|Year : 2022 | Volume
| Issue : 4 | Page : 243-244
Treatment of recalcitrant pemphigoid vegetans in an elderly patient with rituximab
Yi-Wei Huang, Wei-Hsin Wu
Department of Dermatology, National Taiwan University Hospital, Taipei, Taiwan
|Date of Submission||08-Apr-2022|
|Date of Decision||25-Jul-2022|
|Date of Acceptance||26-Jul-2022|
|Date of Web Publication||08-Oct-2022|
Dr. Wei-Hsin Wu
Department of Dermatology, National Taiwan University Hospital, No. 7 Chung San South Road, Taipei
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Huang YW, Wu WH. Treatment of recalcitrant pemphigoid vegetans in an elderly patient with rituximab. Dermatol Sin 2022;40:243-4
We report an otherwise healthy 90-year-old man who presented to our clinic with painful blisters on the trunk, face, and extremities [Figure 1]a. Skin biopsy revealed a subepidermal blister with a mixed inflammatory infiltrate comprising lymphocytes, neutrophils, and many eosinophils in the blister cavity and underlying dermis, as shown in [Figure 2]a. Direct immunofluorescence (DIF) showed linear deposition of immunoglobulin G (IgG) and C3 along the basement membrane zone. Indirect immunofluorescence showed a negative result for intercellular substance antibodies and found the basement membrane zone antibody was 1:160 positive. A diagnosis of bullous pemphigoid was made. In response, we prescribed prednisolone 0.5 mg/kg/day and a course of rituximab, which consists of two 1000 mg infusions given 2 weeks apart. The Bullous Pemphigoid Disease Area Index (BPDAI) decreased from 49 to 7; therefore, prednisolone was gradually tapered to 5 mg per day. The patient achieved complete remission, and the basement membrane zone antibody was undetectable during the 9-month follow-up.
|Figure 1: (a) Tense blisters suggestive of bullous pemphigoid on the right lower legs of a 90-year-old man. (b) Many well-defined vegetative plaques accompanied by erosions on the lateral thighs and buttocks developed 16 months after the initial presentation of tense bullae, which was consistent with pemphigoid vegetans. (c) Close-up photo of the vegetative plaques on the left lateral thigh. (d) Clearing of the plaques on the left lateral thigh after administration of rituximab.|
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|Figure 2: (a) The first biopsy specimen showed a subepidermal blister with mixed inflammatory cells, including many eosinophils. (b and c) Microscopic examination of the second skin biopsy demonstrated marked psoriasiform hyperplasia with dermoepidermal junctional separation and many dermal eosinophils, which may be consistent with pemphigoid vegetans clinically. (d) Direct immunofluorescence of the second biopsy highlighted linear deposition of C3 along the basement membrane zone.|
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During a follow-up of 16 months after the initial presentation, the patient stopped taking prednisolone due to medication nonadherence. Large bullae developed on his hands and lower limbs, with well-circumscribed erosive and vegetative plaques on the groin and thighs [Figure 1]b and [Figure 1]c appearing in the following month. The plaques were unresponsive to 8 weeks of prednisolone 0.5 mg/kg/day, topical clobetasol propionate, and tetracycline ointment. BPDAI worsened to 69, with peripheral blood eosinophilia (eosinophil count 860/μL). Microscopic findings from a second biopsy of the left lateral thigh showed marked psoriasiform hyperplasia [Figure 2]b and [Figure 2]c. The dermis showed papillary dermal edema and an inflammatory infiltrate that comprised lymphocytes, neutrophils, plasma cells, and many eosinophils. Focal exocytosis of eosinophils and subepidermal clefting were noted. Immunohistochemically, a C4D stain indicated continuous expression along the basement membrane. DIF showed linear deposition of C3, but not IgG, along the basement membrane zone [Figure 2]d. Enzyme-linked immunosorbent assay (ELISA) revealed anti-BP180 IgG >200.0 RU/ml (index ≤20.0 RU/ml). Microscopic findings and immunological features support the diagnosis of pemphigoid vegetans. A culture of erosive scrotal lesions yielded methicillin-sensitive Staphylococcus aureus and Proteus mirabilis. We provided wound care with topical tetracycline ointment and clobetasol propionate ointment. The patient achieved complete resolution with another course of rituximab in addition to systemic corticosteroid [Figure 1]d.
Pemphigoid vegetans was reported to be a rare variant of bullous pemphigoid, which is characterized by the development of vegetative plaques and purulent discharge, especially on the intertriginous area, buttocks, and thighs. The clinical appearance resembles pemphigus vegetans, but histopathological and immunological features are consistent with bullous pemphigoid. Autoantibodies against BP180 and BP230 were found in patients with pemphigoid vegetans., The diagnosis of our patient was supported by the typical distribution, histopathological findings, DIF, and ELISA.
Many therapeutic modalities have been used for pemphigoid vegetans, including topical antibiotics, topical or systemic corticosteroids, dapsone, minocycline, and a combination of these choices. Rituximab is an emerging choice for the treatment of severe and recalcitrant pemphigoid diseases, owing to its high remission rates and favorable safety profile., Rituximab was chosen over other immunosuppressants in this elderly patient due to its effectiveness and tolerability. The current case is the first report demonstrating an excellent clinical response to rituximab in a recalcitrant case unresponsive to a high-dose systemic corticosteroid. Total clearance of cutaneous eruptions was achieved, and no relapse was observed in 3 months.
Bacterial colonization has been observed in pemphigoid vegetans,,, and some authors speculate that bacterial infections cause its vegetative and papillomatous lesions. Staphylococcus aureus and Proteus mirabilis were detected in our patient, who had been diagnosed with bullous pemphigoid, suggesting a possible association between bacterial colonization and the development of vegetative plaques.
The publication of this case is important due to the temporal, clinical, and histopathological changes exhibited by the patient. In addition, rituximab may be beneficial in patients with intractable pemphigoid vegetans.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given his consent for his images and other clinical information to be reported in the journal. The patient understands that name and initials will not be published, and due efforts will be made to conceal his identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2]