The so-called amyopathic dermatomyositis is a rare variant of dermatom
yositis which has attracted increasing interest during the last years.
One finds the classical signs of dermatomyositis such as periorbital
edema and erythema, erythematous macular and papular lesions localized
at bony prominences (so-called Gottron's papules), generalized prurit
us, photosensitivity, and a cutaneous histopathologic picture compatib
le with skin lesions of dermatomyositis. Crucial for the diagnosis is
the exclusion of myositis by clinical examination, EMG and histology.
Furthermore, longterm supervision of patients is advisable in order no
t to miss the appearance of early signs of myositis. The longest repor
ted follow-up of amyopathic dermatomyositis patient is 4 years; howeve
r, it cannot be excluded that these cases will eventually culminate in
classical dermatomyositis. In this paper we describe two cases and di
scuss the differential diagnosis and therapy; also, the term ''Premyop
athic dermatomyositis'' is proposed, to indicate that the full picture
is to be expected in most cases.