Successful treatment with thalidomide for pemphigus vulgaris

Abstract

Background: Pemphigus vulgaris (PV) is a potentially life-threatening mucocutaneous autoimmune blistering disease characterized by suprabasal acantholysis, causing painful mucocutaneous blisters and erosions. Current mainstay therapy for pemphigus is systemic corticosteroids in combination with or without immunosuppressive adjuvants, which may cause severe adverse effects and seriously impact on the quality of life in pemphigus patients. The objective of this study was to evaluate the efficacy and safety of thalidomide therapy in patients with PV. Methods: This study examined six PV patients from June 5, 2017, to November 11, 2018, in the dermatology department of Peking Union Medical College Hospital. Treatment with thalidomide was applied at a dose of 50–100 mg/day for disease control. Results: The mean age of the six patients (two male and four female patients) at the time of thalidomide therapy initiation was 50.2 years (range: 38–67 years), and the total duration of follow-up after thalidomide therapy was 13.2 months (range: 5–25 months). All patients responded favorably to thalidomide treatment, and three patients showed a dramatic reduction in anti-Dsg3 autoantibodies in the serologic examinations within 1 year. Five patients were found to have mucosal involvement. Mild adverse effects were noted in three patients, which could be managed after the application of symptomatic treatment and did not interfere with the pemphigus therapy. Conclusion: These results demonstrate that thalidomide could be an effective and safe option for PV patients, especially those who are concerned about steroid-induced severe complications, and have mucosal diseases.

Complete remission of intractable bullous erythema multiforme with thalidomide therapy: A case series

“…Currently, thalidomide is considered 99% effective for erythema nodosum leprosum (type II) and a variety of off-label uses, including HIV-associated mucosal ulceration, aphthae associated with neutrophilic dermatoses, including Behcet disease as well as intractable bullous oral mucous membrane disorders, such as oral pemphigus vulgaris and cicatricial pemphigoid. 7 , 8 , 9 …”

Thalidomide as a potential adjuvant treatment for paraneoplastic pemphigus: A single‐center experience

“…Prior use of thalidomide in pemphigus and pemphigoid groups, namely, PV, BP, cicatricial pemphigoid, and Hailey‐Hailey pemphigus, has been reported to be effective 10 . Three sporadic previous cases of PNP with UCD were also treated with thalidomide as part of the immunomodulatory therapy applied.…”

“…The mortality rate of the patients with benign neoplasms was 33%, which was much lower than the rate of has been reported to be effective. 10 Three sporadic previous cases of PNP with UCD were also treated with thalidomide as part of the immunomodulatory therapy applied. Two of these patients obtained satisfactory outcomes, while one died from respiratory failure.…”

Therapy of autoimmune mouth bullous disease: 2020 review

“…Bullous pemphigoid is a nonhereditary bullous dermatosis, with a chronic-relapsing course and a relatively benign prognosis, affecting predominantly elderly subjects over 60 years of age. 2,[27][28][29] The disease is characterized clinically by large tense blisters often arising on erithematoedematous skin and histologically by the development of non acantholytic cavities in the most superficial part of the lamina lucid of DEJ. BP is considered an autoimmune dermatosis caused by the activity of IgG autoantibodies directed against specific antigens of the lamina lucid and by the activation of autoreactive T lymphocytes (CD4 with the Th2 cytokine profile).…”