Clinical Image: Nasal rim sign: A clinical manifestation of dermatomyositis

Abstract

The patient, a 69-year-old African American woman, presented with a three-month history of digital ulcers, Raynaud phenomenon, and occasional cough in addition to an eruption on her nose, back, and elbows. The patient denies any oral ulcers or nail changes. She also denied any photosensitivity, arthralgia, morning stiffness, muscle weakness or pain, fever, chills, weight loss, difficulty swallowing, hematuria, abdominal pain, headache, seizures, or mood disturbance. Mucocutaneous examination was remarkable for violaceous scaly plaques lining the nasal rim and on the elbows and back and multiple few-millimeter-sized, well-defined, grouped, eroded, and ulcerated violaceous papules overlying the joints on the palmar (palmar papules or inverse gottron papules) 1 and volar aspects of the hands. Muscle strength was within normal limits. Laboratory evaluation revealed a positive anti-melanoma differentiation antigen (MDA)-5 antibody in the setting of unremarkable antinuclear antibody, antineutrophil cytoplasmic antibody, double-stranded DNA, anti-sjogren syndrome A, anti-sjogren syndrome B, cyclic citrullinated peptide, creatinine kinase, uric acid, and complement levels. Erythrocyte sedimentation rate and C-reactive protein showed mild elevation. Skin biopsy from the elbow showed interface dermatitis, whereas that from digits showed infarct. Computed tomography) of the chest showed basilar predominant irregular consolidation with some reticulation and ground-glass attenuation, and a pulmonary function test showed a restrictive pattern. In view of pulmonary involvement, prominent vasculopathy, and to prevent rapid progressions, she was treated with 2 g/kg intravenous immunoglobulin every 28 days for 6 months and mycophenolic acid, prednisone, and nifedipine. The nasal rim sign has not been discussed or documented previously in the literature. However, on extensive literature search of clinical images of dermatomyositis, we came across some suggestions of similar involvement in other patients with anti-MDA-5 dermatomyositis. [2][3][4] Whether or not this sign is suggestive or specific for this antibody and its prognostic implication and association with airway/lung involvement remains to be established. In one review, the authors noted 4 of 10 patients who were anti-MDA-5 positive reported tender gums and/or oral erosions, significantly more than the group that was anti-MDA5 negative. 1 The nasal rim sign could possibly indicate another orifical predilection. Detailed documentation of clinical features and prognosis in further reports would provide more insight into the association.