An Unusual Presentation of a Rare Scleroderma Mimic: What is Behind the Scenes?

Abstract

Background: Scleroderma or systemic sclerosis (SSc) is a rare systemic autoimmune disease. 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 was found 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 of diffuse skin thickening of face, neck, arms, forearms, thighs, chest, back, and excluding both hands and 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 was 100 mm/1st h, autoantibodies for SSc were negative, nail fold capillaroscopy normal, pulmonary function tests showed restrictive pattern and high-resolution computed tomography showed interstitial lung fibrosis. Patient was not fulfilling the American collage of rheumatology/European League Against Rheumatism classification criteria for SSc. Skin biopsy was done and revealed histological appearance of scleredema. Investigations were done for disease association with scleredema. The patient was not diabetic, antistreptolysin O titer was normal, serum protein electrophoresis, immunofixation and bone marrow biopsy were done, and the patient was diagnosed as scleredema associated with immunoglobulin A kappa multiple myeloma. Treatment by combination of bortezomib, cyclophosphamide, and dexamethasone was started with marked clinical and hematological improvement. Conclusion: Many conditions mimic SSc including scleredema, which may be the initial presentation of multiple myeloma. Rheumatologists and dermatologists should be able to recognize these conditions to provide the suitable management and follow-up for these patients.</P>