Asian Journal of Research in Dermatological Science

Sclerodermatomyositis Overlap Syndrome with Bullous Lesion: A Rare Presentation

Meryem Elomari Alaoui *

Department of Dermatology and Venerology, University Hospital Center Ibn SINA, University Mohamed V, Rabat, Morocco.

Amani Fliti

Department of Dermatology and Venerology, University Hospital Center Ibn SINA, University Mohamed V, Rabat, Morocco.

Narjess Er-Rachdy

Department of Dermatology and Venerology, University Hospital Center Ibn SINA, University Mohamed V, Rabat, Morocco.

Mariame Meziane

Department of Dermatology and Venerology, University Hospital Center Ibn SINA, University Mohamed V, Rabat, Morocco.

Nadia Ismaili

Department of Dermatology and Venerology, University Hospital Center Ibn SINA, University Mohamed V, Rabat, Morocco.

Laila Benzekri

Department of Dermatology and Venerology, University Hospital Center Ibn SINA, University Mohamed V, Rabat, Morocco.

Karima Senouci

Department of Dermatology and Venerology, University Hospital Center Ibn SINA, University Mohamed V, Rabat, Morocco.

*Author to whom correspondence should be addressed.

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Abstract

Overlap syndrome is diagnosed when at least two classical connective tissue diseases are observed in one patient, which develops simultaneously or sequentially.

Sclerodermatomyositis (SDM) is one of the overlapping syndromes, defined by the association of dermatomyositis and scleroderma, which may be localized or systemic.

The American College of Rheumatology European League against Rheumatism (ACR-EULAR) classification criteria are used to establish the diagnosis of systemic sclerosis and dermatomyositis.

Clinically, this syndrome is characterized by the association of myalgia or myositis, arthralgia, with the skin changes of scleroderma, as well as Raynaud's phenomenon, interstitial lung disease, calcinosis, masked facies, dysphagia or esophageal dysmotility4, and immunologically, the presence of specific Pm/Scl antibodies in over 50% of cases.

Rare cases in the literature have described this association, but not in its bullous form, which is why we report here on a patient with bullous sclerodermatomyositis.

Keywords: Sclerodermatomyositis, bullous, overlap syndrome, autoimmune condition

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References

Jablonska E, Chorzelski T, Blaszczyk M, Jarzabek-Chorzelska M, Kumar V, Beutner EH. Scleroderma/polymyositis overlap syndromes and their immunologic markers. Clin Dermatol. 199210(4):457-72. Review; PubMed.

Varga J. Disorders of immune-mediated injury: Systemic sclerosis (scleroderma) and related disorders. In: Jameson JL, Fauci AS, Kasper DL, et al. 20th edn. Harrison’s principle of internal medicine. New York: McGraw-Hill, USA, 2546–2560; 2018.

Varga J. Clinical manifestations and diagnosis of systemic sclerosis (scleroderma) in adults. Uptodate; 2021.

Reddy H, Kashiv P, Kumar S, et al. Overlap of Scleroderma and Class V Lupus Nephritis: A Rare Autoimmune Confluence. Cureus. 2024;16(3):e56990. DOI: 10.7759/cureus.56990

Iorizzo LJ, Jorizzo JL. The treatment and prognosis of dermatomyositis: An updated review. J Am Acad Dermatol. 2008;59:99-112.

Laccarino L, Gatto M, Bettio S. et al. Overlap connective tissue disease syndromes. Autoimmunity Reviews. 2013; 12:363-373.

Balbir-Gurman A, Braun-Moscovici Y. Scleroderma Overlap Syndrome. IMAJ 2011;13:14-20

Jury EC, D’Cruz D, Morrow WJ. Autoantibodies and overlap syndromes in autoimmune rheumatic disease. J Clin Pathol. 2001;54:340-7.

Marcus M, Ilyas M, Tolaymat A. Childhood scleromyositis with a negative PM/Scl antibody. Joint Bone Spine. 2010;77: 73-5.

Febyani M, Purbo HD, Hamijoyo L et al. Sclerodermatomyositis. Indon. J. Rheum. 2011;3:32–36.

Ambade GR, Dhurat RS, Lade N, et al. Childhood sclerodermatomyositis with generalized morphea. Indian. J. Dermatol. Venereol. Leprol. 2008;74(2): 148–150.

Febyani M, Purbo DH, Hamijoyo L, Sutedja E, Suwarsa O, Sclerodermatomyositis, Indonesian Journal of Rheumatology; 2011.

Kubo M, Sato S, Kitahara H, Tsuchida T, Tamaki K. Vesicle formation in dermatomyositis associated with gynecologic malignancies. Journal of the American Academy of Dermatology. 1996;34(2):391–394. DOI: 10.1016/s0190-9622(07)80015-6

James WD, Berger TG, Elston DM, editors. Andrews’ diseases of the skin clinical dermatology. 10th ed. Canada: Elsevier; 2006;166-77.

Nagaraju K, Lundberg IE. Inflammatory diseases of muscle and other myopathies. In: Firestein GS, Budd RC, Harris ED, Mcinnes IB, Ruddy S, Sergent JS, editors. Kelley’s textbook of rheumatology. 8th ed. Philadelphia: Elsevier. 2009;1353-78.

Greenberg SA, Amato AA. Disorders of immune-mediated injury: Inflammatory myopathies. In: Jameson JL, Fauci AS, Kasper DL et al. 20th edn. Harrison’s principle of internal medicine. New York: McGraw-Hill, USA. 2018;2590– 2596.

Zenahbezu Abay, Nebiyu Bekele, et al Diagnosis of sclerodermatomyositis in Ethiopian woman, International Journal of Clinical Rheumatology. 2021;16(7).