What is the best treatment for alopecia universalis?

Treatment of Alopecia Universalis

For patients with alopecia universalis, JAK inhibitors such as baricitinib (JAK 1/2 inhibitor) for adults and ritlecitinib (JAK 3/TEC inhibitor) for individuals aged 12 and older are the most effective treatment options, as they are the only EMA-approved medications for severe alopecia areata. 1

First-line Treatment Options

Adults

• JAK inhibitors:
  • Baricitinib (JAK 1/2 inhibitor) - EMA-approved for severe alopecia areata 1
  • Consider other JAK inhibitors with emerging evidence:
    • Deucravacitinib (TYK2 inhibitor) - shown rapid hair regrowth in resistant cases 2
    • Upadacitinib (selective JAK1 inhibitor) - demonstrated complete hair regrowth in some cases 3

Adolescents (12 years and older)

• Ritlecitinib (JAK 3/TEC inhibitor) - EMA-approved for severe alopecia areata 1
• Upadacitinib has shown success in adolescents with complete hair regrowth after 3 months of therapy 3

Second-line Treatment Options

1. Contact immunotherapy:

   • Best-documented treatment for severe alopecia areata 4
   • Response rate <50% in alopecia totalis/universalis 4
   • Requires multiple hospital visits over several months 4
   • May cause temporary local inflammation 4

2. Systemic immunosuppressants:

   • Methotrexate (15-25 mg/week) with or without prednisolone 5
   • Cyclosporine - logical choice due to dual immunosuppressive and hypertrichotic properties 4
   • Sulfasalazine - shows partial response but high relapse rate 5

3. Systemic corticosteroids:

   • Used as pulse or continuous therapy 5
   • Concerns about side effects vs. efficacy ratio 5

Practical Management Considerations

Non-pharmacological Approaches

• Wigs or hairpieces are practical solutions for extensive hair loss 4, 5
  • Acrylic wigs: cheaper and easier to maintain
  • Human hair wigs: better fit but more expensive (£195.40 for bespoke human hair wig vs. £50.70 for acrylic wig) 4

Special Considerations

• Children: Limited treatment options as intralesional corticosteroids are poorly tolerated 4
• Psychological impact: Consider psychological support and referral to patient support groups 5
• Monitoring: Regular follow-up visits to assess treatment response 5

Treatment Challenges and Pitfalls

• Spontaneous remission: Consider the tendency for spontaneous remission when deciding on treatment 4
• Misdiagnosis: Avoid misdiagnosing alopecia universalis as trichotillomania or tinea capitis 5
• Treatment expectations: Inform patients that even the best treatments have limited success rates in alopecia universalis
• TNF-α inhibitors: Be aware that TNF-α inhibitors like adalimumab may rarely cause alopecia universalis 6
• Complementary treatments: Limited evidence supports complementary and alternative medicine approaches 7

Treatment Algorithm

1. Confirm diagnosis using trichoscopy and possibly scalp biopsy
2. Assess severity using SALT score (≥20 indicates need for systemic therapy) 1
3. First-line therapy: JAK inhibitors appropriate for age group
4. If inadequate response after 3-6 months: Consider alternative JAK inhibitor or second-line options
5. If all pharmacological options fail: Consider wigs or hairpieces as practical solutions

Remember that alopecia universalis is among the most challenging forms of alopecia to treat, with lower response rates than patchy alopecia areata. Setting realistic expectations with patients is essential.