Mucosal Morbidity in Patients With Epidermolysis Bullosa Acquisita

Luke, Markham C.; Darling, Thomas N.; Hsu, Richard; Summers, Ronald M.; Smith, Janine A.; Solomon, Beth; Thomas, Giovana R.; Yancey, Kim B.

doi:10.1001/archderm.135.8.954

Cited by 79 publications

(55 citation statements)

References 31 publications

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“…Mucous membrane pemphigoid is associated with major morbidity, potentially leading to definitive functional sequelae and/or death in the case of laryngeal, conjunctival, tracheal, and/or esophageal involvement. [1][2][3][4] For patients with moderate forms of MMP involving only oral mucosa and/or skin and whose scars have mild adverse impact, usual treatments include topical corticosteroids, dapsone, sulfasalazine, and tetracyclines, used either alone or in combined regimens. 1,[5][6][7] However, patients with severe involvement or those responding poorly to the usual treatments require alternative systemic immunosuppressive/ immunomodulatory therapies, for example, systemic corticosteroids, cyclophosphamide, cyclosporine, and/or intravenous immunoglobulins (IVIg).…”

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confidence: 99%

Rituximab for Patients With Refractory Mucous Membrane Pemphigoid

Roux–Villet¹,

Prost-Squarcioni²,

Alexandre³

et al. 2011

Arch Dermatol

126

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Background: Mucous membrane pemphigoid (MMP) still represents a potentially life-and sight-threatening disease. In a subset of patients with severe MMP, conventional immunosuppressants are ineffective or contraindicated.Observations: Twenty-five patients with severe refractory MMP, including 5 with mucous membranedominant epidermolysis bullosa acquisita, received 1 or 2 cycles of rituximab (375 mg/m 2 weekly for 4 weeks). Twenty-one of the patients were receiving concomitant therapy with dapsone and/or sulfasalazine therapy, which was maintained during rituximab cycles. Complete responses in all affected sites (ocular and/or extraocular) were obtained in 17 patients (68%) by a median time of 12 weeks after the first cycle, and 5 additional patients responded completely after a second cycle, yielding an 88% complete response rate. In all but 1 of the 10 patients with ocular lesions, their eyes became noninflammatory within a mean of 10 weeks. Among the 3 patients (12%) who developed severe infectious complications, 2 (8%) died; they had been receiving concomitant conventional immunosuppressants and high-dose corticosteroids and were hypogammaglobulinemic. Treatment with immunosuppressants was discontinued for all other patients, and no other infection was observed. Ten patients experienced relapse after a mean of 4 (range, 1-16) months after achieving complete responses.Conclusions: Rituximab appears to have rapid and dramatic efficacy in patients with severe, refractory MMP. The occurrence of severe infections in patients receiving concomitant conventional immunosuppressants supports using rituximab without other immunosuppressants. Controlled prospective studies are warranted to define an optimal treatment protocol.

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confidence: 99%

Rituximab for Patients With Refractory Mucous Membrane Pemphigoid

Roux–Villet¹,

Prost-Squarcioni²,

Alexandre³

et al. 2011

Arch Dermatol

126

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“…All squamous cell epithelia can be affected, including oral and nasal mucosa, larynx, esophagus, anal and genital mucosa as well as conjunctiva [14,[93][94][95]. While 50-65% of EBA patients have mucosal lesions [26,96,97] (Figure 5) in about 5-10% of patients, mucosal involvement predominates and patients may be classified as mucous membrane pemphigoid-variant of EBA [27,85].…”

Section: Mucous Membrane Pemphigoid-like Variantmentioning

confidence: 99%

Clinical features and diagnosis of epidermolysis bullosa acquisita

Vorobyev

Ludwig

Schmidt

2016

Expert Review of Clinical Immunology

100

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Introduction: Epidermolysis bullosa acquisita (EBA) is a rare autoimmune blistering disease of skin and mucous membranes. EBA is caused by autoantibodies against type VII collagen, which is a major component of anchoring fibrils, attaching epidermis to dermis. Binding of autoantibodies to type VII collagen leads to skin fragility and, finally, blister formation. The clinical picture of EBA is polymorphic, with several distinct phenotypes being described. Despite recent progress in understanding the pathophysiology of EBA, its diagnosis is still challenging. Areas covered: This review provides an update on the clinical manifestations and diagnostic methods of EBA. We searched PubMed using the terms 'epidermolysis bullosa acquisita' covering articles in English between 1 January 2005 and 31 May 2016. Relevant older publications were retrieved form cited literature. Expert commentary: While the clinical picture is highly variable, diagnosis relies on direct immunofluorescence (IF) microscopy of a perilesional skin biopsy. Linear deposits of IgG, IgA and/or C3 along the dermal-epidermal junction with an u-serrated pattern are diagnostic for EBA alike the detection of serum autoantibodies against type VII collagen. Several test systems for the serological diagnosis of EBA have recently become widely available. In some patients, sophisticated diagnostic approaches only available in specialized centers are required. ARTICLE HISTORY

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“…EB is characterized by soft tissue blistering, which results in tissue separation and scarring. As an outcome, adhesions are developed which result in reduced tongue mobility (tongue tie) 4 . Correlated AG appears as an isolated anomaly, but it is also linked to several craniofacial abnormalities such as the X-linked cleft palate that is mentioned above.…”

Section: Aetiologymentioning

confidence: 99%