Case Report: Morbihan disease treated with tofacitinib successfully

Area of Science:

• Dermatology
• Immunology
• Pharmacology

Background:

• Morbihan disease is a rare condition characterized by chronic facial edema and lymphedema.
• Current treatment options for Morbihan disease are limited and lack standardization, often including corticosteroids, antibiotics, and surgery.
• Janus kinase (JAK) inhibitors, such as Tofacitinib, are utilized in managing inflammatory and autoimmune disorders.

Observation:

• Two patients diagnosed with Morbihan disease presented with progressive facial edema and eyelid swelling.
• Skin biopsies revealed characteristic findings of perivascular dermal edema, lymphatic vessel dilatation, and inflammatory cell infiltration.
• Both patients were treated with Tofacitinib (5mg, orally twice daily).

Findings:

• Patient 1 experienced significant reduction in facial edema and erythema after one month of Tofacitinib treatment, with sustained improvement over six months.
• Patient 2 showed gradual lesion improvement within one week of Tofacitinib therapy, with no recurrence during a six-month follow-up period.
• Short-term Tofacitinib administration resulted in substantial clinical success for both individuals with Morbihan disease.

Implications:

• Tofacitinib represents a potential novel oral therapeutic agent for Morbihan disease.
• These findings warrant further investigation into the efficacy and safety of Tofacitinib for Morbihan disease through clinical trials.
• This study highlights the role of JAK inhibition in managing rare inflammatory dermatological conditions.