Scleredema of Buschke is a rare connective tissue disease with a poorly understood pathogenesis. Three types of scleredema have been distinguished according to its association with preceding or underlying conditions. Type 1 is usually secondary to a febrile infection, type 2 is mostly associated with paraproteinemia and type 3, usually named scleredema diabeticorum, has a strict association with Diabetes mellitus. A diffuse, non-pitting swelling and induration of the skin define this disease. The skin histology is characterized by a normal or slightly thinned epidermis, and the dermis containing a decreased number of elastic fibers and thick large swollen collagen bundles separated by mucopolysaccharide deposits in the deep reticular dermis. In this report we present a 58-year-old man with scleredema diabeticorum controlled with a topical steroid cream and an optimization of glycemic control. We reviewed clinical, histopathological characteristics and the various possible treatments.

Scleroderma-like disorders include a set of entities involving cutis, subcutis and, sometimes, even muscular tissue, caused by several pathogenetic mechanisms responsible for different clinical-pathological pictures. The absence of antinuclear antibodies (ANA), Raynaud\'s phenomenon and capillaroscopic anomalies constitutes an important element of differential diagnosis with systemic sclerosis. When scleroderma can be excluded, on the basis of the main body sites, clinical evolution, any associated pathological conditions and specific histological features, it is possible to make a correct diagnosis.

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.
METHODS: 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.

To describe the clinical and ultrasonographic (US) findings in patients with scleredema adultorum of Buschke, who presented with sclerotic skin on their posterior neck.
After obtaining IRB approval, eight patients with scleredema adultorum of Buschke were enrolled. They underwent US examination of their posterior neck. The diagnoses were confirmed pathologically. The clinical history and US images were evaluated retrospectively. Dermal thickness was compared between the patient group and the age- and sex-matched control group.
The patients included seven males and one female with a mean age of 51.5 years. All patients presented with thickening of the skin and/or a palpable mass on the posterior neck. Five (62.5%) of the eight patients showed erythematous discoloration. Six patients (75.0%) had a history of diabetes. The Hemoglobin A1c level was found to be increased in all patients. US images did not show any evidence of a soft tissue mass or infection. The mean dermal thickness in patients (7.01 ± 1.95 mm) was significantly greater than that in the control group (3.08 ± 0.87 mm) (p = 0.001). Multiple strong echogenic spots in the dermis were seen in all patients. Seven patients (87.5%) showed posterior shadowing in the lower dermis.
When a patient with a history of diabetes presents with a palpable mass or erythematous discoloration of the posterior neck and US shows the following imaging features: 1) no evidence of a soft tissue mass or infection, 2) thickening of the dermis, 3) multiple strong echogenic spots and/or posterior shadowing in the dermis, scleredema adultorum of Buschke should be considered in the differential diagnosis.

BACKGROUND: Scleredema adultorum (Buschke\'s scleredema) is a cutaneous mucinosis of unknown origin, clinically characterized by a diffuse induration of the skin usually involving the neck, shoulders and back, which limits patients\' mobility.
METHODS: We report a case of a 50-year-old woman who presented a chronic sclerodermiform syndrome for 2 years associated with type 1 diabetes. Physical examination revealed an extensive skin induration involving the shoulders, neck and back. Histologic examination confirmed the diagnosis of scleredema adultorum. The patient was treated with extracorporeal photopheresis (EPP) twice a month for two months. At follow-up, mobility was highly improved after two months. Beneficial effect of EPP was maintained on the long term while sessions were spaced.
CONCLUSIONS: EPP is an unconventional treatment of Buschke\'s scleredema. We described a case of Buschke\'s scleredema successfully treated with EPP which may represent a therapeutic option for the treatment of scleredema.

Sclerosing skin diseases represent a large number of distinct disease entities, which include systemic sclerosis, localized scleroderma, and scleredema adultorum. These pathologies have a common clinical appearance and share histological features. However, the specific interplay between cytokines and growth factors, which activate different mesenchymal cell populations and production of different extracellular matrix components, determines the biomechanical properties of the skin and the clinical features of each disease. A better understanding of the mechanisms underlying these events is prerequisite for developing novel targeted therapeutic approaches.

BACKGROUND: Scleroderma is a term used to describe diseases that involve hardening and tightening of the skin and the underlying subcutaneous connective tissue. It could be localized to skin and subcutaneous tissue, or may involve the internal organs too in systemic sclerosis.
OBJECTIVE: There are disorders that can cause hardening and tightening of skin and mimic scleroderma but are rarely associated with Raynaud phenomenon, sclerodactyly, and autoantibodies in the serum, features specific to scleroderma/systemic sclerosis.
CONCLUSIONS: These are termed as \"scleroderma variants\" or \"scleroderma like disorders\". This review discusses the various \"scleroderma variants\" e.g. scleromyxedema, scleredema, nephrogenic systemic fibrosis, and eosinophilic fasciitis.

Scleredema is a deposition disorder which presents as diffuse, symmetric, non-pitting skin induration from dermal deposits of glycosaminoglycans (\"mucins\"). It classically affects the upper back and posterior neck, often causing skin tightness and decreased range of motion. In most patients the clinical course is chronic and treatment options are limited. We report a case of a patient diagnosed with scleredema associated with longstanding insulin dependent diabetes who was treated successfully with IVIg. Additionally, we have reviewed the literature reporting other patients with scleredema treated with IVIg.

Cutaneous fibrosing disorders encompass a diverse array of diseases united by the presence of varying degrees of dermal sclerosis. The quality and distribution of skin involvement, presence or absence of systemic complications and unique associated laboratory abnormalities often help to distinguish between these diseases. It is imperative that an effort is made to accurately differentiate between scleroderma and its mimics, in order to guide long-term management and facilitate implementation of the appropriate treatment modality where indicated.

Scleredema is a rare connective tissue disorder of unknown pathogenesis. Three types of scleredema have been described, based on its association with postinfection, monoclonal gammopathy and diabetes mellitus. We report herein a case of scleredema which the diagnosis didn\'t get specified. The patient was followed regularly for 13 years and did not respond to various combinations of immunosuppressants and psoralen plus ultraviolet A therapy. Treatment of scleredema is quite difficult and of limited success. At present, there is no proved treatment for this disease.