Amyopathic Dermatomyositis: A Review

Renal cell carcinoma and amyopathic dermatomyositis

“…6 Carcinomas can affect almost any organ system, however in DM they predominantly involve the reproductive organs, pulmonary system, and gastrointestinal tract. 3,4,8,9 A similar association has been demonstrated with ADM although it remains controversial with interstitial lung disease being the mainstay. [2][3][4]8,9 A detailed literature search revealed no cases of renal cell carcinoma associated with ADM. We report a case of renal cell carcinoma associated with ADM in a 69-yearold man who presented with hematuria.…”

“…3,6 It has a 3:1 female preponderance, and most cases do not progress to myopathy or a diagnosis of DM. 4,6 An association with malignancy similar to that of classic DM has been described in the literature. 3,4,6,8,9 The exact relationship between malignancy and ADM and DM has yet to be demonstrated.…”

“…6 Diagnostic criteria for ADM are cutaneous lesions and skin biopsy results indistinguishable from DM, but with no clinical evidence of proximal muscle weakness or serum muscle enzyme abnormalities for at least 6 months. [2][3][4]6,7 The association between internal malignancy and DM has been reported, with reports as high as 60%. 6 Carcinomas can affect almost any organ system, however in DM they predominantly involve the reproductive organs, pulmonary system, and gastrointestinal tract.…”

“…3 Characteristic skin manifestations include heliotrope rash, periungual telangiectasia, edema, and facial erythema, with the hallmark being Gottron's papules -scaling, erythematous eruptions over the knuckles and other extensor surfaces. [3][4][5] Amyopathic dermatomyositis (ADM), first coined by Pearson in 1963, is a rare but well-recognized clinical subtype of DM, constituting 2% to 18% of patients with this disease. 6 Diagnostic criteria for ADM are cutaneous lesions and skin biopsy results indistinguishable from DM, but with no clinical evidence of proximal muscle weakness or serum muscle enzyme abnormalities for at least 6 months.…”

“…3,4 The primary skin change is a confluent or patchy macular violaceous erythema, as well as pruritus. 3 Characteristic skin manifestations include heliotrope rash, periungual telangiectasia, edema, and facial erythema, with the hallmark being Gottron's papules -scaling, erythematous eruptions over the knuckles and other extensor surfaces.…”