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The records from 12 Chinese adult patients with scleredema, who had attended the Social Hygiene Service of the Hong Kong Department of Health between 22 January 1990 and 19 March 1996, were retrieved and analysed. The neck was the commonest site of involvement (75%), followed by the back (42%), and the shoulder (17%). The vast majority (83%) of scleredema cases were associated with diabetes mellitus; half of these were insulin-dependent. Most of the patients (92%) had hypertension for which medical treatment was needed. No cases of skin disease were preceded by acute infection, and none had any associated paraproteinaemia. The degree of skin involvement did not affect the daily activities of most of the patients. This study revealed differences between the disease in our locality and those described in the western literature.
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PMID:Scleredema in Chinese patients: a local retrospective study and general review. 1183 50

Scleredema adultorum, or Buschke's scleredema, belongs to the group of mucinoses. It is characterised by thickened and indurated skin. Histopathology shows thickened dermis with an infiltration of mucin between swollen collagen bundles. There are reports about many associations with scleredema adultorum, e.g., with diabetes mellitus and multiple myeloma. One case is known with associated primary hyperparathyroidism. For the first time we report a case of scleredema adultorum and secondary hyperparathyroidism, in a 46-year-old patient. Both forms of hyperparathyroidism have increased levels of parathormone. Therefore, these increased levels could have an influence on collagen metabolism.
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PMID:[Scleredema adultorum in secondary hyperparathyroidism]. 1196 92

Heightened awareness of endocrine abnormalities is important in evaluation of patients presenting with musculoskeletal symptoms. Endocrine disorders such as diabetes, hyperthyroidism, hypothyroidism, hyperparathyroidism, hypoparathyroidism, hyperadrenocorticism, and acromegaly cause a unique array of rheumatic manifestations. Such conditions include Dupuytren's contracture, carpal tunnel syndrome, chondrocalcinosis, pseudogout, scleredema, and osteoporosis. Characteristic changes on radiologic evaluation and serum enzyme testing are additional clues to these atypical presentations. Consideration of a possible endocrine cause early in the evaluation may improve management in patients with such an underlying disorder.
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PMID:Endocrine origins of rheumatic disease. Diagnostic clues to interrelated syndromes. 1198 36

A 41 year male, diabetic on treatment presented with persistent erythema and. "taut" skin over neck and back since 1 year. The lesions showed symmetric and gradual progression. There was no history of prior sore throat. On examination effected skin was erythematous, woody hard and unpinchable. Scleredema diabeticorum (diutinum), although sharing clinical and historical features with Scleredema adultorum has no prodromal infection, is more extensive and affected individuals are characteristically obese, middle aged diabetics who often have accompanying microangiopathies and macroangiopathies. Recognition of scleredema by the physician has prognostic and therapeutic implications in the management of the coexisting diabetes.
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PMID:Scleredema diabeticorum--a case report. 1264 48

Dermatologists may commonly see skin lesions that reflect an underlying endocrine disorder. Identifying the endocrinopathy is very important, so that patients can receive corrective rather than symptomatic treatment. Skin diseases with underlying endocrine pathology include: thyrotoxicosis; hypothyroidism; Cushing syndrome; Addison disease; acromegaly; hyperandrogenism; hypopituitarism; primary hyperparathyroidism; hypoparathyroidism; pseudohypoparathyroidism and manifestations of diabetes mellitus. Thyrotoxicosis may lead to multiple cutaneous manifestations, including hair loss, pretibial myxedema, onycholysis and acropachy. In patients with hypothyroidism, there is hair loss, the skin is cold and pale, with myxedematous changes, mainly in the hands and in the periorbital region. The striking features of Cushing syndrome are centripetal obesity, moon facies, buffalo hump, supraclavicular fat pads, and abdominal striae. In Addison disease, the skin is hyperpigmented, mostly on the face, neck and back of the hands. Virtually all patients with acromegaly have acral and soft tissue overgrowth, with characteristic findings, like macrognathia and enlarged hands and feet. The skin is thickened, and facial features are coarser. Conditions leading to hyperandrogenism in females present as acne, hirsutism and signs of virilization (temporal balding, clitoromegaly).A prominent feature of hypopituitarism is a pallor of the skin with a yellowish tinge. The skin is also thinner, resulting in fine wrinkling around the eyes and mouth, making the patient look older. Primary hyperparathyroidism is rarely associated with pruritus and chronic urticaria. In hypoparathyroidism, the skin is dry, scaly and puffy. Nails become brittle and hair is coarse and sparse. Pseudohypoparathyroidism may have a special somatic phenotype known as Albright osteodystrophy. This consists of short stature, short neck, brachydactyly and subcutaneous calcifications. Some of the cutaneous manifestations of diabetes mellitus include necrobiosis lipoidica diabeticorum, diabetic dermopathy, scleredema adultorum and acanthosis nigricans.
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PMID:Cutaneous manifestations of endocrine disorders: a guide for dermatologists. 1268 37

A 72-year-old man with diabetes-associated scleredema is presented. The patient had a long history of diabetes mellitus that had been difficult to control with complications of retinopathy, nephropathy, and arteriosclerosis leading to myocardial infarcts and stroke. The scleredema has remained stable with 4 months of topical clobetasol ointment twice daily and biweekly physical therapy. Diseases associated with scleredema and therapeutic options are summarized.
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PMID:Scleroderma. 1459 84

We describe the case of a 55-year-old man with scleredema of Buschke of the torso complicated by insulin-dependent diabetes mellitus. Due to (i) the patient's poor general health status, (ii) the similarity between scleroderma and scleredema of Buschke, and (iii) the well known efficacy of factor XIII infusions in scleroderma, we attempted an intravenous treatment with factor XIII. This therapy resulted in marked increase of movements and in softening of the skin, together with ultrasonographic and histopathological improvements. In conclusion, to the best of our knowledge, this is the first case in which factor XIII has been successfully used for the treatment of scleredema of Buschke.
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PMID:Scleredema of Buschke: remission with factor XIII treatment. 1557 89

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.
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PMID:Acanthosis nigricans in a plaque of scleredema on the back of a diabetic patient: a case report. 1566 60

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.
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PMID:Scleredema adultorum of Buschke presenting as periorbital edema: a diagnostic challenge. 1569 12

A 52-year-old patient with rheumatoid arthritis (RA) developed scleredema-like skin induration after treatment with the tumor necrosis factor alpha (TNFalpha) blocking agent, infliximab. Skin induration occurred within a few weeks of initiation of infliximab, resolved with discontinuation of the drug, and recurred with rechallenge with the drug, implicating infliximab as the offending agent. Laboratory evaluation revealed a high titer of human antichimeric antibodies (HACA). The skin induration improved within a few weeks of discontinuation of infliximab and did not recur with the use of etanercept. Scleredema has been reported in association with bacterial and viral infections, diabetes mellitus, and monoclonal gammopathies. Infliximab use should be added to the list of potential associations with scleredema. This effect appears possibly specific to infliximab and may be related to the development of HACA because it did not occur with the use of etanercept in this patient. In addition, there appears to be a complex relationship between TNFalpha and tumor growth factor beta (TGF-beta), a cytokine which promotes collagen synthesis and deposition. TNFalpha blockade with infliximab may affect TNFalpha-TGF-beta interactions and may be implicated in the development of scleredema in this case.
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PMID:Infliximab-induced scleredema in a patient with rheumatoid arthritis. 1637 2


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