IV immunoglobulin may lower inflammatory response in patients with epidermolysis bullosa pruriginosa

In patients with epidermolysis bullosa pruriginosa (EBP) who are unresponsive to other therapies, treatment with intravenous immunoglobulin (IVIG) may reduce inflammation, according to a case report published in the International Journal of Dermatology.  

Epidermolysis bullosa pruriginosa is rare a subtype of dystrophic epidermolysis bullosa characterised by intensely itchy skin.

Researchers identified the clinical characteristics of patients diagnosed with EBP from the same family (n = 7), including A heterozygous single nucleotide transition, c.6127G>A, in exon 73 of COL7A1.

Lichenoid papules on extensor surfaces of extremities, excoriations, milia formation, and nail dystrophy were found on all patients, with 86% having elevated serum IgE levels and no history of atopy.

Generalized involvement and severe phenotype were noted in female patients.

Skin lesions were refractory to high dose systemic steroids and cyclosporine treatment but improved significantly with IVIG therapy.

The authors concluded that, “In severe cases, unresponsive to other therapies, IVIG may be a preferable therapeutic approach to modulate the inflammatory response in patients with EBP.”

Reference

Ertop P, Vural S, Gökpınar Ili E, et al.Promising effect of intravenous immunoglobulin therapy for epidermolysis bullosa pruriginosa. Int J Derm. 2020; https://doi.org/10.1111/ijd.14951