Case report: Successful treatment of non-bullous lichen planus pemphigoides with dupilumab

Abstract

Lichen planus pemphigoides (LPP) is a rare autoimmune bullous disease, characterized by the coexistence of lichen planus and subepidermal bullae. However, the minority of LPP patients present with papules rather than vesicles or blisters, which is defined as non-bullous LPP. The diagnosis of LPP relies on manifestations, histopathology, serological assay, and direct immunofluorescence of linear disposition of IgG and/or C3 at the basement membrane zone. Up to now, no standard therapeutic strategies have been proposed for the treatment of LPP. Herein, we describe an uncommon non-bullous LPP patient with widespread papules and erythema, probably induced by vaccination. During hospitalization, he had a poor response to the conventional treatment of topical and systemic corticosteroids, and his condition was finally alleviated by the addition of dupilumab. For LPP patients with a traditional medication failure, or who were not suitable for a higher dose of corticosteroids, a combination with dupilumab could be an alternative option.

Keywords: corticosteroid; dupilumab; lichen planus; lichen planus pemphigoids; non-bullous; vaccine.

Figure 1

Skin and oral lesions of the patient. (A–D) Widespread red papules, edematous erythema, erosions, and multiple crusts on the neck, trunk, and extremities. (E–G) Close-up view of the abdomen, back, and arm. (H) Violet plaques with white stripes on the right buccal mucosa.

Figure 2

Histopathological and immunofluorescent findings. (A, B) Histopathology demonstrated intercellular epidermal edema, a subepidermal blister with an underlying sparse dermal perivascular infiltrate of lymphocytes and eosinophils. [H&E staining, original magnification, × 50 (A), × 200 (B)]. (C, D) Direct immunofluorescence showed linear depositions of IgG (A) and C3 (B) at the basement membrane zone. (E) Indirect immunofluorescence utilizing monkey esophagus substrate demonstrated a linear deposition of serum IgG along the basement membrane zone.

Figure 3

Lesions on the dorsal aspects of the hands. (A) The edematous erythema and crusts before therapy. (B) The violaceous flat-topped papules and plaques after therapy. (C) By dermatoscope, Wickham's striae were presented, a feature of lichen planus.

Figure 4

The remaining lesions after treatment of dupilumab and systemic corticosteroids for 2 months. (A–C) Most of the red papules, edematous erythema, and erosions cleared and left pigmentation.