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Scleredema
Evidence Levels: A Double-blind study B Clinical trial ≥ 20 subjects C Clinical trial < 20 subjects D Series ≥ 5 subjects E Anecdotal case reports
Scleredema (scleredema adultorum or scleredema of Buschke) is characterized by progressive, symmetric induration and thickening of the skin secondary to deposition of collagen and glycosaminoglycans in the dermis. Clinically, scleredema commonly involves the posterior neck, shoulders, trunk, face, and arms. Three clinical categories are recognized: type I, postinfectious, usually resolves spontaneously within 6 months–2 years (55% of cases); type II, malignancy-associated, is linked to paraproteinemias and monoclonal gammopathy with or without multiple myeloma (25% of cases); and type III, scleredema diabeticorum, is associated with poorly controlled insulin-dependent diabetes and persists indefinitely (20% of cases).
Management Strategy
Treatment of scleredema is usually unsatisfactory. However, case-based data demonstrates that several therapies can be effective. A candid discussion with the patient regarding limitations of treatment, cost, and side effects may lead to a decision to withhold treatment. This is particularly appropriate in patients with the postinfectious form, which often resolves spontaneously. Of course, identification of a specific etiology, such as streptococcal pharyngitis, should lead to appropriate antibiotic treatment, even in the absence of evidence that antibiotics alter the rate of clearance. In cases associated with diabetes mellitus and monoclonal gammopathy, progressive involvement can lead to discomfort, unsightly thickening, and systemic complications such as restrictive pulmonary function, dysphagia secondary to tongue swelling, serositis, and cardiac arrhythmias.
Bath- or cream-PUVA (psoralen ultraviolet A) has been recommended as initial therapy in moderately severe disease. More recently, narrowband ultraviolet B (NB-UVB) and UVA1 have been shown to be effective. Electron beam therapy has been proposed for patients with severe disease, especially cases with restrictive pulmonary function. Alternative therapies include ciclosporin , allopurinol , tamoxifen , and high-dose penicillin. Control of serum glucose levels with antidiabetic therapy has no effect on disease progression in scleredema diabeticorum.
Specific Investigations
Scleredema adultorum. Not always a benign self-limited disease
Curtis AC, Shulak BM. Arch Dermatol 1965; 92: 526–41.
A review of 223 scleredema patients revealed 25% had symptoms more than 2 years. The following treatments demonstrated no benefit: calcium gluconate, estradiol, fever, hot baths, hyaluronidase, nicotinic acid, ovarian extract, p-aminobenzoate, penicillin, pituitary extract, corticosteroids, thyroid hormone, and vitamin D.
Scleredema adultorum due to streptococcal infection
Alp H, Orbak Z, Aktas A. Pediatr Int 2003; 45: 101–3.
In 65%–95% of cases, scleredema occurs within a few days to 6 weeks after an acute febrile illness. Of these infections, 58% are streptococcal. These infections may present as tonsillitis, pharyngitis, scarlet fever, erysipelas, cervical adenitis, pneumonia, otitis media, pyoderma, impetigo, or rheumatic fever. Appropriate studies will rapidly determine underlying infection associated with scleredema.
Monoclonal gammopathy in scleredema: observations in three cases
Kovary PM, Vakilzadeh F, Macher E, et al. Arch Dermatol 1981; 117: 536–9.
Many subsequent reports have confirmed this association. Skin manifestations often precede the development of the gammopathy.
Scleredema associated with carcinoma of the gall bladder
Manchanda Y, Das S, Sharma VK, et al. Br J Dermatol 2005; 152: 1373–4.
Scleredema has been associated with various internal malignancies. When clinically indicated, an evaluation for internal malignancies may be appropriate.
Scleredema associated with immunoglobulin A-κ smoldering myeloma: a case report and review of the literature
Keragala BSDP, Herath HMMTB, Janappriya GHDC, et al. J Med Case Rep 2018; 13: 145.
A 66-year-old Sri Lankan woman with diffuse scleredema had no systemic symptoms and negative serologies for underlying autoimmune disease. An abnormal band in the gamma region was present on immunofixation. She improved with chemotherapy for her underlying immunoglobulin A-κ myeloma.
Scleredema: a review of 33 cases
Venencie PY, Powell FC, Su D, et al. J Am Acad Dermatol 1984; 11: 128–34.
Systemic corticosteroids, methotrexate, and d -penicillamine demonstrated no effect on the course of scleredema. Both diabetic and non-diabetic patients were studied. Some patients in both groups suffered from mild complications such as dysphagia and electrocardiogram (ECG) changes, but scleredema is generally a mild disease that poses no threat to overall health.
Fulminans in dermatology: a call to action: a recommendation for consideration of the term scleredema fulminans
Sommer LL, Heymann WR. J Clin Aesthet Dermatol 2014; 7: 42–5.
A 43-year-old man with an 11-year history of human immunodeficiency virus presented with a 3-month history of abrupt-onset progressive neck, shoulder, and back swelling shortly after diagnosis with type 2 diabetes. This case demonstrates the rapid onset that can occur in this chronic disease.
Second-Line Therapies
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