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 ß-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.
Subject(s)
Magnetic Resonance Imaging , Scleredema Adultorum/diagnostic imaging , Scleroderma, Systemic/physiopathology , Smoldering Multiple Myeloma/diagnostic imaging , Whole Body Imaging , Aged , Antineoplastic Agents/administration & dosage , Bortezomib/administration & dosage , Disease Progression , Female , Humans , Immunoglobulins/administration & dosage , Immunologic Factors/administration & dosage , Scleredema Adultorum/drug therapy , Scleredema Adultorum/physiopathology , Scleroderma, Systemic/complications , Scleroderma, Systemic/drug therapy , Smoldering Multiple Myeloma/drug therapy , Smoldering Multiple Myeloma/physiopathology , Treatment OutcomeABSTRACT
This article acquaints the reader with disorders of the skin that might mimic systemic sclerosis but whose pathology is localized to the skin and/or has extracutaneous manifestations that are different than systemic sclerosis. These disorders include localized scleroderma (morphea), eosinophilic fasciitis, scleredema, scleromyxedema, nephrogenic systemic fibrosis, and chronic graft-versus-host disease. Particular emphasis is placed on clinical and histopathologic features that help the clinician differentiate between these disorders. Treatment options are briefly reviewed.
Subject(s)
Scleroderma, Systemic/diagnosis , Skin Diseases/diagnosis , Chronic Disease , Diagnosis, Differential , Eosinophilia/diagnosis , Eosinophilia/physiopathology , Fasciitis/diagnosis , Fasciitis/physiopathology , Graft vs Host Disease/diagnosis , Graft vs Host Disease/physiopathology , Humans , Interdisciplinary Communication , Nephrogenic Fibrosing Dermopathy/diagnosis , Nephrogenic Fibrosing Dermopathy/physiopathology , Scleredema Adultorum/diagnosis , Scleredema Adultorum/physiopathology , Scleroderma, Localized/diagnosis , Scleroderma, Localized/physiopathology , Scleroderma, Systemic/physiopathology , Scleromyxedema/diagnosis , Scleromyxedema/physiopathology , Skin Diseases/physiopathology , Skin Diseases/therapyABSTRACT
El escleredema de Buschke se caracteriza por un engrosamiento de la dermis con depósito de mucina entre las fibras de colágeno, que se manifiesta como un endurecimiento de la piel, predominantemente en la mitad superior del tronco. Se ha relacionado con diabetes mellitus de larga evolución y mal controlada, gammapatías monoclonales e infecciones estreptocócicas. Se estudiaron los pacientes con el diagnóstico clínico de escleredema confirmado mediante estudio histopatológico y asociado a diabetes mellitus. Se revisaron las enfermedades asociadas, las características clínicas e histopatológicas, el curso evolutivo y la respuesta al tratamiento. El escleredema se caracteriza por un endurecimiento cutáneo que afecta a la mitad superior del tronco. En una gran proporción de casos se asocia a diabetes mellitus mal controlada, de curso prolongado durante años y con escasa respuesta al tratamiento
Scleredema of Buschke is characterized by a thickening of the dermis with mucin deposits among the collagen fibers, which manifests as a hardening of the skin, predominantly on the upper trunk. It has been associated with long-standing, poorly controlled diabetes mellitus, monoclonal gammopathies and streptococcus infections. We studied patients with a clinical diagnosis of scleredema confirmed by means of a histopathological study, and associated with diabetes mellitus. We reviewed associated diseases, clinical and histopathological characteristics, evolution and response to treatment. Scleredema is characterized by hardening of the skin, affecting the upper trunk. A large proportion of cases are associated with long-standing, poorly controlled diabetes mellitus, with little response to treatment
Subject(s)
Male , Female , Adult , Aged , Humans , Scleredema Adultorum/complications , Scleredema Adultorum/diagnosis , Scleredema Adultorum/therapy , Diabetes Mellitus/complications , Diabetes Mellitus/therapy , Scleredema Adultorum/pathology , Scleredema Adultorum/physiopathology , Diagnosis, Differential , BiopsyABSTRACT
We report a case of scleredema of Buschke associated with IgG kappa monoclonal hypergammaglobulinaemia. After myeloma polychemotherapy an improvement in skin involvement was observed and confirmed by means of noninvasive skin elasticity measurements. This suggests a relationship between the two diseases. The bioengineering method used can be useful for early detection and monitoring the skin involvement in patients with this disease association.
