Significant improvement of dermatitis herpetiformis with tofacitinib

Dermatol Online J. 2021 Jul 15;27(7). doi: 10.5070/D327754365.

Abstract

Dermatitis herpetiformis (DH) is a rare autoimmune blistering disorder in which patients with celiac disease, a gluten-sensitive enteropathy, present with a severely pruritic papulovesicular eruption over extensor surfaces such as the knees, elbows, lower back, buttocks, and neck. Patients are instructed to adhere to a gluten-free diet for purposes of improving their skin disease and gluten-sensitive enteropathy; this is the only treatment that lowers risk of enteropathy-associated T cell lymphoma. Patients who adhere to a strict gluten-free diet often have remission of their skin disease over months to years. Dapsone is a rapid and extremely effective first-line treatment option and often used while transitioning to a gluten-free diet. Aside from gluten-free diet and dapsone, second-line treatment options include sulfapyridine, sulfasalazine, and colchicine. Some patients have difficulty adhering to a gluten-free diet or develop intolerable side effects to systemic therapies. Furthermore, there is limited data on the use of the second-line treatments. Recent studies have shed light on the role of JAK-STAT-dependent pathways in the pathogenesis of dermatitis herpetiformis. We present a patient treated with tofacitinib, 5mg twice daily, an oral JAK1/3 inhibitor, who demonstrated clinical improvement of DH and control of new lesion development.

Publication types

  • Case Reports

MeSH terms

  • Aged
  • Celiac Disease / complications*
  • Celiac Disease / diet therapy
  • Dapsone / therapeutic use
  • Dermatitis Herpetiformis / diet therapy
  • Dermatitis Herpetiformis / drug therapy*
  • Dermatitis Herpetiformis / etiology
  • Dermatitis Herpetiformis / pathology
  • Diet, Gluten-Free
  • Drug Administration Schedule
  • Humans
  • Janus Kinase 1 / antagonists & inhibitors
  • Janus Kinase 3 / antagonists & inhibitors
  • Male
  • Patient Compliance
  • Piperidines / administration & dosage*
  • Protein Kinase Inhibitors / administration & dosage*
  • Pyrimidines / administration & dosage*
  • Remission Induction / methods
  • Treatment Outcome

Substances

  • Piperidines
  • Protein Kinase Inhibitors
  • Pyrimidines
  • tofacitinib
  • Dapsone
  • JAK1 protein, human
  • JAK3 protein, human
  • Janus Kinase 1
  • Janus Kinase 3