RHEUMATOLOGY 1

A 48-year-old female presents with a rash over her face for the past few weeks. She also complains of recent onset difficulty in rising from seated position and climbing stairs. On examination, you notice a dusky malar rash and a purplish periorbital edema. There is no ocular or facial weakness. Her proximal muscles of both extremities show symmetrical weakness. Which of the following is commonly associated with this presentation?

A. Inflammatory bowel disease.
B. Anti-Scl-70 antibodies.
C. Gottron’s sign.
D. HLA-B27 association.
E. Esophageal smooth muscle dysfunction.
F. Acetyl choline receptor antibodies
G. Positive Anti-DsDNA antibodies.

the answer is C Gottron’s sign.

Explanation:

In dermatomyositis, patients can have a classic malar rash, like systemic lupus erythematosus (SLE). Periorbital edema with a purplish suffusion is called the heliotrope rash. Scaly patches over the dorsum of proximal interphalangeal and metacarpophalangeal joints (Gottron’s sign) are fairly unique to dermatomyositis. Unlike myasthenia facial or ocular muscle weakness is uncommon. Unlike scleroderma, which affects the lower smooth muscle of esophagus, it involves striated muscle of the upper pharynx and can make deglutition difficult. Suspected patients should have measurement of serum creatine kinase and aldolase levels. Specific diagnosis is usually made by muscle biopsy. These patients should have a complete metastatic workup to rule out malignancy. It includes PAP smear, mammogram, CT chest / abdomen / pelvis, and colonoscopy.

Choice (A): Inflammatory bowel disease is usually associated with ankylosing spondylitis.

Choice (B): Anti-Scl-70 antibodies are positive in scleroderma. Rheumatoid factor is positive in few patients with dermatomyositis. Majority of theses patients have positive anti nuclear antibody.

Choice (D): HLA B27 is negative and is usually positive in seronegative spondyloarthropathies such as ankylosing spondylitis, Reiter’s arthritis etc.

Choice (E): Esophageal smooth muscle dysfunction is not a feature of dermatomyositis. The striated muscles of the pharynx are affected leading to difficulty in the initial phase of swallowing.