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Violaceous Macules on the Auricles: A Clinical Sign of Dermatomyositis

Author Affiliation(s)

Case Presentation

A 66-year-old male with a recently diagnosed metastatic bladder carcinoma presented with muscle weakness and generalized pruritic rash. Physical examination revealed heliotrope erythema, V-neck sign, shawl sign, Gottron papules and dilated nailfold capillary loops. Interestingly, several purpuric macules, together with ulcers and crusting were observed in both auricles ( Figure 1 ). Blood tests showed high muscle enzymes (creatine-kinase 5954 U/l [normal value <300 U/l] and aldolase 43.5 U/l [normal range 0.3–6.0 U/l]), and anti-transcriptional intermediary factor 1 gamma (TIF1-γ) antibody positivity. The remaining myositis-specific antibodies were negative. Skin and muscle biopsies confirmed the diagnosis of dermatomyositis. Treatment with prednisone 120 mg/day (1 mg/kg/day) and hydroxychloroquine 400 mg/day was initiated with progressive improvement of muscle weakness and cutaneous involvement. The patient also started chemotherapy with gemcitabine and cisplatin and is currently under follow-up in the Oncology and Dermatology departments.

Figure 1 .

(A, B) Violaceous macules, crusting and ulcers on both auricles. The lesions are more evident in anatomical protuberances such as the helix and antihelix.

Teaching Point

Dermatomyositis can present with a myriad of cutaneous manifestations. Violaceous macules on the ears have recently been described in anti-melanoma differentiation-associated gene 5 (MDA-5) dermatomyositis and may correlate with a poor prognosis [ 1 ] . As the violaceous macules and ulcers are predominantly located in anatomic protuberances in the auricle, a pressure-induced microangiopathy has been proposed as the pathogenic mechanism [ 2 ] . We have not found any described case presenting with positive anti-TIF1-γ antibodies, which have been associated with malignancies.

We report a case of a paraneoplastic anti-TIF1-γ positive dermatomyositis presenting with violaceous macules in both auricles. Clinicians should be aware of this recently described clinical sign since it may be related to a poor prognosis.

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References

  1. Erythematous auricular papules in the fatal cases of anti-MDA5 antibody-positive interstitial lung disease Intapiboon P, Siripaitoon B. Respir Med Case Rep.2020;19(31):101299. CrossRef PubMed
  2. et al. Antihelix/helix violaceous macules in Japanese patients with anti-melanoma differentiation-associated protein 5 (MDA5) antibody-associated dermatomyositis Okiyama N, Inoue S, Saito A. Br J Dermatol.2019;180(5):1226-1227. CrossRef PubMed

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