ABSTRACT
Objective To firstly report 4 cases of dermatomyositis characterized by painful palmar eruptions complicated by fatal rapidly progressive interstitial lung disease (RP-ILD) in China.Methods Four patients with dermatomyositis with painful palmar eruptions complicated by fatal RP-ILD were enrolled from the Department of Dermatology,Zhongshan Hospital,Fudan University between December 2014 and April 2017,and their clinical and pathological features were analyzed.Results Among these patients,3 were female and 1 was male.Their age ranged from 47 to 59 years.Of the 4 patients,3 had no muscular involvement.All of the 4 patients had multiple solid red papules or nodules on the bilateral palms,palmar and lateral surfaces of fingers,which preceded,followed or concurred with the onset of other skin lesions of dermatomyositis.The occurrence of type Ⅰ respiratory failure was preceded by 3 weeks to 5 months of painful palmar eruptions in the 4 patients.Early-stage palmar eruptions were easily misdiagnosed as contact dermatitis,eczema or erythema multiforme.Histopathological examination of the skin lesions on the finger palmar surface showed perivascular infiltration of a few lymphocytes in the dermis,and deposition of varying amounts of mucin-like substances around blood vessels and appendages.Of the 4 patients,3 showed positive staining for anti-melanoma differentiation-associated gene 5 antibody.Although the 4 patients received anti-inflammatory and immunosuppressive therapies,they all finally died of respiratory failure.Conclusions Dermatomyositis with painful palmar eruptions may indicate the occurrence of fatal RP-ILD,and early biopsy of skin lesions is needed to help to identify the disease.Immunosuppressive treatment should be performed timely to improve the prognosis in these patients.
ABSTRACT
Objective To analyze clinical and pathological features of dermatomyositis with panniculitis as a skin manifestation.Methods Clinical data were collected from 9 cases of dermatomyositis with panniculitis as a skin manifestation in Department of Dermatology of Zhongshan Hospital affiliated to Fudan University from October 2012 to July 2016,and their clinical and pathological features were analyzed.Results Of the 9 cases,6 were female and 3 were male,and the age ranged from 28 to 73 years.Panniculitis lesions of the 9 patients all manifested as painful indurated plaques or nodules on the buttock,thigh,waist,back,abdomen,upper extremities and cheeks.These lesions occurred before,after or simultaneously with the onset of characteristic skin and muscle lesions of dermatomyositis,especially preceded the onset of characteristic lesions of dermatomyositis by 30 years in 1 case.Histopathological examination of lesions showed liquefaction degeneration of basal cells,inflammatory infiltration of lymphocytes and plasma cells around blood vessels,in the fat lobules as well as between the lobules and septa in the dermis.The necrosis and calcification of lipocytes,lipomembranous changes,fibrinoid necrosis of damaged vessel walls and microvascular occlusion were observed in some cases.Because panniculitis preceded the onset of characteristic lesions of dermatomyositis,2 patients were misdiagnosed with lupus panniculitis and morphea profunda for several times.Most patients had good response to systemic glucocorticoids combined with immunosuppressive agents,while the patients with lipomembranous fat necrosis had poor response to the combination therapy.Conclusions Panniculitis lesions of dermatomyositis are histologically characteristic,and may do not coincide with the onset of characteristic lesions of dermatomyositis.If panniculitis lesions precede characteristic lesions of dermatomyositis,patients will be easily misdiagnosed.Thus,persistent follow-up visit will be of great importance for the diagnosis.