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Scleredema is a rare cutaneous mucinosis characterized by chronic diffuse induration of the skin, and it is occasionally associated with a monoclonal gammopathy (MG). Ankylosing spondylitis (AS) is noted to be another, chronic systemic inflammatory disorder of the axial skeleton that may accompany the MG. However, patients with scleredema and AS accompanied with a MG have not been reported in the literature. We here report a 40-yr-old man with scleredema and advanced AS accompanied with a MG of IgA-kappa protein. Widespread, long-standing scleredema has been developed over 10 yrs after the initial manifestation of AS. It is uncertain whether the coexistence of scleredema and AS is more than coincidental.

The cutaneous mucinoses are a complex group of dermatologic diseases with local, follicular, or diffuse disease. The diffuse cutaneous mucinoses are remarkable not only for their dermal disease, but also for the numerous systemic manifestations. It is important that the clinical dermatologist be able accurately to diagnose and differentiate scleredema, scleromyxedema, REM, generalized myxedema of hypothyroidism, and pretibial myxedema of hyperthyroidism. Because of the variability of associated systemic manifestations, some with substantial morbidity and mortality, accurate diagnosis is vital for awareness and appropriate management.

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.

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.

Unlabelled: A 6-year-old female patient presented with a rapidly progressive scleroderma-like syndrome involving almost the entire integument. Initially clinical patterns and histopathological data of both eosinophilic fasciitis and scleredema adultorum were present. The course of the disease remained unusual for both conditions but finally argued in favour of the diagnosis of eosinophilic fasciitis., Conclusion: Eosinophilic fasciitis and scleredema adultorum might be subtypes of one disease entity.

Scleredema adultorum is a rare connective tissue disorder reported usually following streptococcal infection, influenza, measles, and mumps. It has been reported occasionally following trauma and tuberculous lymphadenitis. This is a report of scleredema adultorum developing after chicken pox in an eight-year-old male child. The diagnosis was established by characteristic picture on skin biopsy using special stain. The patient had a benign course and a spontaneous recovery in two weeks. The case has been reported as the first case of scleredema adultorum developing after chicken pox.

Scleredema adultorum of Buschke is a rare disorder of unknown aetiology. It is characterized by diffuse, non-pitting swelling and induration of the skin. Skin biopsies reveal marked thickening of the dermis due to collagenous replacement of the subcutis and deposition of hyaluronic acid between the collagen fibers. The disease classically only affects the skin. In 24 cases an associated monoclonal gammopathy has been detected. A 75-year-old patient had a 19-year history of scleredema adultorum. In addition to a monoclonal gammopathy the patient suffered from involvement of the tongue, pharynx and upper esophagus. Furthermore a polyneuropathy, ocular involvement with restricted eye movements and a sicca syndrome were present. The simultaneous occurrence of cutaneous scleredema with any one of the above mentioned symptoms has been reported before. The wide variety of extracutaneous manifestations of scleredema as found in our patient is amazing and has not been previously described.

A 46-year-old white man had classic findings of scleredema; subsequently, a monoclonal immunoglobulin G lambda light chain was detected in his serum. Findings of a bone marrow biopsy specimen revealed that he had an increased percentage of plasma cells, some of which were atypical. A diagnosis of scleredema and smoldering myeloma was made.

Death due to scleredema is rare. We report a patient with scleredema, which had an acute onset and rapid progression, leading to death within a month. Such an acute course terminating in fatality has not been described earlier.

We studied 26 formalin-fixed biopsy specimens taken from 22 patients with scleredema and found positive staining in 67% of the tissues when both colloidal iron and alcian blue methods were used. Single stains were positive in only half the tissues. Positive findings were noted in both patients with diabetes (11 patients) and in those without it (11 patients). Our experience indicates that multiple biopsies may be necessary to demonstrate mucin in the dermis. Specimens obtained by incisional biopsy are preferred.

Heart failure as a complication of Buschke's scleredema was not reported in the literature on this rare disease. A young girl with a severe form of Buschke's disease is described. She developed severe right-sided heart failure--proven by the typical clinical, auscultatory, electrocardiographic and echocardiographic findings; was treated successfully with penicillin, anticongestive measures and steroids, and showed a remarkable complete recovery.

A review of thirty-three cases of scleredema, with particular reference to clinical and histologic findings, revealed that the disease was often of insidious onset and the course was usually prolonged. A preceding respiratory tract infection was uncommon and usually did not indicate a short course. The patients were divided into two groups according to the presence or absence of diabetes. Diabetes, when present, was typically the late-onset, insulin-dependent type and difficult to control. In many of these patients, the onset of scleredema was so subtle that it went unnoticed by the patient until pointed out by an examining physician.

An adequate biopsy that includes the subcutaneous tissue is very helpful in the evaluation of patients with suspected connective tissue disease. However, the histologic features must often be correlated with the clinical features and other laboratory tests for a definitive diagnosis. An objective histologic diagnosis can usually be made in scleroderma, lupus erythematosus panniculitis, amyloidosis, and angioedema, and in diseases such as lepromatous leprosy and mycosis fungoids (which are sometimes confused with connective tissue diseases). Correlation with clinical features and, sometimes, other laboratory tests is often required to establish a diagnosis of scleredema, dermatomyositis, myxedema, and lichen myxedematosus. The features in cheilitis granulomatosa usually are not specific, but a biopsy is helpful to rule out other diseases.

A 3-month-old infant with scleredema had associated cytomegalovirus pneumonia with a rapidly fatal course. Because differentiation of scleredema from scleredema neonatorum, subcutaneous fat necrosis, and scleroderma may be difficult, histologic diagnosis is important. Cetylpyridinium chloride was used successfully as a fixative to demonstrate acid mucopolysaccharides histologically.

The skin thickness in a group of patients with scleredema was measured using ultrasonic techniques and then compared with a group of normal individuals. The mean dermal thickness in scleredema was 7.7 mm, compared to 3.3 mm in the normal control group. Ultrasonic evaluation of skin thickness may be of value in following the natural history or response to therapy of certain diseases that affect dermal thickness.

A case of scleroedema is reported in a 28-year-old man with severe neuromuscular involvement. Failure in neural conduction as well as muscular degeneration and necrosis were seen. Histopathological study showed resorption and formation of scar tissue probably secondary to ischaemia. In addition there was increase in neutral, sulphated and non-sulphated mucosubstances in the dermal and endomysial connective tissue.

A 55-yr-old white female with biopsy proven scleredema adultorum was evaluated for progressive dysphagia. Esophageal motility studies revealed persistently high upper esophageal pressures without response to deglutition, and minimal peristalsis in the upper body of the esophagus. A cine-esophagram revealed massive aspiration of barium into the nasopharynx and lungs, concommitant with stenosis in the upper esophagus. The patient died presumptively from aspiration. This patient exemplifies a potentially lethal complication of scleredema adultorum i.e., upper esophageal infiltration with resultant aspiration.

Sclerotic changes in the skin are most frequently caused by scleroderma. In those cases in which scleroderma is not implicated, careful historical, physical, histologic, and laboratory evaluation will allow an appropriate diagnosis to be made. In this way, appropriate therapy and prognostication can be given.