Juvenile Pemphigus Foliaceus in a Patient With Psoriasis Receiving Narrow-Band Ultraviolet-B: Successful Treatment With Rituximab

August 2023 | Volume 22 | Issue 8 | 830 | Copyright © August 2023


Published online July 7, 2023

doi:10.36849/JDD.7241

Jenna Yousif BS, Alice B. Gottlieb MD PhD, Roudha Al-Dehneem MD MSc

Department of Dermatology, Icahn School of Medicine at Mount Sinai, New York, NY

Abstract
Pemphigus foliaceus is an autoimmune blistering disease of the skin that is not frequently associated with mucous membrane involvement. It is characterized by immunoglobulin G (IgG) antibodies against desmoglein-1, a component of epidermal intercellular adhesion, in the granular layer of the epidermis. Pemphigus foliaceus consists of scattered, arcuate, crusted erythematous lesions often in a seborrheic distribution that may progress to diffuse skin involvement and exfoliative erythroderma. Several cases in the literature discuss pemphigus foliaceus arising in patients with pre-existing psoriatic disease following treatment with narrow-band ultraviolet-B (NB-UVB) therapy. Although this is a rare occurrence and the exact mechanism of this phenomenon remains unclear, providers should be aware of this association to better improve management and care. We present a case of a 16-year-old-male who developed pemphigus foliaceus following NB-UVB treatment for psoriasis.

Yousif J, Gottlieb AB, Al-Dehneem R. Juvenile pemphigus foliaceus in a patient with psoriasis receiving narrow-band ultraviolet-b: successful treatment with rituximab. J Drugs Dermatol. 2023;22(8):830-831. doi:10.36849/JDD.7241

INTRODUCTION

There have been several case reports of pemphigus foliaceus arising in patients with psoriasis vulgaris treated with narrow-band ultraviolet B (NB-UVB) therapy.1 It is thought that treatment with NB-UVB can trigger desmoglein autoantibodies in pemphigus foliaceus by damaging the dermal-epidermal junction.2 Herein, we report a case of juvenile pemphigus foliaceus following NB-UVB therapy in a patient with a history of psoriasis vulgaris.

CASE REPORT

A 16-year-old male with no significant past medical history presented with a 14-month history of pruritic erythematous silver, scaly plaques on his scalp, face, chest, back, and legs sparing mucosal membranes with additional nail pitting. A family history of psoriasis was notable in his father and uncle. A skin biopsy was consistent with psoriasis vulgaris. After failing topical corticosteroid therapy, systemic treatment was initiated with ustekinumab with mild improvement in his psoriasis. 

Ustekinumab was discontinued and secukinumab was initiated. However, he showed only slight improvement in his skin lesions following 3 months of secukinumab, which was discontinued. NB-UVB therapy was added for 11 cycles, but he began to progress to erythroderma with diffuse and generalized painful arcuate and polycyclic plaques with excoriations and scaling, and a few blisters that ruptured leaving superficial erosions (Figure 1). No mucosal lesions or joint pains were present. NB-UVB therapy was discontinued, and he was started on 




prednisone 60 mg daily. A repeat skin biopsy demonstrated an intracorneal split and direct immunofluorescence showed granular immunoglobulin G (IgG) deposition consistent with pemphigus foliaceus. Additionally, his blood was positive for anti-desmoglein-1 antibodies. Considering the adverse effects of