From the Research
The first line of management for lesions on the palms and soles in patients with ataxia telangiectasia (AT) should involve a combination of intravenous immunoglobulin (IVIG) therapy and topical mometasone 0.1% ointment, as this approach has been shown to effectively heal granulomatous skin lesions in AT patients. This recommendation is based on a case report from 2014 1, which demonstrated the successful treatment of cutaneous granulomas in an AT patient using IVIG and topical mometasone. The use of IVIG therapy aims to modulate the immune system, while topical mometasone helps to reduce inflammation and promote healing.
Key Considerations
- Lesions on the palms and soles in AT patients can be manifestations of telangiectasia or dermatitis related to the underlying immune dysregulation characteristic of the disease.
- Topical corticosteroids, such as mometasone, can help reduce inflammation and associated symptoms like itching or discomfort.
- IVIG therapy can help modulate the immune system and promote healing of granulomatous skin lesions.
- Monitoring for skin thinning with prolonged use of topical corticosteroids is essential, particularly on palms and soles where absorption differs from other body areas.
- Moisturizers should be used regularly between steroid applications to maintain skin barrier function.
Alternative Treatments
If lesions do not respond to the combination of IVIG and topical mometasone within 3-4 weeks, referral to a dermatologist experienced with AT is recommended for consideration of alternative treatments, such as calcineurin inhibitors (tacrolimus, pimecrolimus) or phototherapy. However, the most recent and highest quality study available 1 supports the use of IVIG and topical mometasone as the first line of management.