title: Amyopathic dermatomyositis: a review by the Italian Group of Immunodermatology.
abstract: OBJECTIVE
  To analyze the average age, sex distribution, duration of follow-up, clinical course, serologic abnormalities, and incidence of possibly associated malignancy in patients with amyopathic dermatomyositis.
  
  
  METHODS
  Retrospective study.
  
  
  METHODS
  University hospitals.
  
  
  METHODS
  Thirteen patients with amyopathic dermatomyositis.
  
  
  RESULTS
  The 13 patients represented 8.2% of 157 patients with dermatomyositis seen retrospectively in a 10-year period by the Italian Group of Immunodermatology of the Italian Society of Dermatology and Venereology. Gottron papules and sign and periungual telangiectasias were found in approximately 50% of cases (papules in 7 patients, Gottron sign and periungual telangiectasias in 6), while periorbital violaceous erythema was seen in 70% (9 patients). Arthralgias occurred in 2 patients and Raynaud phenomenon in 4. An elevated erythrocyte sedimentation rate was detected in 6 patients, hepatitis B virus antigen in 3, speckled antinuclear antibodies in 7, and anti-Ro and antimitochondrial antibodies in 1 case each. None of our patients had evidence of internal malignancy. Neither cardiopulmonary nor esophageal dysfunction was demonstrated. Electromyography showed a protopathic muscle abnormality in 3 patients. Muscle biopsy disclosed myositis and a neurogenic myopathy in another one.
  
  
  CONCLUSIONS
  Amyopathic dermatomyositis is a rare disease. So far, only 2 series of a few cases each have been reported. The "amyopathic" subset of dermatomyositis is peculiar in that its cutaneous lesions are predominant for long periods or even permanently, although they are indistinguishable from those of classic dermatomyositis. The minimal or absent muscle disease and the rarity of serum immunologic findings imply a favorable prognosis in white patients.
authors.affiliation: Dipartimento di Scienze Dermatologiche, Clinica Dermatlogica II, Università degli Studi di Firenze, Firenze, Italy.
authors.forename: Marzia
authors.initials: M
authors.lastname: Caproni
authors.forename: Carla
authors.initials: C
authors.lastname: Cardinali
authors.forename: Aurora
authors.initials: A
authors.lastname: Parodi
authors.forename: Barbara
authors.initials: B
authors.lastname: Giomi
authors.forename: Manuela
authors.initials: M
authors.lastname: Papini
authors.forename: Mario
authors.initials: M
authors.lastname: Vaccaro
authors.forename: Angelo
authors.initials: A
authors.lastname: Marzano
authors.forename: Clara
authors.initials: C
authors.lastname: De Simone
authors.forename: Marcello
authors.initials: M
authors.lastname: Fazio
authors.forename: Alfredo
authors.initials: A
authors.lastname: Rebora
authors.forename: Paolo
authors.initials: P
authors.lastname: Fabbri
mesh_terms: D000328:Adult; D017677:Age Distribution; D000368:Aged; D000369:Aged, 80 and over; D001707:Biopsy, Needle; D003882:Dermatomyositis / Q000453:epidemiology* / Q000473:pathology*; D004576:Electromyography; D005260:Female; D006801:Humans; D007150:Immunohistochemistry; D015994:Incidence; D007558:Italy / Q000453:epidemiology; D008297:Male; D008875:Middle Aged; D010257:Paraneoplastic Syndromes / Q000453:epidemiology* / Q000473:pathology*; D012189:Retrospective Studies; D012307:Risk Factors; D017678:Sex Distribution