited text no. 2
    
3.Hawk A, English JC 3 rd . Localized and systemic scleroderma. Semin Cutan Med Surg 2001;20:27-37.  Back to cited text no. 3
    
4.Schwartz RA. Systemic Sclerosis. e- Medicine. Available from: http://www.emedicine.medscape.com. [accessed on 2010 Jun 11].  Back to cited text no. 4
    
5.Sharma VK, Trilokraj T, Khaitan BK, Krishna SM. Profile of systemic sclerosis in a tertiary care center in North India. Indian J Dermatol Venereol Leprol 2006;72:416-20.  Back to cited text no. 5
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7.Flower C, Nwankwo C. Systemic sclerosis in an Afro-Caribbean population: A review of demographic and clinical features. West Indian Med J 2008;57:118.  Back to cited text no. 7
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8.Dia D, Dieng MT, Sy TN, Diallo M, Fall S, Ndongo S, et al. Systemic scleroderma: 92 cases in Dakar. Dakar Med 2003;48:82-6.  Back to cited text no. 8
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9.Krishnamurthy V, Porkodi R, Ramakrishnan S, Rajendran CP, Madhavan R, Achuthan K, et al. Progressive systemic sclerosis in south India. J Assoc Physicians India 1991;39:254-7.  Back to cited text no. 9
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13.Almeida MS, Lima SC, Carvalho LL, Almeida JV, Santos LG, Rolim JR, et al. Panniculitis: An unusual cutaneous manifestation of systemic sclerosis. J Cutan Pathol 2010;37:1170-3.   Back to cited text no. 13
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14.Ee HL, Tan SH. Reticulate hyperpigmented scleroderma: A new pigmentary manifestation. Clin Exp Dermatol 2005;30:131-3.  Back to cited text no. 14
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15.Reveille JD, Fischbach M, McNearney T, Friedman AW, Aguilar MB, Lisse J, et al. Systemic sclerosis in 3 US ethnic groups: A comparison of clinical, sociodemographic, serologic and immunogenetic determinants. Semin Arthritis Rheum 2001;30:332-46.  Back to cited text no. 15
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    Figures

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    Tables

  [Table 1], [Table 2]

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