Abstract

Parry-Romberg syndrome is a rare disorder characterized by progressive hemifacial atrophy of the skin, adipose tissue, muscle, cartilage, and underlying bony structures. Although various autoantibodies such as antinuclear antibodies (ANA), rheumatoid factor (RF), and anti-single-stranded DNA antibodies are found in patients with Parry-Romberg syndrome, coexistence with systemic lupus erythematosus is rare. We report a Parry-Romberg syndrome patient with positive ANA and presence of anti-double- stranded DNA antibodies who then developed systemic lupus erythematosus. We emphasized a thorough physical examination and periodic immunological evaluation in patients with Parry-Romberg syndrome. The presence of ANA or PT in a patient of Parry-Romberg syndrome carries the risk of developing systemic collagen-vascular disorders.

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