Abstract
BackgroundScleredema is a rare sclerodermoid skin condition characterized by diffuse symmetrical thickening of the upper part of the body. Its association with monoclonal gammopathy and myeloma was recently described; very few cases have been reported to date.Case presentationA 66-year-old Sri Lankan woman who had been followed in a dermatology unit for 34 years with diffuse systemic sclerosis presented with an acute exacerbation of the skin disease. Absence of Raynaud’s phenomenon; sclerodactyly; characteristic lung, gastrointestinal, and cardiac involvement of systemic sclerosis; and repeatedly negative antinuclear antibodies test results led to reevaluation for the possibility of scleredema. Skin biopsies from four body sites showed normal epidermis and thickened reticular dermis with swollen collagen bundles separated from one another by clear spaces, resulting in fenestration. The skin appendages were not atrophied or bound down. Alcian blue staining showed interstitial mucin deposition. Serum protein electrophoresis demonstrated an abnormal monoclonal band in the β-region with a paraprotein level of 8.9 g/dl. Immunofixation showed an abnormal band in the γ-region consisting of immunoglobulin A and κ. Bone marrow biopsy revealed abnormal monoclonal plasma cells (15%) with multinuclearity. There was no evidence of end organ damage, and whole-body magnetic resonance imaging did not reveal any evidence of bone involvement. The patient’s diagnosis was revised as scleredema type 2 associated with IgA-κ, and she was referred to a hemato-oncologist for chemotherapy, which led to significant improvement in the skin condition.ConclusionsScleredema is a rare disorder that has an enigmatic, rare association with monoclonal gammopathy. Dermatologists should be aware of this rare but important association.
Highlights
Scleredema is rare sclerodermoid skin disease characterized by symmetrical diffuse woody induration of the upper part of the body owing to thickened dermis and excessive dermal mucin deposition
We report a case of a patient with long-standing widespread scleredema associated with immunoglobulin A-κ smoldering myeloma, which was misdiagnosed as scleroderma for many years
A few case reports are available in the literature on scleredema associated with myeloma; to the best of our knowledge, this is the first patient to be Keragala et al Journal of Medical Case Reports (2019) 13:145 reported with scleredema who was diagnosed with smoldering myeloma of IgA-κ
Summary
Scleredema is rare sclerodermoid skin disease characterized by symmetrical diffuse woody induration of the upper part of the body owing to thickened dermis and excessive dermal mucin deposition. A few case reports are available in the literature on scleredema associated with myeloma; to the best of our knowledge, this is the first patient to be Keragala et al Journal of Medical Case Reports (2019) 13:145 reported with scleredema who was diagnosed with smoldering myeloma of IgA-κ This case report highlights the importance of awareness of scleredema because it is rare and can be misdiagnosed and, if diagnosed, it can be treated. Case presentation A 66-year-old Sri Lankan woman who had been followed in a dermatology unit for 34 years for diffuse systemic sclerosis presented to our institution with an acute exacerbation of the skin disease She was treated with corticosteroids and cyclophosphamide pulses and subsequently with mycophenolate mofetil for the skin condition. She denied cold-induced episodic acral bluish discoloration suggestive of Raynaud’s phenomenon She was not febrile, and the result of her general examination was normal without pallor, cyanosis, clubbing, lymphadenopathy, or bilateral ankle edema. She was commenced on intravenous immunoglobulin (IVIG) monthly (1 g/kg for 2 days per month), and a hemato-oncologist started
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