Background: Scleroderma or systemic sclerosis (SSc) is a rare systemic autoimmunedisease. Many conditions mimic the presentation of SSc, especially skin thickening and fibrosis.One of these conditions is scleredema, a rare collagen and mucin deposition disorder which wasfound to be associated with diabetes mellitus, streptococcal infection or monoclonal gammopathy.
Case Presentation: A 55 years old female presented with insidious onset and progressive course ofdiffuse skin thickening of face, neck, arms, forearms, thighs, chest, back, and excluding both handsand feet of 6 years’ duration associated with arthralgia, dysphagia and dyspnea on exertion of 1-year duration. There was no history of Raynaud’s phenomenon. Erythrocyte sedimentation rate was100 mm/1st h, autoantibodies for SSc were negative, nail fold capillaroscopy normal, pulmonaryfunction tests showed restrictive pattern and high-resolution computed tomography showed interstitiallung fibrosis. Patient was not fulfilling the American college of rheumatology/European LeagueAgainst Rheumatism classification criteria for SSc. Skin biopsy was done and revealed histologicalappearance of scleredema. Investigations were done for disease association with scleredema. Thepatient was not diabetic, antistreptolysin O titer was normal, serum protein electrophoresis, immunofixationand bone marrow biopsy were done, and the patient was diagnosed as scleredema associatedwith immunoglobulin A kappa multiple myeloma. Treatment by combination of bortezomib,cyclophosphamide, and dexamethasone was started with marked clinical and hematologicalimprovement.
Conclusion: Many conditions mimic SSc including scleredema, which may be the initial presentationof multiple myeloma. Rheumatologists and dermatologists should be able to recognize theseconditions to provide the suitable management and follow-up for these patients.
Keywords: Scleroderma, scleredema, scleroderma mimics, multiple myeloma, monoclonal gammopathy, fibrosis.