Immunologic underpinnings and treatment of morphea

Expert Rev Clin Immunol. 2022 May;18(5):461-483. doi: 10.1080/1744666X.2022.2063841. Epub 2022 May 4.


Introduction: Morphea is a chronic autoimmune fibrosing condition of the skin and underlying tissue with the potential for significant disease-associated morbidity. While the exact etiology of morphea is not fully elucidated, many studies have explored the immunologic drivers of this disease.

Areas covered: Using PubMed, we performed a systematic review on morphea, with a focus on both the immune-mediated pathophysiology and treatment of this disease. Based on these findings, we review the literature surrounding what is understood about the role of the immune system in disease onset and course. Additionally, we discuss current treatments used in this disease as well as the potential role for more targeted therapies in the future.

Expert opinion: Much work remains to fully elucidate each step in the immunologic march causing morphea. However, there is evidence to suggest that the early inflammatory stages of morphea may be driven predominantly by immunologic events in the Th1/Th17 pathway, while the Th2 pathway may be responsible for the fibrosis and damage observed later in the disease. Standard of care treatments currently continue to focus on therapeutics with broad immune modulating properties. Further work exploring the immunologic underpinnings of morphea will facilitate more targeted treatment approaches over time.

Keywords: Morphea; autoimmune skin disease; connective tissue disease; en coup de sabre; fibrosing disorder; localized scleroderma.

Publication types

  • Systematic Review

MeSH terms

  • Autoimmune Diseases*
  • Graft vs Host Disease*
  • Humans
  • Scleroderma, Localized* / therapy
  • Skin
  • Th17 Cells