2022Journal of Marine Medical SocietyOpen access

Scleroderma – Lupus Erythematosus Overlap or Lupoderma

Durga Madhab Tripathy, Preema Sinha, Arun Hegde, Shekhar Neema

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Abstract

Overlap syndromes are distinct entities with manifestations of rheumatic diseases in a patient that are not restricted to a particular disease. Rheumatoid arthritis, systemic lupus erythematosus (SLE), scleroderma, Sjogren's syndrome, and idiopathic inflammatory myositis (IIM) are definite connective tissue diseases that can have symptoms with significant overlap. If the clinical, biochemical, and serological parameters do not fulfill the criteria of any of the four diseases, it is termed as undifferentiated connective tissue disease. On the contrary, mixed connective tissue disease is a distinct clinical entity with specific parameters and positive U1RNP titers. Although scleroderma and SLE have their overlap profiles with other inflammatory rheumatic conditions, overlap with IIMs has been the commonest co-association in both the conditions. SLE-scleroderma overlap has been infrequently reported in the literature and can be considered as the rarest overlap as far as prevalence is concerned. We report a case of SLE-scleroderma overlap in a 21-year-old female along with discussing various aspects of the manifestations and complications of the overlap along with the challenges faced by the clinician in managing this relatively rare entity.

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Overlap syndromes are distinct entities with manifestations of rheumatic diseases in a patient that are not restricted to a particular disease. Rheumatoid arthritis, systemic lupus erythematosus (SLE), scleroderma, Sjogren's syndrome, and idiopathic inflammatory myositis (IIM) are definite connective tissue diseases that can have symptoms with significant overlap. If the clinical, biochemical, and serological parameters do not fulfill the criteria of any of the four diseases, it is termed as undifferentiated connective tissue disease. On the contrary, mixed connective tissue disease is a distinct clinical entity with specific parameters and positive U1RNP titers. Although scleroderma and SLE have their overlap profiles with other inflammatory rheumatic conditions, overlap with IIMs has been the commonest co-association in both the conditions. SLE-scleroderma overlap has been infrequently reported in the literature and can be considered as the rarest overlap as far as prevalence is concerned. We report a case of SLE-scleroderma overlap in a 21-year-old female along with discussing various aspects of the manifestations and complications of the overlap along with the challenges faced by the clinician in managing this relatively rare entity.

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Available abstract

Overlap syndromes are distinct entities with manifestations of rheumatic diseases in a patient that are not restricted to a particular disease. Rheumatoid arthritis, systemic lupus erythematosus (SLE), scleroderma, Sjogren's syndrome, and idiopathic inflammatory myositis (IIM) are definite connective tissue diseases that can have symptoms with significant overlap. If the clinical, biochemical, and serological parameters do not fulfill the criteria of any of the four diseases, it is termed as undifferentiated connective tissue disease. On the contrary, mixed connective tissue disease is a distinct clinical entity with specific parameters and positive U1RNP titers. Although scleroderma and SLE have their overlap profiles with other inflammatory rheumatic conditions, overlap with IIMs has been the commonest co-association in both the conditions. SLE-scleroderma overlap has been infrequently reported in the literature and can be considered as the rarest overlap as far as prevalence is concerned. We report a case of SLE-scleroderma overlap in a 21-year-old female along with discussing various aspects of the manifestations and complications of the overlap along with the challenges faced by the clinician in managing this relatively rare entity.

Key concepts: Medicine, Scleroderma (fungus), Overlap syndrome, Mixed connective tissue disease, Undifferentiated connective tissue disease, Rheumatoid arthritis, Dermatology, Polymyositis

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