What is the treatment for calcinosis cutis?

Treatment of Calcinosis Cutis

Calcinosis cutis is notoriously difficult to treat with no universally effective therapy, but intensification of immunosuppressive therapy should be considered when calcinosis develops or progresses, particularly in the context of dermatomyositis or juvenile dermatomyositis, with diltiazem being the most evidence-supported pharmacologic option for partial response. 1

Primary Treatment Approach

Intensify Underlying Disease Control

• When calcinosis develops or is already established in dermatomyositis/juvenile dermatomyositis, intensification of immunosuppressive therapy is the first-line approach 1
• Early and aggressive treatment of the underlying inflammatory disease (particularly JDM) may prevent or limit calcinosis development, as calcinosis presence suggests active disease and delayed diagnosis 1
• Mycophenolate mofetil (MMF) may be useful for both muscle and skin disease including calcinosis in JDM 1

Pharmacologic Options

Diltiazem is the most commonly used medication with the best evidence for partial response:

• Diltiazem may produce a partial response, though evidence shows only partial efficacy even at standard doses 1
• In clinical practice, diltiazem was most frequently used in monotherapy with partial response in 5 of 8 cases (62.5%) 2
• Lower doses of diltiazem have only partial efficiency 2

Other pharmacologic agents have been tried with minimal or no improvement:

• Colchicine, bisphosphonates, probenecid, warfarin, and intralesional corticosteroids show limited efficacy 1, 3, 4
• Minocycline, ceftriaxone, and aluminum hydroxide have been reported in case reports 3, 4

Emerging therapies for refractory cases:

• Sodium thiosulfate (a vasodilator that chelates calcium) combined with abatacept showed improvement in severe ulcerative skin disease and calcinosis in a case report of recalcitrant JDM, though this is off-label use 1
• Intravenous immunoglobulin may be useful as adjunct therapy, particularly when skin features are prominent 1

Surgical Management

Surgical excision is at least partially effective in localized calcinosis:

• Surgical treatment resulted in at least partial response in all followed cases (n=7,100% at least partial response) 2
• Surgical options include curettage, surgical excision, carbon dioxide laser, and extracorporeal shock wave lithotripsy 3
• Negative pressure wound therapy can be used as an adjunct to surgical management, particularly for ulcerative lesions 5

Treatment Algorithm by Clinical Scenario

For calcinosis in active dermatomyositis/JDM:

1. Intensify systemic immunosuppression first (high-dose corticosteroids + methotrexate or MMF) 1
2. Add diltiazem for symptomatic calcinosis 1
3. Consider IVIG for resistant disease with prominent skin features 1

For localized, symptomatic calcinosis:

1. Surgical excision is preferred for localized disease 2
2. Consider diltiazem as adjunct therapy 1

For severe, refractory calcinosis:

1. Consider sodium thiosulfate (off-label) with or without abatacept 1
2. Rituximab may be considered for refractory disease (takes up to 26 weeks to work) 1
3. Anti-TNF therapies (infliximab or adalimumab preferred over etanercept) for refractory cases 1

Important Clinical Caveats

• Natural history: Calcinosis may eventually regress spontaneously, though joint contractures and secondary infections are potential complications 1
• Location matters: Calcinosis classically occurs at subcutaneous level but may be intramuscular, in fascial planes, or in trauma-prone areas (elbows, knees) 1
• Pain management: Treatment options may need to be adjusted based on pain severity associated with calcinosis 1
• Realistic expectations: The condition is "discouraging to treat" with no treatment showing consistent, complete resolution 1
• Evidence quality: Most treatment evidence consists of case reports and small case series with mixed findings; no controlled trials exist 3, 6