Scleredema

Published on 18/03/2015 by admin

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Scleredema

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

image

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).

Management strategy

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.

Specific investigations

First-line therapies

image Identify and treat any underlying disease (diabetes mellitus, monoclonal gammopathy, acute infection) D
image Conservative management using the principle ‘do no harm’ D

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 that 25% have had symptoms for more than 2 years. The following treatments demonstrated no therapeutic benefit: calcium gluconate, estradiol, fever, hot baths, hyaluronidase, nicotinic acid, ovarian extract, p-aminobenzoate, penicillin, pituitary extract, corticosteroids, thyroid hormone, and vitamin D.

Because of the expense and potential side effects of the second-line therapies discussed below, as well as the recognition that many patients with mild scleredema either improve without treatment or do not respond to the therapy, a policy of ‘do no harm’ and withholding of treatment is recommended in patients with mild disease.

Second-line therapies

image Narrowband UVB phototherapy E
image UVA1 phototherapy D
image Electron beam therapy E
image Bath PUVA and cream PUVA E
image Physical therapy E
image Tamoxifen E
image High-dose penicillin E
image Cyclosporine E
image Extracorporeal photephoresis E
image Chemotherapy (melphalan) E

Ultrasonic massage and physical therapy for scleredema: improving activities of daily living.

Bray SM, Varghese S, English JC 3rd. Arch Dermatol 2010; 146: 453–4.

A 42-year-old man with diabetes-associated scleredema was treated with hydroxychloroquine and psoralen UVA without clinical improvement. Physical therapy with ultrasonic massage, active range-of-motion, and flexibility exercises three times a week was initiated. Ultrasonic massage therapy was performed using a Dynatron 709 series machine with the following parameters: 100%, 1 MHz, and 1.5 W/cm2 for 10 minutes per session. The patient showed objective improvement in nine of 10 range-of-motion parameters measured after 1 year of physical therapy. Ultrasonic massage and physical therapy may improve the quality of life for patients with scleredema.

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Scleredema

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

image

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).

Management strategy

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.

Specific investigations

First-line therapies

image Identify and treat any underlying disease (diabetes mellitus, monoclonal gammopathy, acute infection) D
image Conservative management using the principle ‘do no harm’ D

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