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Dermal manifestations of diabetes
Published in Robert A. Norman, Geriatric Dermatology, 2020
Three aspects of skin changes in this group of patients were demonstrated; one group had clinically inapparent, but measurable changes; another group had clinically apparent changes ranging from pebbled skin to scleroderma-like changes; and an infrequent syndrome of scleredema developed in some that resulted in marked thickening of the skin of the upper back9. Support for this comes from the finding that patients with type I diabetes, even those without clinical evidence of skin thickening, showed changes when biopsies were examined and compared with controls19. Hundley and Walter demonstrated that increased skin thickness was not universal in all diabetics, nor that it was necessarily even over all parts of the body20. It has also been shown that thickening occurs predominantly on the extremities9.
Differential diagnoses of systemic sclerosis
Published in Aparna Palit, Arun C. Inamadar, Systemic Sclerosis, 2019
Scleredema is a disorder characterized by symmetrically indurated skin due to excessive dermal mucin deposition. Although termed as scleredema adultorum (of Buschke) and frequently affecting middle-aged obese adults, children can be affected as well. Etiologically, scleredema can be grouped into diabetes-associated (commonly with uncontrolled type II diabetes mellitus), post-infectious (streptococcal upper respiratory tract infection, influenza, mumps, measles, varicella, cytomegalovirus, diphtheria, encephalitis, and dental abscesses), and with paraproteinemias. Exact pathogenesis of scleredema is unclear. Nonenzymatic glycosylation of collagen and stimulation of fibroblast proliferation by chronic hyperinsulinemia are implicated in diabetic scleredema. The induration is non-pitting and predominantly involves the posterior neck and upper back and may extend on to the face and proximal extremities. The affected area appears erythematous with a peau d’ orange appearance (mattress sign) and has a woody hard consistency. Scleredema associated with diabetes (scleredema diutinum) and paraproteinemia are usually progressive and unresponsive to treatment. The postinfectious form carries a good prognosis and usually resolves in a few months to couple of years.17,18
Skin Changes Associated With Diabetes
Published in Jack L. Leahy, Nathaniel G. Clark, William T. Cefalu, Medical Management of Diabetes Mellitus, 2000
Most cases of this rare condition, scleredema adultorum, are preceded by a febrile illness, often streptococcal. The condition develops slowly over a period of weeks to months, reaches a peak and then gradually recedes over a period of months. However, the condition can be seen in association with diabetes, usually in obese diabetics with severe long-standing disease. The etiology of thick skin and scleredema is unknown, but may be related to the effect of hyperglycemia and hyperinsulinemia on the metabolism of collagen and ground substance in the dermis. There is sometimes an associated paraproteinemia, in which there is a monoclonal gammopathy of IgG, IgM, or IgA. Paraproteinemias have occasionally been associated with thickening of skin, with deposits of mucin, but biopsies of scleredema usually show no increase or only slight increase in dermal mucin, the predominate finding being swollen collagen bundles and increased hyaluronic acid.
Implementation and evaluation of a rehabilitation concept in a patient suffering from Scleredema Adultorum Buschke: a case report
Published in Disability and Rehabilitation, 2018
Michael Mickel, Ahmad Jalili, Christina Gesslbauer, Richard Crevenna
Scleredema Adultorum Buschke (SAB) is a disorder manifesting as non-pitting indurations of the skin that start on the head and spread to other distal areas of the body. Buschke was the first to report this clinical entity in 1902 [1]. Extracutaneous organs such as the muscles, joints, heart, or eyes may also be affected. Based on etiology Graff [2] classified SAB as following: (Type 1) streptococcal infections, the most frequent cause, (Type 2) associated with developing paraproteinemias including multiple myeloma, and (Type 3) associated with diabetes mellitus. In rare cases, SAB can be associated with hyperparathyroidism, connective tissue disease or human immunodeficiency virus infection. SAB is classified as a rare disorder with unknown prevalence [3]. In a review of 163 cases of SAB a percentage frequency is reportet as 55% of total of Type 1, 25% of total of Type 2, and 20% of total of Type 3 [4]. SAB affects all races and there is no gender difference in predilection for the disease [5].
Advances in understanding and treatment of scleromyxedema
Published in Expert Opinion on Orphan Drugs, 2018
Laura Atzori, Caterina Ferreli, Franco Rongioletti
The characteristic pathological triad is composed of dermal mucin accumulation, increased collagen deposition, and fibroblast proliferation (Figure 6) [3]. Mucin in scleromyxedema is made mainly of hyaluronic acid and accumulates in the upper and mid-reticular dermis. The material is alcian blue-positive at pH 2.5 and digested by hyaluronidase, Congo red, and periodic-acid Schiff stains negative [23]. Dermal fibroblasts are numerous, stellate or spindle shaped, associated with thickened collagen bundles, irregularly arranged, while the elastic fibers are usually fragmented and decreased in number, explaining the loose redundant folds of the back skin referred as the Shar-pei sign [1] The above epidermis is normal or thinned by the underlying mucin and fibrosis, with atrophic hair follicles. Slight epidermal hyperkeratosis with acanthosis is present in a minority of cases, as well as other additional microscopic findings, including a mild superficial perivascular lymphoplasmacytic infiltrate, proliferation of eccrine sweat glands, and dermal eosinophilia [1]. Differential diagnosis (Table 2) includes the localized variants of lichen myxedematosus such as acral persistent popular mucinosis with a completely different prognosis and treatment approach [5]. The clinico-pathological correlation is the key to distinguish the other two major sclerodermoid disorders: scleredema adultorum of Buschke, typical of diabetic patients, whose interstitial mucin deposition is located between fenestrated collagen bundles in up to three times thickened reticular dermis; and the nephrogenic systemic fibrosis, which develops in a person with severe renal insufficiency exposed to gadolinium [2]. The latter also shows variable amounts of mucin, but differs for an extensive involvement of the subcutis, with thick elastic fibers oriented parallel to the collagen bundles, dystrophic calcification, and sclerotic bodies, also known as elasto-collagenous balls.
Serositis in a child with scleredema of Buschke: an unusual association and improvement following glucocorticoids
Published in Scandinavian Journal of Rheumatology, 2018
D Sharma, A Somasekhara-Aradhya, P Vignesh, U-N Saikia, S Singh
Scleredema of Buschke is an uncommon skin condition characterized by wooden-like, non-pitting induration of skin that predominantly involves the neck, face, shoulders, and chest (1). Three types are described; type I is the most common type (55%), described predominantly in children (2). This subtype is common in girls and usually follows a febrile illness.