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Amyopathic Dermatomyositis
A Review by the Italian Group of Immunodermatology
Marzia Caproni, MD;
Carla Cardinali, MD;
Aurora Parodi, MD;
Barbara Giomi, MD;
Manuela Papini, MD;
Mario Vaccaro, MD;
Angelo Marzano, MD;
Clara De Simone, MD;
Marcello Fazio, MD;
Alfredo Rebora, MD;
Paolo Fabbri, MD
Arch Dermatol. 2002;138:23-27.
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.
Design Retrospective study.
Setting University hospitals.
Patients 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.
From the Dipartimento di Scienze Dermatologiche, Clinica Dermatologica
II, Università degli Studi di Firenze, Florence (Drs Caproni, Cardinali,
Giomi, and Fabbri); Sezione di Dermatologia, Università di Genova,
Dipartimento di Scienze Endocrinologiche e Metaboliche (DISEM), Genoa (Drs
Parodi and Rebora); Dipartimento delle Specialità Medico-Chirurgiche,
Sezione di Dermatologia di Terni, Università di Perugia, Perugia (Dr
Papini); Istituto di Dermatologia, Università degli Studi di Messina,
Messina (Dr Vaccaro); Istituto di Scienze Dermatologiche, Istituto di Ricovero
e Cura a carattere Scientifico (IRCCS) Ospedale Maggiore, Università
degli Studi di Milano, Milan (Dr Marzano); Istituto di Dermatologia, Università
Cattolica del Sacro Cuore, Rome (Dr De Simone); and Istituto Dermopatico dell'Immacolata—IRCCS,
Rome (Dr Fazio), Italy.
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