Scleredema

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

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.

Scleredema: a review of thirty cases.

Venencie PY, Powell FC, Su D, Perry HO. J Am Acad Dermatol 1984; 11: 128–34.

Mucinoses (Chapter 46).

Rongioletti F, Rebora A. In: Bolognia J, Jorizzo J, Schaffer JV, eds. Dermatology, 3rd ed. Edinburgh: Elsevier, 2012; 687–98.

Specific investigations

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

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 if an underlying infection is associated with scleredema.

Since 50% of cases of scleredema occur in childhood, the adultorum appellation is a misnomer.

Monoclonal gammopathy in scleredema: observations in three cases.

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.

Scleredema associated with carcinoma of the gall bladder.

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.

Scleredema and diabetes mellitus.

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.

First-line therapies

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

Scleredema: a review of thirty-three cases.

Venencie PY, Powell FC, Su D, Perry HO. J Am Acad Dermatol 1984; 11: 128–34.

A review of 33 cases of scleredema, in which systemic corticosteroids, methotrexate, and D-penicillamine demonstrated no effect on the disease course. Both diabetic and non-diabetic patients were studied, and, although some patients in both groups suffered from mild complications such as dysphagia and ECG changes, the authors emphasize that scleredema is generally a mild disease that poses no threat to overall health.

Only gold members can continue reading. Log In or Register to continue

Related

Stay updated, free articles. Join our Telegram channel

Tags: Treatment of Skin Disease Comprehensive Therapeutic Strategies

Aug 7, 2016 | Posted by admin in Dermatology | Comments Off

Full access? Get Clinical Tree

Get Clinical Tree app for offline access

Get Clinical Tree app for offline access