Amyophatic dermatomyositis presenting as a flagellated skin eruption with positiveMDA5antibodies and thyroid cancer: a real association?

Abstract

Amyopathic dermatomyositis (ADM) is characterized clinically by typical skin lesions with hypomyopathy or no muscular involvement. ADM has been recently reported to be complicated by rapidly progressive interstitial lung disease (ILD), especially in patients with positive antibodies against melanoma differentiation-associated gene 5 (MDA5). These patients may have a low risk of cancer, but no clinical, histological or laboratory markers completely specific for paraneoplastic DM have been identified to date. We report a case of flagellate erythema as the initial presentation of ADM associated with ILD, positive MDA5 antibodies and a concomitant diagnosis of thyroid cancer. We discuss the unusual clinical features and associations that make this case particularly interesting.

Zebra Stripes in Dermatomyositis: Case Report and Review of Flagellate Erythema‐Associated Dermatomyositis

“…Tests for other rheumatologic antibodies were negative (Table 1). [2][3][4][5][6][7][8][9][10][11][12] Correlation of the clinical and laboratory findings established the diagnosis of amyopathic dermatomyositis with flagellate erythema.…”

“…7,14 In the cases of dermatomyositis-associated flagellate erythema described, classic features such as heliotrope erythema and Gottron's papules were present in all cases except one. [2][3][4][5][6][7][8][9][10][11][12] A total of 60% of cases (9/15) were seen in males, while the ages of females and males exhibit a similar distribution (Table 1). [2][3][4][5][6][7][8][9][10][11][12] To date, only one case of juvenile dermatomyositis has been described in association with flagellate erythema.…”

“…3 While pruritus was reported in 20% (3/15) of cases, none of the cases exhibited features of dermatographism (Table 1). [2][3][4][5][6][7][8][9][10][11][12] To date, three cases (3/15, 20%) of flagellate erythemaassociated amyopathic dermatomyositis have been described in the literature, 9,11,12 although two other cases (2/15, 13%), including our current case, may represent clinically amyopathic dermatomyositis (CADM) 10 (Table 1). [2][3][4][5][6][7][8][9][10][11][12] Two of the three amyopathic patients tested positive for antimelanoma differentiation-associated protein 5 (CADM-140) 9 and antitranscriptional intermediary factor 1 gamma (TIF-1c), 12 respectively.…”

“…15 In our present review, 20% (3/15) of cases were complicated by lung disease, one of these cases exhibited CADM-140 + dermatomyositis. 9 Only one case in the literature that was complicated by interstitial pneumonitis failed to respond to treatment 10 .…”

Rapidly progressive interstitial lung disease due to anti-MDA-5 antibody-positive clinically amyopathic dermatomyositis complicated with cervical cancer: Successful treatment with direct hemoperfusion using polymyxin B-immobilized fiber column therapy

“…To date, case reports on the association between CADM with cervical cancer have been rare [18], and it remains controversial whether this patient's cancer should be considered as relevant or accidental. We are aware of only two other reports of anti-MDA-5 antibody-positive patients with malignancy, CADM, and active ILD [19], [20]. One case did not recover from the respiratory failure after the complete removal of thyroid cancer, and the other case (with advanced gastric cancer) could not be treated because of acute respiratory failure.…”