A patient aged 40 years presents with complaints of a mildly itchy rash on his eyelids, chest, back, and hands for the past three months. He reported feeling weak over the past month and that the weakness was getting progressively worse. The patient admitted he found it difficult to stand up from sitting. He was previously healthy.
A physical exam reveals the patient had a poikilodermatous eruption on his eyelids and glabella, upper chest, upper back, flank. Pinkish papules were found on his knuckles, which are classic for Gottron’s papules. Blood tests demonstrated elevated levels of muscle enzymes.
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Dermatomyositis is an autoimmune condition that presents with characteristic cutaneous findings and progressive proximal muscle weakness that originates in the extensor muscle groups.
Dermatologic findings include:
The heliotrope sign: edema, erythema, and scaly patches on the upper eyelid
Gottron’s papules: elevated red-purple lesions on the knuckles of hands
Gottron’s sign: red-purple discoloration of knuckles, area surrounding nail folds, elbows or knees
Shawl sign: erythematous/ poikilodermatous scaling on shoulders
V sign: erythematous/ poikilodermatous scaling on the V area of the anterior chest
Holster sign: erythematous/ poikilodermatous scaling on the lateral thigh/hip (where a gun holster might sit)
The patient may complain of fatigue, joint pain, and general malaise.
When a patient presents with the characteristic cutaneous and muscular findings, dermatomyositis should be suspected. Suggestive lab findings include elevated creatine kinase or aldolase levels. Skin biopsy, sometimes muscle biopsy, and muscle imaging (such as magnetic resonance imaging) is often used to confirm the diagnosis.
Once a diagnosis of dermatomyositis is confirmed in an adult patient, further studies must be conducted to rule out other systemic manifestations of dermatomyositis such as pulmonary and cardiac disease. Due to the co-existence of multiple autoimmune diseases in this patient population, a workup for other autoimmune conditions is commonly conducted.
Adult patients with dermatomyositis have a high risk of developing malignancies such as genitourinary, breast, lung, and pancreatic cancers. After the diagnosis of dermatomyositis is made, the patient should be followed up every six months for the next two years to survey for the development of malignancy.
First line treatment of dermatomyositis is systemic corticosteroids, followed by the addition of agents such as methotrexate or azathioprine. Severe cases of dermatomyositis may be treated with intravenous immunoglobulin, cyclophosphamide, tacrolimus, mycophenolate mofetil or biologic agents such as infliximab.
Adam Rees, MD, a graduate of the David Geffen School of Medicine at UCLA, practices dermatology in Los Angeles.
Danielle Brown is medical student at Baylor College of Medicine.
- Bolognia J, Jorizzo J, Rapini R. 2008. “Chapter 43- Dermatomyositis.” Dermatology. St. Louis, MO: Mosby/Elsevier. Print.
- Williams J, Berger T, Elston D. 2011. “Chapter 8- Connective Tissue Disease.” Andrews’ Diseases of the Skin: Clinical Dermatology. Philadelphia: Saunder Elsevier. Print.