What is the treatment for alopecia universalis?

Treatment of Alopecia Universalis

For alopecia universalis, baricitinib is the only treatment with high-quality evidence demonstrating significant hair regrowth, while contact immunotherapy remains an option despite low response rates, and wigs provide the most reliable cosmetic solution. 1

First-Line Systemic Treatment

JAK Inhibitors (Preferred)

• Baricitinib (JAK 1/2 inhibitor) is EMA-approved for severe alopecia areata in adults and demonstrates the strongest evidence for efficacy 2

  • Results in significant increase in short-term hair regrowth ≥75% compared to placebo (RR 7.54,95% CI 3.90-14.58) 1
  • Also increases long-term hair regrowth ≥75% (RR 8.49,95% CI 4.70-15.34) 1
  • May result in little to no difference in serious adverse events versus placebo (RR 1.47,95% CI 0.60-3.60) 1

• Ritlecitinib (JAK 3/TEC inhibitor) is EMA-approved for patients aged 12 and older with severe alopecia areata 2

• Tofacitinib has shown success in case reports for alopecia universalis, though evidence is limited to case series 3

• The evidence is very uncertain about oral ruxolitinib versus oral tofacitinib (RR 1.08,95% CI 0.77-1.52) 1

Alternative Systemic Therapies (Off-Label)

Traditional Immunosuppressants

• The evidence is very uncertain about the effect of oral prednisolone or cyclosporine versus placebo (RR 4.68,95% CI 0.57-38.27) for achieving short-term hair regrowth ≥75% 1

• Continuous or pulsed systemic corticosteroids cannot be recommended due to potentially serious side-effects and inadequate evidence of efficacy 4

• Other off-label systemic medications include methotrexate and azathioprine, though evidence for alopecia universalis specifically is limited 2

Contact Immunotherapy

• Contact immunotherapy (DPCP or SADBE) is the best-documented treatment for alopecia totalis/universalis but stimulates cosmetically worthwhile hair regrowth in <50% of patients 4

  • Strength of recommendation C (based on lower quality evidence) 4
  • Requires multiple hospital visits over several months 4
  • Response rate in alopecia universalis is even lower than in extensive patchy disease 4
  • May cause temporary local inflammation but serious side-effects are rare 4

• The evidence is very uncertain about diphencyprone or squaric acid dibutyl ester versus placebo (RR 1.16,95% CI 0.79-1.71) 1

Cosmetic Management

Wigs (Essential Component)

• Wigs are recommended as a primary management strategy (strength of recommendation D) 4
  • For many patients with extensive hair loss, a wig provides the most effective cosmetic solution 4
  • Acrylic wigs are cheaper and easier to maintain but need replacing every 3-4 months with daily wear 4
  • Human hair wigs look more natural and last 1-2 years if maintained properly, but are expensive 4
  • Modern manufacturing techniques allow some wigs to stay in place during sleeping, exercising, swimming, and showering 4

Treatments NOT Recommended

• Dithranol (anthralin) and minoxidil lotion are widely prescribed but there is no convincing evidence that they are effective 4

• PUVA therapy cannot be recommended due to potentially serious side-effects, inadequate evidence of efficacy, high relapse rates, and need for continued treatment leading to unacceptably high cumulative UVA doses 4

• Topical corticosteroids lack convincing evidence of effectiveness in alopecia universalis 4

Critical Clinical Considerations

Prognostic Factors

• Poor prognostic factors include the presence of alopecia universalis itself and positive family history of alopecia areata 5
  • Although initial regrowth may be achieved in 64% of patients, only 14% present persistent response 5
  • The median time between presentation of alopecia areata and development of extensive disease is 1 year (less than 4 years in 91% of patients) 5

Treatment Expectations

• Not treating is the best option in many cases, given the tendency to spontaneous remission and lack of adverse effects on general health 4
  • However, alopecia universalis causes considerable psychological and social disability, particularly in children who often experience bullying including physical aggression 2
  • Treatment should be considered when quality of life is significantly impacted 4

Safety Monitoring

• Adverse side effects from medications occur in 33% of patients 5
• The small incidence of serious adverse events with baricitinib should be balanced against expected benefits 1