Dermatomyositis

Dermatomyositis (DM) is an idiopathic inflammatory myopathy (IIM) with characteristic cutaneous findings. In 1975, Bohan and Peter first suggested a set of criteria to aid in the diagnosis and classification of DM and polymyositis (PM). Four of the 5 criteria are related to the muscle disease, as follows: progressive proximal symmetrical weakness, elevated muscle enzymes, an abnormal finding on electromyogram, and an abnormal finding on muscle biopsy. The fifth criterion was compatible cutaneous disease. DM is probably caused by complement-mediated (terminal attack complex) vascular inflammation, while PM is caused by the direct cytotoxic effect of CD8+ lymphocytes on muscle.

In the US: The incidence of DM/PM has been estimated at 5 In the US: The incidence of DM/PM has been estimated at 5.5 cases per million people, and the incidence is apparently increasing. Blacks are more frequently affected than whites. Females are affected twice as often as males. DM can occur in people of any age. Two peak ages of onset exist. In adults, the peak age of onset is approximately 50 years, and, in children, the peak age is approximately 5-10 years.

Patients often present with skin disease as one of the initial manifestations. Sometimes, perhaps in as many as 40% of the patients, the skin disease may be the sole manifestation at the onset. Muscle disease may occur concurrently, may precede the skin disease, or may follow the skin disease by weeks to years. Patients often notice an eruption on exposed surfaces. The rash is often pruritic, and intense pruritus may disturb sleep patterns. Patients may also report a scaly scalp or diffuse hair loss. Muscle involvement is manifested by proximal muscle weakness. Patients often begin to note fatigue of their muscles or weakness when climbing stairs, walking, rising from a sitting position, combing their hair, or reaching for items in cabinets that are above their shoulders. Muscle tenderness may occur, but tenderness is not a regular feature of the disease. Systemic manifestations may occur; therefore, the review of systems should assess for the presence of arthralgia, arthritis, dyspnea, dysphagia, arrhythmia, and dysphonia.

Malignancy is possible in any patient with DM, but it occurs more frequently in adults older than 60 years. The characteristic, and possibly pathognomonic, cutaneous features of DM are heliotrope rash and Gottron papules. Several other cutaneous features, including malar erythema, poikiloderma (ie, variegated telangiectasia, hyperpigmentation) in a photosensitive distribution, violaceous erythema on the extensor surfaces, and periungual and cuticular changes, are characteristic of the disease even though they are not pathognomonic. The heliotrope rash consists of a violaceous-to-dusky erythematous rash with or without edema in a symmetrical distribution involving periorbital skin.

The Gottron papules are found over bony prominences, particularly the metacarpophalangeal joints, the proximal interphalangeal joints, and/or the distal interphalangeal joints. Papules may also be found overlying the elbows, knees, and/or feet. The lesions consist of slightly elevated violaceous papules and plaques. A slight scale and, occasionally, a thick psoriasiform scale may be present. These lesions may resemble lesions of lupus erythematosus (LE), psoriasis, or lichen planus (LP). Nailfold changes consist of periungual telangiectases and/or a characteristic cuticular change with hypertrophy of the cuticle and small hemorrhagic infarcts with this hypertrophic area. Periungual telangiectases may be apparent clinically or may be visible only on capillary microscopy. Poikiloderma may occur on exposed skin, such as the extensor surfaces of the arm, and may appear in a V-shaped distribution over the anterior neck and upper chest and back (ie, shawl sign).

Scalp involvement in DM is relatively common and is manifested by an erythematous-to-violaceous psoriasiform dermatitis. Clinical distinction from seborrheic dermatitis or psoriasis is occasionally difficult. Nonscarring alopecia may occur in some patients and often follows a flare of systemic disease. DM-sine myositis, also known as ADM, is diagnosed in patients with typical cutaneous disease and no evidence of muscle weakness and in patients who repeatedly have serum muscle enzyme levels within the reference range. Some patients with ADM have abnormal findings on ultrasound, MRI, or muscle biopsy. These patients have subclinical muscle involvement, but their condition still may be classified as ADM. Because many ADM patients are not evaluated beyond clinical and enzymatic studies, many think that ADM represents a systemic process requiring systemic therapies. Calcinosis of the skin or muscle is unusual in adults but may occur in as many as 40% of children or adolescents with DM. Calcinosis cutis is manifested by firm yellow or flesh-colored nodules, often over bony prominences. Occasionally, the nodules can extrude through the surface of the skin, in which case secondary infection may occur.

Joint swelling occurs in some patients with DM Joint swelling occurs in some patients with DM. The small joints of the hands are most frequently involved. The arthritis associated with DM is not erosive or deforming. Immunological abnormalities are common in patients with DM. Patients frequently have circulating autoantibodies. Abnormal T-cell activity may be involved in the pathogenesis of both the skin and the muscle disease. In addition, family members may have other diseases associated with autoimmunity. Autoantibodies to nuclear antigens (ANA) and cytoplasmic (ie, antitransfer RNA synthetases) antigens may be present. While their presence may help to define subtypes of DM and PM, their role in pathogenesis is uncertain. Infectious agents, including viruses (eg, coxsackievirus, echovirus, human T-cell lymphotrophic virus type 1 [HTLV-1], HIV) and Toxoplasma and Borrelia species, have been suggested as possible triggers of the disease.

Muscle enzyme levels are often abnormal during the course of DM, except in patients with the amyopathic variant. The most sensitive/specific enzyme is an elevated creatine kinase (CK), but aldolase levels and other tests (eg, aspartate aminotransferase [AST], lactic dehydrogenase [LDH]) may also yield abnormal results. At times, the elevation of the enzymes precedes clinical evidence of myositis. Thus, if a patient who is presumably stable develops an elevation of an enzyme previously within the reference range, the clinician should assess the possibility of a flare of the muscle disease. A positive ANA is common in patients with DM. Anti-Mi-2 antibodies are highly specific for DM but lack sensitivity because only 25% of the patients with DM demonstrate them. They are associated with acute-onset classic DM with the V-shaped and shawl rash (poikiloderma) and a relatively good prognosis. Anti-Jo-1 (antihistidyl transfer RNA [t-RNA] synthetase) is more frequent in patients with PM than in patients with DM. It is associated with pulmonary involvement (interstitial lung disease), Raynaud phenomenon, arthritis, and mechanic's hands. .

Histologic Findings: A skin biopsy reveals an interface dermatitis that is difficult to differentiate from LE. Vacuolar changes of the columnar epithelium and lymphocytic inflammatory infiltrates at the dermal-epidermal junction basement membrane can occur. Muscle biopsy in DM reveals perivascular and interfascicular inflammatory infiltrates with adjoining groups of muscle fiber degeneration/regeneration. This contrasts with PM infiltrates, which are mainly intrafascicular (endomysial inflammation) with scattered individual muscle fiber necrosis.

Medical Care: Bedrest is often valuable for those patients with severe muscle inflammation. In patients with muscle weakness, especially children, a program of physical therapy is useful to help prevent contractures that can complicate the disease when patients do not fully move their joints. For patients with dysphagia, recommending elevating the head of their bed and not eating before bedtime is useful, possibly preventing aspiration pneumonitis. Occasionally, nasogastric tube feeding may be needed to increase caloric input.

The mainstay of therapy for the muscle disease is systemically administered corticosteroids. Traditionally, prednisone (1-2 mg/kg/d) is administered as initial therapy. Taper slowly to avoid relapse of the disease. Because most patients develop steroid-related toxic effects, many authorities begin an immunosuppressive or cytotoxic agent early in the course. Most clinical and published experience is with the use of methotrexate as a steroid-sparing agent, but azathioprine and mycophenolate mofetil have been used. Results with cyclophosphamide in severe cases have been disappointing. In patients for whom these measures fail, the use of monthly high-dose intravenous immunoglobulin (IVIG) for 6 months has proved beneficial in the short term.

First-line therapy is to recognize that the patient is photosensitive and to prescribe sun avoidance and sun protective measures, including broad-spectrum sunscreens. Hydroxychloroquine and chloroquine have been beneficial in small open-label case studies. Methotrexate is useful. Recently, mycophenolate mofetil has been reported to be useful. IVIG not only benefits the muscle but also clears the skin lesions.

Drug Category: Immunoglobulins -- High-dose IV immunoglobulin has been reported to be useful for patients with recalcitrant DM. Drug NameImmune globulin intravenous (Gamimune, Gammagard, Sandoglobulin) -- For patients in whom corticosteroids and immunosuppressives have failed. Adult Dose1 g/kg IV on 2 consecutive d, then 400 mg/kg IV every mo, generally for a 6-mo course Pediatric DoseAdminister as in adults ContraindicationsDocumented hypersensitivity; IgA deficiency; anti-IgE/IgG antibodies InteractionsNone reported PregnancyC - Safety for use during pregnancy has not been established. PrecautionsConsider checking serum IgA before administering IVIG and using IgA-depleted IVIG (G-Gard-SD), if indicated; IVIG may increase serum viscosity and thromboembolic events; infusions may increase risk of migraine attacks, aseptic meningitis (10%), urticaria, pruritus, or petechiae (2-30 d postinfusion); increased risk of renal tubular necrosis in elderly patients and in patients with diabetes, volume depletion, and preexisting kidney disease; laboratory result changes associated with infusions include elevated antiviral or antibacterial antibody titers for 1 mo, 6-fold increase in ESR for 2-3 wk, and apparent hyponatremia