Amyopathic Dermatomyositis: A Review.

Abstract

Jim Gilliam's research interests throughout his career were forced upon better defining the relationships that exist between the cutaneous and systemic manifestations of the rheumatic diseases. Although the majority of his time was spent studying such relationships in lupus erythematosus patients, he was also intensely interested in dermatomyositis (DM) in this regard as well. He was particularly intrigued with the dissociation of the cutaneous and muscular manifestations of this disorder that occasionally occurs. The term "dermatomyositis siné myositis" has been used in the past to describe patients who present with only the cutaneous manifestations of DM; however, very little published data is available from systematic examinations of such patients. For several reasons, we have preferred the term "amyopathic dermatomyositis" to describe that rare patient who for long periods of time suffers from the classical skin lesions of DM as the only clinically significant manifestation of their disease. In this presentation, we review our own personal experience with a group of six such patients and compare and contrast it to that of other workers who have dealt with this subject over the past two decades.

Clinical manifestations of dermatomyositis and clinically amyopathic dermatomyositis patients with positive expression of anti–melanoma differentiation–associated gene 5 antibody

“…Twelve healthy volunteers were also included as control subjects. DM or CADM was diagnosed based on the criteria of Bohan and Peter or following the modified Sontheimer's definitions (15,16). Clinical data, including medical history and physical examination results, were collected upon admission and during followup.…”

Interstitial lung diseases associated with amyopathic dermatomyositis

“…A myopathic dermatomyositis (ADM) is recognised as a distinct subgroup of dermatomyositis (DM) with the typical skin rash of classic DM, but without muscle involvement [1][2][3][4][5][6]. Several studies have demonstrated that rapidly progressive interstitial lung disease (ILD) with a poor prognosis occurs in patients with ADM [7][8][9][10][11][12].…”

Rapidly Progressive Interstitial Lung Disease in a Dermatomyositis Patient with High Levels of Creatine Phosphokinase, Severe Muscle Symptoms and Positive Anti-Jo-1 Antibody.

“…Amyopathic dermatomyositis (ADM) was first described by Pearson (14) and is a disease which only has the cutaneous manifestations of DMwithout any clinical evidence of underlying inflammatory muscle disease. Euwer and Sontheimer (15) discussed that the diagnostic criteria set forth for DMby Bohan and Peter (I: polymyositis, II: dermatomyositis, III: myositis with malignancy, IV: childhood myositis, V: myositis with overlap syndromes) (8) should be altered to include a sixth diagnostic category, ADM. The rapidly progressive ILD has occurred in many DMpatients with mild muscle symptoms (2, 6) or slightly increased CPK levels (5, 7), but it is not clear whether or not all of such patients correspond to ADM. Anti-Jo-1 antibody is found in about 20% in PM/DMpatients and other types of antisynthetase antibodies, such as anti-PL-12, anti-OJ, are also found (16).…”