Severe acral contractures and nail loss in a patient with mechano-bullous Epidermolysis bullosa acquisita

Meißner, Carl; Hoefeld-Fegeler, M.; Vetter, Robert; Bellutti, Michael; Vorobyev, Artem; Gollnick, H; Leverkus, Martin

doi:10.1684/ejd.2010.1002

Cited by 9 publications

(5 citation statements)

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“… 31 , 32 Esophageal stenosis usually occurs in its upper portion and leads to dysphagia, weight loss, malnutrition, and even lung infection from food aspiration. 30 , 32 , 33 , 34 , 35 Major lesions in the respiratory tract can lead to nasal septum perforation, pharyngeal and laryngeal stenosis, and, more rarely, tracheal and bronchial stenosis, which can lead to asphyxia. 36 A multidisciplinary approach is mandatory in these cases ( Fig.…”

Section: Clinical Aspects and Classificationmentioning

confidence: 99%

Epidermolysis bullosa acquisita

Miyamoto¹,

Gordilho²,

Santi³

et al. 2022

Anais Brasileiros de Dermatologia

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Section: Clinical Aspects and Classificationmentioning

confidence: 99%

Epidermolysis bullosa acquisita

Miyamoto¹,

Gordilho²,

Santi³

et al. 2022

Anais Brasileiros de Dermatologia

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“…However, cases of EBA treated with rituximab have rarely been reported. Table 1 provides an overview of previously reported cases of EBA successfully treated with rituximab 4–10 . A total of eight cases with five mechanobullous and two inflammatory types have been reported.…”

Section: Previously Reported Cases Of Epidermolysis Bullosa Acquisitmentioning

confidence: 99%

Successful treatment of epidermolysis bullosa acquisita with rituximab therapy

Kim

Lee

Kim

2011

The Journal of Dermatology

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Dear Editor, Rituximab is a promising therapeutic option for patients with recalcitrant pemphigus, as demonstrated by previous reports. Although rituximab may reduce the disease activity of subepidermal autoimmune blistering skin diseases, 1 the value of rituximab in epidermolysis bullosa acquisita (EBA) is uncertain because there are only a few case reports on the successful use of rituximab in recalcitrant EBA. We report herein a case of recalcitrant EBA successfully treated with rituximab and also provide a review of the previous reported cases of EBA treated with rituximab.A 68-year-old woman was referred to our department with a 4-year history of blister formation on her entire body. Physical examination revealed bullae and erosions on the whole body including oral mucosa. She also showed dystrophic changes such as atrophic scars, milia, loss of finger and toe nails, and digital contracture ( Fig. 1a,c). She complained of swallowing difficulty due to esophageal stricture. Although the patient had been treated with prednisolone (20 mg ⁄ day), azathioprine (150 mg ⁄ day), colchicine (1.2 mg ⁄ day), dapsone (50 mg ⁄ day), and methotrexate (7.5 mg ⁄ week) in another hospital over the past 20 months, her mucocutaneous lesions were refractory to the treatment. Skin biopsy revealed a subepidermal bulla with mild lymphocytic infiltration on the dermis (Fig. 1e). Direct immunofluorescence (DIF) study demonstrated linear deposition of immunoglobulin (Ig) G along the basement membrane zone. Indirect immunofluorescence (IIF) using salt-split skin showed linear deposition of IgG on the dermal side of salt-split sections at a titer of 1:40 (Fig. 1f). The enzyme-linked immunosorbent assay (ELISA) (MBL, Nagoya, Japan) value for type VII collagen was 78.3 U ⁄ mL (cut-off value, 6.14 U ⁄ mL) and total IgG (Cobas Integra 800; Roche Diagnostics, Seoul, South Korea) was 1783.5 mg ⁄ dL (normal reference range, 673-1734 mg ⁄ dL). Based on the clinical, histological, and immunohistochemical studies, we made a diagnosis of classic mechanobullous type of EBA. The patient was treated with a weekly infusion of rituximab at a dose of 375 mg ⁄ m 2 body surface area for 4 weeks. The treatment was well tolerated without any side-effects occurring during rituximab therapy. Rituximab was used in combination with low dose methylprednisolone (8 mg ⁄ day), colchicine (1.2 mg ⁄ day), and dapsone (50 mg ⁄ day). Complete remission with minimal therapy was achieved 4 months after rituximab therapy. Minimal therapy included methylprednisolone (4 mg ⁄ day), colchicine (1.2 mg ⁄ day) and dapsone (50 mg ⁄ day). After complete remission, although IIF titer was maintained at 1:40, ELISA value and total IgG was reduced to 12.4 U ⁄ mL and 1297.2 mg ⁄ dL, respectively. No blister formation was sustained during the 10-month follow-up period (Fig. 1b,d) and she readily swallowed solid foods without any Figure 1. The patient showed blisters and scars on (a) the right thigh and (c) hand. At 6 months after completion of rituximab therapy, marked improvement of t...

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“…Table provides an overview of 16 EBA cases treated with rituximab (Cavailhes et al, ; Crichlow et al, ; Iranzo et al, ; Kim et al, ; Lamberts et al, ; McKinley et al, ; Meissner et al, ; Mercader et al, ; Sadler et al, ; Saha et al, ; Schmidt et al, & Wallet‐Faber et al, ). Prior to initiating rituximab therapy, these patients were treated with various agents as presented in Table .…”

Section: Discussion and Review Of The Literaturementioning

confidence: 99%

The use of rituximab in treatment of epidermolysis bullosa acquisita: Three new cases and a review of the literature

Bevans

Sami

2018

Dermatologic Therapy

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Epidermolysis bullosa acquisita (EBA) is a rare, subepidermal blistering disease affecting the skin and mucous membranes that often remains refractory to standard immunosuppressive therapy. We present three original cases and a review of the literature of 20 cases of refractory EBA treated with rituximab as monotherapy or in combination with other agents. Complete control (with or without therapy) and remission were seen in 56% of patients treated with rituximab monotherapy and 75% of patients treated with rituximab and immunoadsorption (IA). We conclude EBA refractory to standard immunosuppressive therapy may show a more favorable long‐term response to the addition of rituximab; and rituximab in combination with intravenous immunoglobulin or IA may provide utility in terminating acute disease. Additional data are needed to evaluate the safety and long‐term outcomes of rituximab‐based treatment.

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Severe acral contractures and nail loss in a patient with mechano-bullous Epidermolysis bullosa acquisita

Cited by 9 publications

References 0 publications

Epidermolysis bullosa acquisita

Epidermolysis bullosa acquisita

Successful treatment of epidermolysis bullosa acquisita with rituximab therapy

The use of rituximab in treatment of epidermolysis bullosa acquisita: Three new cases and a review of the literature

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