Christian R. Halvorson, MD Richard Colgan, MD Department of Family and Community Medicine, University of Maryland School of Medicine, Baltimore crhalvor@gmail.com
The authors reported no potential conflict of interest relevant to this article.
Bohan and Peter criteria help with dermatomyositis diagnosis A diagnosis of dermatomyositis can be established using the following criteria developed by Bohan and Peter in 1975: (1) symmetric muscle weakness; (2) elevation of muscle enzymes, most notably creatinine phosphokinase; (3) evidence of inflammation on muscle biopsy; (4) electromyographic features of myositis; and (5) characteristic dermatologic signs, including a heliotrope rash and Gottron’s papules.28,29 A definitive diagnosis can be made when the patient meets 3 of the first 4 criteria, as well as the fifth.
Corticosteroids are the mainstay of treatment for dermatomyositis, although the dose and duration are subject to debate. Cutaneous manifestations of dermatomyositis are commonly treated with topical corticosteroids and oral hydroxychloroquine, as well as emollients and antipruritic agents.
Cancer screening. All adults with signs and symptoms of dermatomyositis should undergo cancer screening. The most common malignancies are ovarian cancer, gastric cancer, and lymphoma.21
Photoprotection. As with cutaneous LE, UV exposure can exacerbate dermatomyositis, and patients should use a broad-spectrum sunscreen.
Scleroderma: Localized and systemic disease
The major cutaneous manifestation of scleroderma is that of thickened, leathery, bound-down skin, seen in both localized and systemic disease. In both cases, the lesions typically evolve through 3 characteristic stages: the initial inflammatory and edematous stage; a fibrotic stage, during which the skin lesions appear hard, tight, and hidebound; and the final, atrophic stage. However, not all lesions progress to this final stage.8
Localized scleroderma (also known as morphea) affects roughly 1 in 100,000 people. It is more prevalent in females than males, with a ratio as high as 3 to 1.30-33 Cutaneous findings vary, and numerous clinical presentations are possible. The most widely used classification system, the Mayo Clinic Classification, recognizes 5 subtypes: plaque morphea (the most common), generalized morphea, linear morphea, deep morphea, and bullous morphea (TABLE 2).34 The mean age of onset depends on the subtype, and ranges from 12 years for linear morphea to 45 years for deep morphea.35
FAST TRACK
While systemic involvement is not common with localized scleroderma, extracutaneous manifestations have been reported in up to 25% of cases.
While systemic involvement is not common with localized scleroderma, extracutaneous manifestations have been reported in up to 25% of cases.31,36,37 In a 2005 multicenter study of 750 pediatric patients with localized scleroderma, the most frequently reported extracutaneous symptom was arthritis, found in 12% of patients.36 Less frequent findings included neurologic symptoms such as seizures and headaches, vascular changes such as deep vein thrombosis, and gastrointestinal, cardiac, and renal conditions. Although the precise etiology of localized scleroderma is unknown, it is thought to be associated with trauma, prior infection by Borrelia burgdorferi, chronic venous insufficiency, and irradiation for breast cancer.30,38,39
TABLE 2 Localized scleroderma: What to look for8,34
Plaque morphea: asymmetric, circumscribed, hyper- or hypopigmented, indurated plaques most commonly found on the trunk and proximal extremities
Generalized morphea: similar to plaque morphea, but appearing confluent and more widespread
Linear morphea: skin changes similar to plaque morphea, occurring in an asymmetric linear fashion, most commonly occurring in children
– En coup de sabre: lesions on the face or scalp, in a pattern that resembles a mark caused by the stroke of a sword during a duel. (See image.)
Deep morphea: poorly defined subcutaneous nodules and plaques
Bullous morphea: vesicular eruptions on top of morphea lesions
Systemic scleroderma is a heterogenous disorder Commonly called systemic sclerosis (SSc), systemic scleroderma is characterized by proliferative vascular lesions; fibrosis of internal organs, including the lungs, heart, kidneys, and gastrointestinal tract; and distinct cutaneous manifestations.3 SSc affects about 240 people per million adults, mostly between the ages of 30 and 50 years, with women affected 3 times as often as men.40-42
The cause of SSc is unknown, although a genetic predisposition is likely. Environmental factors likely play a role in triggering the disease, with possible causative factors including cytomegalovirus and other viral infections and exposure to certain chemicals.43
As in localized scleroderma, cutaneous manifestations are a prominent part of SSc and typically develop early on. Raynaud’s phenomenon (RP) (FIGURE 3), which occurs in 90% to 98% of patients with SSc,44 can develop in association with other cutaneous findings or precede additional skin changes by months or years.
Other early skin changes include nonpitting edema of the fingers and toes, which creates a sausage-like appearance. This is typically followed by hardening and thickening of the skin in these areas, which can result in highly disabling sclerodactyly (FIGURE 4).45 Patients can also develop painful ulcerations on their fingertips and knuckles (rat bite necroses) due to local ischemia and vascular insufficiency. This may be complicated by secondary bacterial infection, gangrene, and acroosteolysis, leading to articular deformities and dissolution of terminal phalanges (FIGURE 5).45-48
