Eosinophilic myositis/perimyositis: frequency and spectrum of cutaneous manifestations.
BACKGROUND
Eosinophilic myositis/perimyositis (EM/P) are a group of rare idiopathic muscle disorders associated with eosinophilia.


OBJECTIVE
We describe the frequency and spectrum of cutaneous manifestations in EM/P and compare them with the idiopathic hypereosinophilic syndrome (HES).


METHODS
We review the literature on EM/P and describe an additional case associated with angioedema.


RESULTS
Of a total of 26 reported patients with EM/P, cutaneous manifestations were observed in 10. These were, in order of frequency, deep subcutaneous induration, erythema, angioedema, urticaria, and papular lesions.


CONCLUSIONS
Skin lesions occur less frequently in EM/P than in HES. Although erythematous papulonodular lesions and urticaria/angioedema are most commonly observed in HES, the most frequent skin manifestations of EM/P are subcutaneous induration and erythema. In HES, angioedema has been correlated with 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, particularly when muscle lesions are focal, and the principal histopathologic finding is perimysial infiltrates.
Trüeb
R M
RM
Department of Dermatology, University Hospital of Zurich, Switzerland.
Pericin
M
M
Winzeler
B
B
Wüthrich
B
B
Burg
G
G
D000328:Adult; D000368:Aged; D004802:Eosinophilia / Q000150:complications* / Q000473:pathology; D005260:Female; D006801:Humans; D017681:Hypereosinophilic Syndrome / Q000150:complications; D008297:Male; D008875:Middle Aged; D018482:Muscle, Skeletal / Q000473:pathology; D009220:Myositis / Q000150:complications* / Q000473:pathology; D012867:Skin / Q000473:pathology; D012871:Skin Diseases / Q000150:complications*