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Panniculitis in dermatomyositis: A systematic review of the clinicopathologic features
Jonathan D Ho1,2, Trimane McKenzie1
1Division of Dermatology, Department of Medicine, The University of the West Indies, Mona Campus, Kingston, Jamaica.
Background:
Panniculitis in patients with dermatomyositis (PDMS) is rare.
Objectives:
Assess the clinicopathologic features described for PDMS.
Methods:
A systematic review of the PubMed/MEDLINE database was performed. We included cases with dermatomyositis (DMS) and confirmed panniculitis (histology/imaging). Extracted data included demographics, global region of origin, comorbidities, hallmark/nonhallmark features, muscle disease, panniculitis onset relative to classical DMS, symptomatology, physical findings, triggers, autoantibodies, extracutaneous involvement, histopathology, treatment, and prognosis.
Results:
Fifty-eight studies were included (91 patients). PDMS primarily occurred in relatively young women (mean age 35 years, female to male ratio 4.4:1). Adults predominated (adult:juvenile ratio 2.6:1). All had hallmark DMS features. A minority had nonhallmark findings. <1/4 reported extracutaneous involvement (pulmonary complications predominated). Cancer-associated disease was uncommon (7.4%). Panniculitis occurred before/simultaneously/after features of classical DMS. The clinical and histopathologic findings mimic lupus panniculitis, although lipoatrophy was uncommon (12.7%). A lobular/lobular predominant, lympoplasmacytic panniculitis was most common. Treatment regimens included combinations of systemic steroids/traditional steroid-sparers/Janus kinase inhibitors/biologics. The majority had a good/complete response (67.3%), but recalcitrant disease (22.4%) was reported.
Limitations:
Retrospective nature, inconsistent reporting of parameters, lack of longitudinal data.
Conclusions:
PDMS primarily occurs in females, mimics lupus panniculitis, and responds to therapy.
Insights
Panniculitis in dermatomyositis (PDMS) predominantly affects young women and shares features with lupus panniculitis. While often responsive to treatment, some cases may be recalcitrant.
Area of Science:
- Dermatology
- Rheumatology
- Pathology
Background:
- Panniculitis in dermatomyositis (PDMS) is a rare condition.
- Dermatomyositis (DMS) is an idiopathic inflammatory myopathy characterized by muscle inflammation and characteristic skin rashes.
Purpose of the Study:
- To assess the clinicopathologic features of panniculitis in patients with dermatomyositis (PDMS).
Main Methods:
- A systematic review of the PubMed/MEDLINE database was conducted.
- Included studies featured patients with confirmed dermatomyositis (DMS) and panniculitis (histology/imaging).
- Data extracted included demographics, clinical features, histopathology, treatment, and outcomes.
Main Results:
- Ninety-one patients from 58 studies were analyzed, predominantly young women (mean age 35 years, 4.4:1 female to male ratio).
- All patients exhibited hallmark DMS features; extracutaneous involvement, primarily pulmonary, occurred in less than 25%.
- Clinical and histopathologic findings mimicked lupus panniculitis, with a lobular or predominantly lobular lymphoplasmacytic panniculitis being most common; lipoatrophy was infrequent (12.7%).
Conclusions:
- Panniculitis in dermatomyositis primarily affects females and can mimic lupus panniculitis.
- The majority of patients responded well to treatments including systemic steroids, steroid-sparing agents, JAK inhibitors, and biologics.
- Recalcitrant disease occurred in a minority of patients, highlighting the need for tailored therapeutic approaches.
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