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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.

Scleredema, which also is known as scleredemaadultorum of Buschke, is an uncommonsclerodermiform condition that is characterizedby progressive thickening and hardening of theskin due to excessive dermal mucin and collagendeposition. The clinical course is variable, andprogression of disease may lead to functionalimpairment with limitations in mobility. The etiologyand pathogenesis are unknown although severalwell-known associations include streptococcalinfection; diabetes mellitus, particularly withmetabolic syndrome; and monoclonal gammopathy.We present a case of scleredema in a 52-year-oldman with no identifiable associated condition,who experienced improvemement with UVBphototherapy.

We read with interest the guidelines recently published on sclerosing diseases of the skin (Part 2: Scleromyxedema, scleredema and nephrogenic systemic fibrosis)[1, 2]. However, we are concerned that the guideline recommendations proposed for prevention of nephrogenic systemic fibrosis (NSF) are potentially dangerous. Although we recognise the challenges in constructing comprehensive guidelines, we are concerned that this may be because the guidelines have not involved a multidisciplinary team.

The new American College of Rheumatology/European League Against Rheumatism classification criteria will enable earlier diagnosis and, therefore, the use of newer treatment modalities for systemic sclerosis (SSc). It is therefore critical to exclude non-SSc causes for diffuse skin thickening as early as possible.The recently described gadolinium-induced nephrogenic systemic fibrosis may mimic SSc as may other conditions which require a different treatment strategy. Recently, treatment with immunoablation and autologous stem cell transplantation has been shown to significantly benefit some patients with conditions such as scleromyxoedema and SSc. The more accurate measurement of SSc-specific autoantibodies such as topoisomerase 1, centromere and RNA polymerase has recently allowed a more precise subclassification of SSc with implications for treatment and prognosis.Skin thickening is a nonspecific manifestation of many different processes including (rarely) early scleroderma, which is mostly symmetrical and associated with Raynaud's phenomenon, nailfold capillaroscopic changes and antinuclear antibodies. If the latter three factors are absent, then other conditions must be excluded, the commonest being eosinophilic fasciitis. Skin biopsy (looking for eosinophil infiltration, increased mucin or amyloid deposition), SSc-specific autoantibodies or paraproteins in blood and a careful medical history and system screening will exclude nonscleroderma conditions.

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.

SUMMARY Scleredema of Buschke (SB) is a rare disorder of connective tissue characterized by diffuse non-pitting induration of the skin, mainly on the cervical, deltoid and dorsal regions. It is a cutaneous mucinosis of unknown etiology and is associated with bacterial or viral infections, hematological disorders and diabetes mellitus. Histopathological examination shows thickened dermis with wide collagen bundles separated by gaps that correspond to mucopolysaccharide deposits, visualized using special staining. Several treatments are reported in the literature without well-established results. We report a case of SB in a patient with type 2 diabetes mellitus. RESUMO Escleredema de Buschke (EB) é doença rara do tecido conjuntivo caracterizada por endurecimento difuso e não depressível da pele, principalmente nas regiões cervical, deltoideanas e dorso. Enquadrado no grupo das mucinoses cutâneas, tem etiologia desconhecida e associação com: infecções bacterianas ou virais, alterações hematológicas e diabetes mellitus. O exame histopatológico evidencia derme espessada com fibras colágenas calibrosas separadas por fendas que correspondem a depósito de mucopolissacárides, observados por colorações especiais. Diversos tratamentos são relatados na literatura sem resultados bem definidos. Descrevemos caso de EB em paciente com diabetes mellitus tipo 2.

SUMMARY Scleredema of Buschke (SB) is a rare disorder of connective tissue characterized by diffuse non-pitting induration of the skin, mainly on the cervical, deltoid and dorsal regions. It is a cutaneous mucinosis of unknown etiology and is associated with bacterial or viral infections, hematological disorders and diabetes mellitus. Histopathological examination shows thickened dermis with wide collagen bundles separated by gaps that correspond to mucopolysaccharide deposits, visualized using special staining. Several treatments are reported in the literature without well-established results. We report a case of SB in a patient with type 2 diabetes mellitus.

A 61-year-old man with history of hypertension, type 2 diabetes, and hypothyroidism presented for evaluation of neck pain, stiffness, and occipital headaches lasting 4 years. Acetaminophen and meloxicam provided only partial relief. He also noted progressive thickening of skin in the nape of his neck. He denied any prior history of Raynaud phenomenon; heart, kidney, or liver disease; diabetic retinopathy; nephropathy; or neuropathy. He had quit smoking 24 years ago and reported currently drinking 3 glasses of wine per week. His medications included rosiglitazone, glimepiride, and levothyroxine.

The mucinoses of the type of scleredema and scleromyxedema are diseases marked by excessive production of mucin deposits in the skin and subcutaneous tissue, which causes skin hardening. The skin and subcutaneous deposits hamper the movement of limbs, the thorax as well as mouth. The same mechanism also damages other organs (the heart, lungs, oesophagus). It is probably caused by the stimulation of mucin production in fibroblasts by immunoglobulins, frequently monoclonal immunoglobulin. Therefore these diseases are typically associated with monoclonal gammopathy.We describe a cohort of 4 patients, skin manifestations were twice identified as scleredema and twice as scleromyxedema. All the four patients had type IgG monoclonal immunoglobulin and had clonal plasma cells in the bone marrow proven by histologic examination and flow cytometry. Therefore we commenced chemotherapy in all of them. In one case this chemotherapy was ended by a high-dose chemotherapy with transplanting of autologous red blood cells. This therapy attained the complete disappearance of monoclonal immunoglobulin as well as cutaneous and extracutaneous manifestations of scleredema (obstipation). In one case chemotherapy led to partial hematologic remission and partial improvement of skin manifestations. The other two patients did not respond to standard chemotherapy. The condition of one of them resulted in dermato-neuro syndrome (confusion, somnolence passing into coma and grand mal seizure) and improved following an intensive treatment including also intravenous application of immunoglobulins in a dose of 2 g/per 1 kg weight. This patient has now been under long-term treatment with these immunoglobulins, during which the skin symptoms have significantly diminished, but the concentration of monoclonal immunoglobulin has not changed. The fourth patient not responding to standard chemotherapy was treated with intravenous immunoglobulins also in a dose of 2 g/per 1 kg of weight 1× in a month. After 4 applications the thickening of skin and subcutaneous tissue moderately diminished, so the range of possible movement of the upper limbs and neck became larger and the itchy skin morphs which accompanied the disease disappeared completely.It is possible to use chemotherapy and high-dose chemotherapy in the treatment of mucinosis associated with monoclonal gammopathy, as in the treatment of multiple myeloma. If such treatment is not possible or it has not attained disappearance of monoclonal immunoglobulin, improvement can be achieved through repeated application of intravenous immunoglobulins. The treatment with intravenous immunoglobulins in an immunomodulation dose of 2 g/per 1 kg of weight effects the moderation of skin manifestations, but it does not lead to the decrease in monoclonal immunoglobulin.

Aim To recognize a line of treatment for scleredema of Buschke in an adolescent. Background Scleredema of Buschke is an uncommon disorder characterized by induration of the skin, which includes a non pitting hardening of the skin around the neck, shoulders, and trunk sometimes the face. Three variants are recognized. The histopathologic features of scleredema are characterized by thickened collagen bundles within the reticular dermis that are separated by mucin containing fenestrations. No consistent treatment modality is currently followed. Case report The present case report describes scleredema of Buschke in a 10-year-old female child reported with stiffness of facial skin and difficulty in opening the mouth. The patient was treated with antibiotics and vitamin supplements and there was drastic improvement with decrease in skin stiffness and increase in mouth opening. The stiffness of facial muscles decreased and mouth opening increased. Conclusion Multiple treatment modalities for scleredema have been used. In the present case, high-dose antibiotic therapy was used with good prognosis. Clinical significance High-dose antibiotic therapy can be used for treatment of dermatologic disorders with reasonably good prognosis. How to cite this article Rani JD, Patil SG, Murthy STS, Koshy AV, Nagpal D, Gupta S. Juvenile Scleredema of Buschke. J Contemp Dent Pract 2012;13(1):111-114.

Background Scleromyxedema is associated with a monoclonal gammopathy and other comorbidities. Its prognostic and therapeutic features are poorly documented because most reports deal with single cases or small series. Objective We sought to describe the characteristics of patients with scleromyxedema regarding demographics, clinical characteristics, comorbidities, therapeutic interventions, and course. Methods We conducted a retrospective and prospective multicenter study. Results We identified 30 patients with scleromyxedema (17 men and 13 women). The mean age at diagnosis was 59 years. The mean delay between disease onset and diagnosis was 9 months. Monoclonal gammopathy was detected in 27 patients. Extracutaneous manifestations were present in 19 patients including neurologic (30%), rheumatologic (23.3%), and cardiac (20%) manifestations. Two patients developed hematologic malignancies. The most common therapies included oral steroids and intravenous immunoglobulins. Although corticosteroids were ineffective, intravenous immunoglobulins (alone or in combination with other drugs) induced complete remission in 4 and partial remission in 9 patients with a mean treatment duration of 2 years. In all, 21 patients were followed up for a mean period of 33.5 months, at which time 16 patients were alive, 12 with and 4 without skin disease. Five patients died: 2 with dermatoneuro syndrome and 1 each with myeloid leukemia, Hodgkin lymphoma, and myocardial insufficiency. Limitations This is mainly a retrospective study. Conclusions Our study confirms that scleromyxedema is a chronic and unpredictable disease with severe systemic manifestations leading to a guarded prognosis. There is no specific definitive treatment. Our data support the contention that intravenous immunoglobulin is a relatively effective and safe treatment. The response is not permanent and maintenance infusions are required.

Scleroderma is a rare systemic autoimmune disease with multiple organ manifestations, including skin fibrosis. The groups of disorders classified as scleroderma mimics share the common thread of skin thickening but are otherwise quite incongruous in terms of underlying disease process and other organ involvement. This article reviews the clinical presentation, etiology, and treatment options available for scleroderma mimics, including morphea, scleredema, diabetic cheiroarthropathy, scleromyxedema, nephrogenic systemic fibrosis, and eosinophilic fasciitis. Through greater understanding of these diseases and the associated extradermal implications, we hope to facilitate recognition of scleroderma and its mimics.

Buschke Scleredema is a rare connective tissue disorder of unknown aetiology, characterized by thickening of the dermis whose characteristics may mainly to mime systemic sclerosis, eosinophilic fasciitis and cutaneous amyloidosis. Scleredema may be associated with a history of an antecedent febrile illness, diabetes mellitus, or blood dyscrasia. Scleredema can be classified into three clinical groups; each has a different history, course, and prognosis. Each one of these share reduction in chest articular movements and limitation of limbs movements. The skin histology is characterised by thickened dermis and increased spaces between large collagen bundles due to increased deposition of mucopolysaccharide in the dermis. Differential diagnosis can be made considering the typical clinical features and the histologic peculiarity. No therapy has been found effective. The authors describe a case of Buschke Scleredema successfully treated by steroids and colchicine. Clinical evaluation of skin induration and thickness as well as ultrasonography were performed at baseline and after treatment.

ResumenPresentamos la adaptación española realizada por el Comité Español Interdisciplinario para la Prevención Cardiovascular (CEIPC) de la Guía Europea de Prevención de las Enfermedades Cardiovasculares 2008. Esta guía recomienda el modelo SCORE de riesgo bajo para valorar el riesgo cardiovascular. El objetivo es prevenir la mortalidad y la morbilidad debidas a las enfermedades cardiovasculares (ECV) mediante el tratamiento de sus factores de riesgo en la práctica clínica. La guía hace énfasis en la prevención primaria y en el papel del médico y el personal de enfermería de atención primaria en la promoción de un estilo de vida cardiosaludable, basado en el incremento de los grados de actividad física, la adopción de una alimentación saludable y, en los fumadores, el abandono del tabaco. La meta terapéutica para la presión arterial es en general

Buschke Scleredema is a rare connective tissue disorder of unknown aetiology and characterized by thickening of the dermis. It resembles systemic sclerosis, eosinophilic fasciitis and cutaneous amyloidosis. Scleredema may be associated with a history of an antecedent febrile illness, diabetes mellitus, or blood dyscrasia. It may be classified into three clinical groups, each has a different history, course, and prognosis. Histology of skin is characterized by thickened dermis and increased spaces between large collagen bundles due to increased deposition of mucopolysaccharide in the dermis. Differential diagnosis can be made considering the typical clinical features and the histological peculiarity. No therapy is effective. In this communication we have presented a 54 year old man with scleredema successfully treated by PUVA and methotrexate. We reviewed associated diseases, clinical and histopathological characteristics, evolution and response to treatment.

Scleredema adultorum of Buschke is a rare disorder characterized by diffuse swelling and non-pitting induration of the skin usually involving the face, neck, arms and upper trunk. It has been associated with previous infectious diseases, diabetes, paraproteinemia and, more rarely, malignant neoplasms or autoimmune disorders. We report the case of a 30-year-old man who presented with a 2-year history of scleredema. Further investigation led to the diagnosis of primary Sjögren’s syndrome. The association between scleredema and autoimmune disorders has been rarely seen. To our knowledge, there are no other reports describing the association between primary Sjögren’s syndrome and scleredema adultorum of Buschke.

Summary Scleredema of Buschke or scleredema diabetorum is a skin complication of diabetes with deposits of collagen and aminoglycans in the dermis. This disease characterized by thickening and hardening of the skin, is usually localized in nape, back and shoulder areas. Consequences could be a decrease in motility of the shoulders and an impairment of respiratory function. Other possible complications are sleep apnoea syndrome and monoclonal gammapathy. Type 1 or type 2 diabetes may be associated with scleredema of Buschke in more than 50% of cases. Diabetes-related risk factors are long duration of the disease, presence of microangiopathy, overweight and need of insulin. Various specific treatments proposed in the literature are poorly validated. In most severe cases, radiation therapy may be useful.

Background: The efficacy and safety of UVA1 (340–400 nm) phototherapy were established by studies from European countries. Purpose: Evaluate experience with UVA1 phototherapy for patients with cutaneous diseases in the United States. Methods: A retrospective analysis of 92 cases of UVA1-treated cutaneous conditions from four medical centers in the United States was performed. Results: Two-third of the patients showed a fair to good response (26–100% improvement) and one-third of the patients showed a poor response (0–25% improvement). Diseases with a moderate to good response (51–100% improvement) included scleredema adultorum, hand or foot dermatitis, atopic dermatitis, morphea (medium or medium- to high-dose UVA1), systemic sclerosis, and urticaria pigmentosa. Besides tanning, other adverse effects were found in 15% of patients, which include pruritus, erythema, tenderness, and burning sensation. Patients with skin types I–III responded better that those with a darker skin type. Conclusion: UVA1 phototherapy is a useful and well-tolerated treatment option for a variety of skin conditions.

Objectives To present a case of scleredema adultorum of Buschke associated with hypergammaglobulinemia and review the literature pertaining to this disease. Methods Search of MEDLINE (PubMed) was performed using the words "Scleredema Adultorum." Only cases in the English language, with pertinent clinical information for analysis, were included. Results We present a case of scleredema associated with hypergammaglobulinemia. The skin findings had been progressive for 2 years before he was diagnosed with scleredema. Our patient was found to have a B-cell lymphoma before being diagnosed with scleredema. The progression of skin thickening halted with no apparent correlation to immunosuppressive therapies or chemotherapeutic agents. A total of 165 cases of scleredema adultorum are described in the literature. There are 3 types of scleredema adultorum. Type 1 is usually preceded by a febrile episode and resolves spontaneously. Type 2 is associated with developing paraproteinemias including multiple myeloma. Type 3 is associated with diabetes mellitus. Conclusions Scleredema adultorum of Buschke is a rare disorder which is sometimes associated with hypergammaglobulinemia; our patient had a B-cell lymphoma. Physicians should suspect scleredema in any patient with diffuse skin thickening where the hands and feet are spared, particularly if diabetes or a preceding febrile episode were present. Once the diagnosis of scleredema is made, evidence for the presence of hypergammaglobulinemia should be sought.

Scleredema (also called scleredema of Buschke) is a fibromucinous connective tissue disorder of unknown cause that belongs to a group of scleroderma-like disorders. We report the case of a 64-year-old lady with long-standing scleredema, associated with a paraprotein, and progressing to multiple myeloma and AL amyloidosis. The relationship of scleredema with paraprotein and multiple myeloma is well established, but only two cases of scleredema associated with amyloidosis have been reported to date. We suggest that amyloidosis may be underdiagnosed in patients with scleredema and paraproteinaemia. Features attributed to extracutaneous manifestations of scleredema could represent systemic amyloidosis. We review published reports of scleredema associated with paraprotein and discuss the difficulties in the differential diagnosis of scleroderma-like disorders. We discuss the diagnosis of plasma cell dyscrasias and amyloidosis and their relevance in rheumatology practice.

Scleredema adultorum is a rare sclerotic disorder characterized by diffuse swelling and nonpitting induration of the skin. Its occurrence has been documented in association with infections, diabetes mellitus, paraproteinemia, multiple myeloma, and monoclonal gammopathy. We report an unusual case of a 48-year-old man with an asymptomatic bilateral eyelid edema of sudden onset. During a period of 6 months, the condition slowly progressed to extensive nonpitting edematous swelling restricted to the periorbital sites. The presumptive diagnosis of scleredema adultorum was confirmed by the presence of typical histologic findings. This case is unique in that the periorbital swelling remained as the sole clinical manifestation of scleredema during the 5-year follow-up and was complicated with partial vision blockage.

Scleredema adultorum Buschke is a rare disorder characterized by thickening of the dermis of the neck, head and upper trunk. Its etiology is unknown, but there may be a preceding history of infection and there is a known association with diabetes mellitus. Women are more frequently affected. Usually, the disease is self-limiting but some patients show progressive disease. In these cases therapeutic options are poor, with only case reports and small series supporting their use.A 58-year-old patient with a scleredema of the neck and upper trunk is described, who was treated twice within 6 months by electron-beam radiation therapy. After the second course his symptoms improved significantly. A review of the literature of radiation treatment of this disease is given.. Regardless of the possible mechanisms in pathogenesis and treatment of scleredema adultorum Buschke, the application of ionizing radiation is an important, effective and well-tolerated therapy option in the treatment of severe cases and may candidate as the first-line treatment of this disease.

Two children with chronic and asymmetric skin indurations are presented. The clinical and pathologic features are suggestive of asymmetric childhood scleredema and stiff skin syndrome. The key features of scleredema and stiff skin syndrome are discussed.

A 53-year-old woman suffering from brown plaques occasionally ulcerating at both lower legs and in the course of disease on the back of the foot presented at our department. Moreover she complained about an increasing sclerosis of her skin at the back over the last 15 years. She had suffered from diabetes mellitus type-1 for more than 40 years.Skin biopsies form the ulcerated plaques showed changes typical for necrobiosis lipoidica. Biopsies of the back were diagnosed as scleredema adultorum Buschke. Laboratory tests displayed a normal antinuclear antibody titer. The differential diagnosis of systemic sclerosis could be ruled out.We repeatedly performed debridement of ulcers, cream-PUVA therapy, applied a vacuum-sealing and topical corticosteroids at the lower legs. Meanwhile she received oral pentoxyfillin. The scleredema was treated with UVA-1 phototherapy but had to be stopped for high photosensitivity. Additionally physiotherapy was prescribed. Nonetheless the course of disease was chronic and therapy-resistant.Skin diseases are common in diabetes mellitus. Necrobiosis lipoidica and scleredema adultorum Buschke are rare complications but often refractory to treatment.

We present a case of scleredema with a leonine facies in a 56-year-old man with a history of poorly controlled diabetes mellitus. The patient initially presented with erythematous, edematous papules and plaques on the face, neck, and upper back.

A woman, 74 years of age, presented to the emergency department with a lesion on the lower abdominal wall that had started a month earlier and was not associated with any other symptoms. Her family physician had treated it with emollient creams. Relevant past medical history included congestive heart failure, hypertension, hypertensive heart disease, pulmonary hypertension, mitral regurgitation, chronic atrial fibrillation, rosacea and diabetes mellitus that was being treated with oral hypoglycaemics (metformin). Physical examination revealed an area of skin on the lower abdominal wall that was ill-defined and indurated, with whitish papules and a 'cobblestone' appearance (Figure 1).

A non-invasive, in vivo suction device was used to investigate the mechanical properties of the skin in a patient with scleredema of Buschke. Clinical scoring of skin induration and measurements of skin elasticity were performed over 9 anatomic regions on admission and after 3 (on discharge), 17 and 28 months. Immediate distension, final distension and immediate retraction were significantly decreased, while the viscoelastic to elastic ratio was significantly increased in the patient as compared to the healthy controls. Delayed distension and biological elasticity were preserved. Low values of skin distensibility correlated with a severe skin induration (p0.001). The changes were more expressive with the 8 mm-diameter measuring probe than the 2 mm-diameter probe. The method applied can be used for objective and quantitative assessment of skin involvement in scleredema of Buschke.

We report a patient with the typical lesions of diabetic scleredema. Histological findings of the involved skin were thickening of the dermis, depositions of mucins, and fibrosis. Biochemical analysis revealed an increase in glycosaminoglycans in the involved skin as well as in the cutaneous lupus mucinosis. Mucinous materials were composed of hyaluronic acid.

Acanthosis nigricans (AN) and scleredema are two skin conditions that have been reported in association with diabetes mellitus. Few cases associating scleredema and AN have been reported. A literature search did not reveal any reports of diabetic patients developing AN on top of a scleredema plaque. Here we report a patient with diabetes mellitus who developed AN within the same indurated scleredema plaque.

Skin fibrosis is involved in several pathologies as hypertrophic scar or scleroderma. The determination of the mechanisms at the origin of these problems is however difficult due to the low number of in vivo models. To bypass this absence of animal models, studies typically use human pathological cells cultured in a monolayer way on plastic. However, cell behavior is different according to the fact that cells are on plastic or embedded in matrix. Using a tissue engineering method, we have developed new in vitro models to study these pathologies of the skin. Human pathological cells are used to reconstitute a three dimensional fibrotic tissue comprising the dermal and the epidermal parts of the skin. This method is called the self-assembly approach and is based on the cell capacity to reconstitute in vitro their own environment as in vivo. In this chapter, protocols generating reconstructed pathological skin using this approach are detailed. The methods include extraction and culture of human skin keratinocytes and fibroblasts from very small cutaneous biopsies. In addition, a description of the protocols for the production of fibrotic dermal sheets can be found to obtain a model of fibrotic dermis that can be associated or not with a fully differentiated epidermis.

Skin manifestations associated with diabetes mellitus are common and, with close scrutiny, can occur in most, if not all, patients. Little information is available on what common pathophysiologic thread is responsible for the skin manifestation and diabetes. Although controlled studies are lacking, a recognition of the skin manifestations, treatment, and prognosis will help patients and their caregivers to understand treatment alternatives more clearly.

Maximilian C. Aichelburg, MD; Robert Loewe, MD; Nikolaus Schicher, MD; Paul-Gunther Sator, MD; Franz M. Karlhofer, MD; Georg Stingl, MD; Ahmad Jalili, MD, PhD; Divisions of Immunology, Allergy, and Infectious Diseases (Drs Aichelburg, Karlhofer, Stingl, and Jalili) and General Dermatology (Drs Loewe and Schicher), Department of Dermatology, Medical University of Vienna, and Department of Dermatology, Hietzing Municipal Hospital (Dr Sator), Vienna, Austria

Scleredema is a rare connective disease which must be differentiated from scleroderma in childhood. Scleredema is characterized by thickening of the dermis of the neck, head, and upper trunk. We report a case of scleredema in an 8-year-old boy with coincident streptococcal colonization. The patient report demonstrates many of the common features of scleredema, including an associated streptococcal infection, a relatively benign presentation of illness, and the characteristic mucopolysaccharide intradermal staining on skin biopsy. The literature on scleredema is reviewed, focusing on the disease course, differential diagnosis, and an overview of the proposed three subgroups of scleredema. The association of scleredema to a prior streptococcal infection is explored, and a proposed autoimmune pathophysiology of the disease, as it relates to streptococcal infection, is presented.

Scleredema adultorum (SA) is a rare sclerotic disorder characterized by non-pitting induration of the neck with acral progression, sparing hands and feet. We report on a 57-year-old male with severe SA associated with paraproteinemia, treated with methotrexate. Such widespread skin thickening followed by severe movement restriction and inability to function on daily basis, as in our patient, has never been described. Severe osteoarthritis and finding of HLA-B39 allele in association with SA has not been previously described either. To the best of our knowledge, up to 40 patients with SA associated with paraproteinemia has been reported so far, and currently, there is no established effective treatment protocol. In our patient, low-dose methotrexate resulted in stiffness reduction, increased motility of the trunk and extremities, and ability to function on daily basis. We believe that any information about treatment outcome in SA patients should be disseminated in order to establish consensual treatment protocol for this rare disease.

We report the case of a patient with a 20-year history of diabetes mellitus type 2 who developed sclerotic skin lesions on his neck and upper back. Physical and histological findings were compatible with diabetic scleredema. T(2)-weighted magnetic resonance imaging (MRI) revealed diffuse thickening of the dermis and subcutaneous tissue, with hyperintense signals. The MRI findings in the dermis corresponded with the accumulation of collagen tissue with deposition of glycosaminoglycans.

Scleredema adultorum is characterized by induration of the skin on the neck, shoulders and upper back caused by increased accumulation of collagen and aminoglycans in the dermis. The induration may progress and lead to pronounced restriction of mobility. Scleredema diabeticorum is one type of scleredema adultorum associated with diabetes mellitus. Multiple therapies have been tried, but most of them have not proven to be consistently effective. We report two cases of scleredema diabeticorum treated successfully with UVA-1- as well as physiotherapy and topical corticosteroids; this approach led to improvement in skin changes and mobility.

Scleredema adultorum case report

Scleredema is an uncommon condition characterized by induration of skin. The pathogenesis is not known, although the increased expression of collagen-producing fibroblasts in the skin has been demonstrated. Scleredema has been difficult to treat with scattered reports of success with bath psoralen plus ultraviolet A (PUVA), cream PUVA, UVA1 and others. The use of low-dose broad-band UVA phototherapy was found to be effective in the treatment of morphea. Colchicine has been shown to interfere with collagen synthesis and is used successfully in scleroderma. We report the first case of scleredema that improved markedly with low-dose broad-band UVA plus colchicine treatment.