Scleredema associated with immunoglobulin A-κ smoldering myeloma: a case report and review of the literature

Abstract

Background Scleredema 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 presentation A 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. Conclusions Scleredema is a rare disorder that has an enigmatic, rare association with monoclonal gammopathy. Dermatologists should be aware of this rare but important association.

Concurrent Scleredema and Pyoderma Gangrenosum: Case Report and Review of Comorbid Conditions

“…It most often presents on the neck and extends to the shoulders and back; the face and scalp can also be affected. The clinical differential diagnoses of scleredema include dermatoses characterized by skin thickening, mucin deposition, or both; these conditions include cardiac or renal edema, cutaneous amyloidosis, eosinophilic fasciitis, lymphedema, myxedema, scleromyxedema, and systemic sclerosis [ 1 , 3 , 4 ].…”

“…The dermis can be up to four times thicker than the dermis found in the normal adjacent tissue. Either an Alcian blue stain or a colloidal iron stain will reveal mucin between collagen fibers of the thickened dermis [ 1 , 3 ]. The diagnosis of scleredema is established by correlating the clinical appearance of the affected skin to these pathologic findings [ 4 ].…”

“…A 34-year-old man with hypergammaglobulinemia and B-cell lymphoma was found to have tightness and thickening of the skin on the chest; the diagnosis of scleredema was established by skin biopsy and he was treated with prednisone [ 7 ]. Another example of paraprotein-associated scleredema is a 66-year-old woman with immunoglobulin A-k smoldering myeloma who was found to have scleredema; after a skin biopsy confirmed the diagnosis of scleredema, chemotherapy was initiated for her myeloma and the diffuse thickening of her skin improved [ 3 ].…”

“…Pyoderma gangrenosum occurring in the same location as scleredema, to the best of our knowledge, has not been described. Both diseases can be associated with monoclonal gammopathy [ 3 , 14 ]. Single patients with other conditions in common include pregnancy and chronic myeloid leukemia [ 9 , 10 , 16 , 18 ].…”

Cutaneous manifestations of monoclonal gammopathy

“…Skin-directed therapy can include ultraviolet (UV) light phototherapy (including narrowband UVB or UVA-1). A case report showed significant improvement with bortezomib and intravenous immunoglobulin [ 126 ]. In two other cases report, the combination of cyclophosphamide, bortezomib, and dexamethasone (CyBorD) led to a marked clinical and hematological improvement [ 127 , 128 ].…”