Rm. Trueb et al., EOSINOPHILIC MYOSITIS PERIMYOSITIS - FREQUENCY AND SPECTRUM OF CUTANEOUS MANIFESTATIONS, Journal of the American Academy of Dermatology, 37(3), 1997, pp. 385-391
Background: Eosinophilic myositis/perimyositis (EM/P) are a group of r
are idiopathic muscle disorders associated with eosinophilia. Objectiv
e: We describe the frequency and spectrum of cutaneous manifestations
in EM/P and compare them with the idiopathic hypereosinophilic syndrom
e (HES).Methods: We review the literature on EM/P and describe an addi
tional case associated with angioedema. Results: Of a total of 26 repo
rted patients with EM/P, cutaneous manifestations were observed in 10.
These were, in order of frequency, deep subcutaneous induration, eryt
hema, angioedema, urticaria, and papular lesions. Conclusion: Skin les
ions occur less frequently in EM/P than in HES. Although erythematous
papulonodular lesions and urticaria/angioedema are most commonly obser
ved in HES, the most frequent skin manifestations of EM/P are subcutan
eous induration and erythema. In HES, angioedema has been correlated w
ith a favorable prognosis. At least some of these patients apparently
have an idiopathic eosinophilic disorder distinct from HES, including
EM/P. In contrast to HES, the overall prognosis of EM/P is good, parti
cularly when muscle lesions are focal, and the principal histopatholog
ic finding is perimysial infiltrates.