Sleeve and Holster Skin Signs Associated With Dermatomyositis

By
Kazuhiko Hirokawa
Yoshitaka Ueda
Naoto Yokogawa
October 28, 2026

Clinical Rheumatology

At a Glance

Category	Detail
Condition	Anti-NXP-2 antibody-positive dermatomyositis with sleeve and holster signs
Key Mechanisms	The sleeve and holster signs are bilateral, symmetric violaceous macular erythema at the lateral upper arms and lateral hips with proximal thighs. Mechanical stress has been proposed as a possible contributor because these sites are non-sun-exposed and adjacent to bony prominences.
Target Population	Adults with dermatomyositis, including patients with bilateral proximal-extremity erythema and muscle weakness.
Care Setting	Clinical assessment of suspected dermatomyositis, supported in this case by antibody testing, muscle evaluation, MRI, and skin biopsy.

Key Highlights

A 76-year-old woman with anti-NXP-2-positive dermatomyositis had bilateral lateral upper-arm and lateral thigh erythema, proximal weakness, dysphagia, and dysphonia.
The sleeve sign affects the lateral upper arms; the holster sign affects the lateral hips and proximal thighs.
Neither sign is pathognomonic, but both are characteristic cutaneous manifestations of dermatomyositis.
The holster sign has been reported in 40%–51% of adults with dermatomyositis; the sleeve sign is comparatively uncommon and less widely recognized.
After immunosuppressive therapy, the patient's erythema and muscle strength improved, and CK returned to normal.

Guideline-Based Recommendations

Diagnosis

Consider dermatomyositis when bilateral proximal-extremity erythema occurs with muscle weakness, even if classic cutaneous findings are absent.
In the reported case, evaluation included anti-NXP-2 antibody detection, CK measurement, non-contrast MRI, and biopsy of an upper-limb lesion.

Management

The reported patient received immunosuppressive therapy; the article does not specify the regimen.

Monitoring & Follow-up

The case report describes follow-up of erythema, muscle strength, and serum CK after treatment.

Risks

NXP-2-positive dermatomyositis is associated with dysphagia, peripheral edema, calcinosis, and malignancy.
The reported patient had liquid aspiration on swallowing evaluation and a history of diffuse large B-cell lymphoma in complete remission at presentation.

Patient & Prescribing Data

The case concerns a 76-year-old woman with anti-NXP-2-positive dermatomyositis, progressive extremity erythema, proximal muscle weakness, dysphagia, and dysphonia.

Immunosuppressive therapy was followed by improvement in erythema and muscle strength and normalization of serum CK. The article provides no drug, dose, or duration.

Clinical Best Practices

Recognize the sleeve sign on the lateral upper arms and the holster sign on the lateral hips and proximal thighs.
Assess for muscle weakness and swallowing symptoms when these skin findings are present.
Do not treat either sign as pathognomonic; interpret findings in the clinical context.