Clinical profile of cutaneous manifestations of connective tissue diseases in North-East India
Keywords:Connective tissue diseases, Lupus, Systemic sclerosis, Photosensitivity, Raynaud’s phenomenon, Sclerodactyly
Background: Connective tissue diseases (CTDs) are a heterogeneous group of autoimmune disorders having overlapping clinical features. Skin is often involved and it may be the earliest sign of the disease. This study highlighted the various cutaneous manifestations of common CTDs.
Methods: A hospital-based cross-sectional study was carried out for a period of two years in 83 patients with CTDs in dermatology OPD, RIMS, Imphal. Detailed history taking, examination and relevant serological tests were performed.
Results: The mean age was 39.78±17.29 years with female to male ratio of 4.5:1. Majority of the patients had lupus erythematosus (LE) (N=45) followed by systemic sclerosis (SSc) (N=25), rheumatoid arthritis (RA) (N=6), mixed connective tissue disease (MCTD) (N=4) and morphea (N=3). The most common presentation was raised skin lesions (45.8%) followed by Raynaud’s phenomenon (36.1%), photosensitivity (27.7%), skin tightness (26.5%) and joint pain (19.3%). Among LE patients, chronic cutaneous lupus erythematosus (CCLE) was the commonest variant and localised discoid lupus erythematosus (DLE) (22.9%) was the commonest presentation followed by malar rash and annular subacute lupus erythematosus (SCLE). Skin induration, microstomia and sclerodactyly were seen in most patients of SSc. Antinuclear antibodies were positive in 89.1% of patients. Anti-dsDNA and anti-Sm antibodies were positive in 62.2% and 33.3% of LE patients, anti-Scl 70 antibody was positive in 68% of SSc patients.
Conclusions: CTDs are rare but potentially life-threatening. Proper understanding of the spectrum of cutaneous manifestations of CTDs is therefore necessary for early diagnosis and efficient management.
Jacobe HT, Sontheimer RD. Autoantibodies encountered in patients with autoimmune connective tissue diseases. In: Bolognia JL, Jorizzo JL, eds. Dermatology. 3rd ed. China: Elsevier Saunders; 2012: 603-4.
Durosaro O, Davis MD, Reed KB, Rohlinger AL. Incidence of cutaneous lupus erythematosus, 1965-2005: a population-based study. Arch Dermatol. 2009;145(3):249-53.
Agrawal SR, Tiewsoh I, Rajput A, Jain A. A cross-sectional hospital based study of clinical and immunological profile of systemic lupus erythematosus patients from central rural India. Indian J Allergy Asthma Immunol. 2013;27(1):33-7.
Makol A, Crowson CS, Wetter DA, Sokumbi O, Matteson EL, Warrington KJ. Vasculitis associated with rheumatoid arthritis: a case control study. Rheumatology. 2014;53(5):890-9.
Font J, Cervera R, Espinosa G, Pallares G, Casals MR, Jimenez S, et al. Systemic lupus erythematosus (SLE) in childhood: analysis of clinical and immunological findings in 34 patients and comparison with SLE characteristics in adults. Ann Rheum Dis. 1998;57(8):456-9.
Kosaraju K, Shenoy S, Suchithra U. A cross-sectional hospital-based study of autoantibody profile and clinical manifestations of systemic lupus erythematosus in south Indian patients. Indian J Microbiol. 2010;28(3):245-7.
Moghadam-Kia S, Chilek K, Gaines E, Costner M, Rose ME, Okawa J, et al. Cross-sectional analysis of a collaborative web-based database for lupus erythematosus associated skin lesions: 114 prospectively enrolled patients. Arch Dermatol. 2009;145(3):255-60.
Jallouli M, Frigui M, Hmida MB, Marzouk S, Kaddour N, Bahloul Z. Clinical and immunological manifestations of systemic lupus erythematosus: a study on 146 south Tunisian patients. Saudi J Kidney Dis Transpl. 2008;19(6):1001-8.
Merola JF, Prystowsky SD, Iversen C, Puerta JA, Norton T, Tsao P, et al. Association of discoid lupus with other clinical manifestations among patients with systemic lupus erythematosus. J Am Acad Dermatol. 2013;69(1):19-24.
Heimovski FE, Simioni, Skare TL. Systemic lupus erythematosus and Raynaud’s phenomenon. An Bras Dermatol. 2015;90(6):837-40.
Santiago-Casas Y, Vila LM, McGwin G, Cantor RS, Petri M, Golman RR, et al. Association of discoid lupus with clinical manifestations and damage accrual in profile: a multiethnic lupus COHORT. Arthritis Care Res. 2012;64(5):704–12.
Das NK, Dutta RN, Sengupta SR. Skin lesions in lupus erythematosus: a marker of systemic involvement. Indian J Dermatol. 2011;56(5):537-40.
Pradhan V, Rajadhyaksha A, Nadkar M, Pandit P, Surve P, Lecerf M, et al. Clinical and autoimmune profile of scleroderma patients from western India. Int J Rheumatol. 2014;2014:983781.
Dickey BZ, Holland KE, Drolet BA, Galbraith SS, Lyon VB, Siegel DH, et al. Demographic and clinical characteristics of cutaneous lupus erythematosus at a paediatric dermatology referral centre. Br J Dermatol. 2013;169(2):428-33.
Li J, Leng X, Li Z, Ye Z, Li C, Li X, et al. Chinese SLE treatment and research group registry: III. Association of autoantibodies with clinical manifestations in Chinese patients with systemic lupus erythematosus. J Immunol Res. 2014;25:1-6.
Wang J, Assassi S, Guo G, Tu W, Wu W, Yang L, et al. Clinical and serological features of systemic sclerosis in a Chinese COHORT. Clin Rheumatol. 2013;32(5):617-21.
Chularojanamontri L, Sethabutra P, Kulthanan K, Manapajon A. Dermatology life quality index in Thai patients with systemic sclerosis: a cross-sectional study. Indian J Dermatol Venereol Leprol. 2011;77(6):683-7.
Shanavas N, Das AK. Profile of systemic sclerosis and associated renal involvement. Arch Med Health Sci. 2015;3(2):209-14.
Basel ME, Khalil N. Disease characteristics of systemic sclerosis among Egyptian patients. Kasr El Ainy Med J. 2015;21(2):41-6.
Flower C, Nwankwo C. Systemic sclerosis in an Afro-Caribbean population: a review of demographic and clinical features. West Indian Med J. 2008;57(2):118-21.
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(3):201-5.
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(6):416-20.
Deepa AS, Rachel RP, Ramchandran P, Devaraj U, Arnold SA, Shobha V, et al. Pulmonary involvement in systemic sclerosis: a clinical profile. Lung India. 2016;33(2):144-7.
Purnima G, Bhavani VG, Kumar TSP, Dhankar SU. Profile of systemic sclerosis in tertiary care centre, Vijayawada. IOSR. 2016;15:24-8.
Letenberger J, Cayce RL, Haley RW, Huet BA, Bergstresser PR, Jacobe HT. Morphea subtypes are distinct autoimmune syndromes: a review of 245 adults and pediatric cases. Arch Dermatol 2009;145:545-50.
Bartels C, Bell C, Rosenthal A, Shinki K, Bridges A. Decline in rheumatoid vasculitis prevalence among US veterans: a retrospective cross-sectional study. Arthritis Rheum. 2009;60(9):2553-7.
Cojocaru M, Cojocaru IM, Silosi I, Doina C. Extra-articular manifestations in rheumatoid arthritis. J Clin Med. 2010;5(4):286-91.
Watts RA, Mooney J, Lane SE, Scott DG. Rheumatoid vasculitis: becoming extinct? Rheumatology. 2004;43(7):920-3.
Gurman AB, Moscovici YB. Scleroderma overlap syndrome. Isr Med Assoc J. 2011;13(1):14-20.
Vegh J, Szilasi M, Soos G, Devenyi K, Dezso B, Soltesz P, et al. Interstitial lung disease in mixed connective tissue disease. Orv Hetil. 2005;146:2435-43.
Maazoun F, Frikha F, Snoussi M, Kaddour N, Masmoudi H, Bahloul Z. Systemic lupus erythematosus myositis overlap syndrome: report of 6 cases. Clin Pract. 2011;1(4):89.
Szodoray P, Hajas A, Kardos L, Dezso B, Soos G, Zold E, et al. Distinct phenotypes in mixed connective tissue disease: subgroups and survival. Lupus. 2012;21(13):1412-22.