Case Presentation
An otherwise healthy 40-year-old woman, working as a secretary, presented to our clinic complaining of photosensitivity and muscle weakness. Physical examination revealed facial erythematous desquamative plaques, and non-tender pale hyperkeratotic papules over the palmar aspect of the interphalangeal joints (Figure 1). She was sent to the Rheumatology department, where a muscle biopsy was performed, confirming the diagnosis of Dermatomyositis. An autoimmunity panel showed positivity for antiMi2 antibodies. All other autoantibodies were negative. No malignancies were found on imaging tests. A high-resolution computed tomography of the lungs showed no pathologic findings. Prednisone 30 mg/day, methotrexate 15 mg/week and hydroxychloroquine 400 mg/day were started, with resolution of muscular symptomatology after 2 months. Facial cutaneous manifestations and photosensitivity showed only partial response. Palmar papules persisted during the follow-up (12 months), and antiMi2 antibodies remained positive.
Figure 1.
Inverse Gottron papules. Pale hyperkeratotic papules over the palmar aspect of interphalangeal joints.
Teaching Point
Dermatomyositis can be associated with a wide range of cutaneous manifestations. Inverse Gottron papules are keratotic papules located on the flexor aspects of the fingers (distal and proximal interphalangeal joints, and metacarpophalangeal joints), and are an infrequently reported clinical sign of dermatomyositis [1,2]. In some case series, an association between inverse Gottron papules and antiMDA5 antibodies or Juvenile dermatomyositis has been reported, and patients with this clinical sign might have an increased risk of developing interstitial lung disease, and a worse prognosis [1,2]. However, there is scarce literature regarding this manifestation, and clinical implications remain unclear and should be further assessed. Regarding physiopathology, inverse Gottron papules may be induced by keratinocyte damage secondary to mechanical injury [1].
Inverse Gottron papules are an infrequent manifestation of dermatomyositis. Recognizing this clinical sign can help in the diagnosis of this disorder.
Footnotes
Funding: None.
Authorship: All authors have contributed significantly to this publication.
Competing Interests: None.
References
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