Published on 18/03/2015 by admin
Filed under Dermatology
Last modified 18/03/2015
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Mouhammad Aouthmany, Amy E. Flischel, Stephen E. Helms and Robert T. Brodell
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 a connective tissue disorder characterized by progressive, symmetric induration and thickening of the skin secondary to increased amounts of collagen and glycosaminoglycans. Clinically, scleredema most commonly involves the posterior neck, shoulders, trunk, face, and arms. The three clinical forms are: (1) scleredema following acute viral or bacterial infection, which usually resolves spontaneously in 6 months to 2 years; (2) scleredema associated with diabetes mellitus, which persists indefinitely; and (3) scleredema associated with malignancy (monoclonal gammopathy, insulinoma, and carcinoma of the gallbladder).
Treatment of scleredema is difficult and usually unsatisfactory (or frequently inadequate) however, there are case-based data to support the effectiveness of several therapies. In many cases, a candid discussion with the patient regarding limitations of treatment, cost, and side effects will lead to a decision to withhold treatment. This is particularly appropriate in patients with the post-infectious form, which can resolve spontaneously without any specific therapy. 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 would alter the rate of clearing in this self-limited form of scleredema. In the forms associated with diabetes mellitus and monoclonal gammopathy, progressive involvement can lead to discomfort, unsightly thickening, and even systemic complications such as restrictive pulmonary function, dysphagia secondary to tongue swelling, and cardiac arrhythmias. In these cases, patients will demand treatment.
Bath or cream PUVA is recommended as initial therapy in moderately severe disease. More recently, narrowband UVB and UVA1 have shown to be moderately effective. Electron beam therapy is the primary recommendation for patients with severe disease, especially cases with restrictive pulmonary function. Alternative therapies include cyclosporine and high-dose penicillin. Anti-diabetic therapy has no effect on the evolution of scleredema in diabetics, as the progression of scleredema has been found to be unrelated to control of serum glucose levels.
Venencie PY, Powell FC, Su D, Perry HO. J Am Acad Dermatol 1984; 11: 128–34.
Rongioletti F, Rebora A. In: Bolognia J, Jorizzo J, Schaffer JV, eds. Dermatology, 3rd ed. Edinburgh: Elsevier, 2012; 687–98.
Fasting blood sugar, glucose tolerance test, hemoglobin A1c (glycosylated hemoglobin)
Serum protein electrophoresis, immunoelectrophoresis
Antistreptolysin O, bacterial culture, other efforts to identify an acute infectious agent, erythrocyte sedimentation rate
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 if an underlying infection is associated with scleredema.
Since 50% of cases of scleredema occur in childhood, the adultorum appellation is a misnomer.
Kovary PM, Vakilzadeh F, Macher E, Zaun H, Merk H, Goerz G. Arch Dermatol 1981; 117: 536–9.
Immunoelectrophoresis studies in six patients with severe persistent scleredema revealed that three patients also had a monoclonal gammopathy, and many subsequent reports have confirmed this association. The skin manifestations often precede the development of the gammopathy, and, therefore, immunoelectrophoresis should be performed at regular intervals in all cases of widespread scleredema.
Manchanda Y, Das S, Sharma VK, Srivastava DN. Br J Dermatol 2005; 152: 1373–4.
Scleredema has been associated with internal malignancies including one report in a female with carcinoma of the gallbladder.
When clinically indicated, an evaluation for internal malignancies may be appropriate.
Fleischmajer R. Arch Dermatol 1970; 101: 21–6.
Scleredema in patients with diabetes mellitus generally follows a progressive course that is unrelated to control of serum glucose levels.
Treatment of Skin Disease Comprehensive Therapeutic Strategies 4e
Identify and treat any underlying disease (diabetes mellitus, monoclonal gammopathy, acute infection)
Conservative management using the principle ‘do no harm’
