Mycophenolate Mofetil in Epidermolysis bullosa acquisita

Kowalzick, L.; Suckow, Susanne; Ziegler, H.; Waldmann, Thomas A.; Pönnighaus, J. M.; Gläser, Volkmar

doi:10.1159/000073104

Cited by 20 publications

(7 citation statements)

References 2 publications

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“…[14] In contrast, our patient has not developed any evidence of conjunctival involvement thus far. [14] In contrast, our patient has not developed any evidence of conjunctival involvement thus far.…”

Section: Discussioncontrasting

confidence: 66%

Premonitory Epidermolysis Bullosa Acquisita Mimicking Eyelid Dermatitis

Kubisch

Diessenbacher

Schmidt

et al. 2010

American Journal of Clinical Dermatology

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We report a unique case of a 71-year-old female patient with epidermolysis bullosa acquisita. The patient initially presented with the clinical symptoms of bilateral eyelid dermatitis that occurred several months prior to the development of oral and pharyngeal erosions and blisters. While no contact allergy was found by patch testing, direct immunofluorescence microscopy demonstrated linear deposits of IgG at the basement membrane zone. By indirect immunofluorescence microscopy on human salt-split skin, IgG antibodies bound to the dermal side of the split. Immunoblot analysis showed predominant IgG4 reactivity of the patient's serum with the recombinant non-collagenous-1 domain of type VII collagen. Because of treatment resistance to systemic corticosteroids, dapsone, and colchicine, we initiated a combination treatment of protein A immunoapheresis and rituximab. With this treatment, complete remission was achieved within 4 months. Our case highlights that epidermolysis bullosa acquisita may initially mimic an eyelid dermatitis. Consequently, dermatologists should be aware of this rare differential diagnosis of eyelid dermatitis where a contact allergy or atopic dermatitis is absent.

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Section: Discussioncontrasting

confidence: 66%

Premonitory Epidermolysis Bullosa Acquisita Mimicking Eyelid Dermatitis

Kubisch

Diessenbacher

Schmidt

et al. 2010

American Journal of Clinical Dermatology

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“…160,161 Less frequently, the diagnosis of IBD follows the development of the blistering disorder. 160,162 The treatment of EBA is represented by immunosuppressants and anti-inflammatory agents, including systemic corticosteroids, dapsone, 163 colchicine, 164 cyclosporine, 165 mycophenolate mofetil, 166 azathioprine, and recently, the anti-CD20 mAb rituximab. 167 …”

Section: Epidermolysis Bullosa Acquisitamentioning

confidence: 99%

Cutaneous Manifestations in Patients With Inflammatory Bowel Diseases

Marzano

Borghi

Stadnicki

et al. 2014

Inflammatory Bowel Diseases

120

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The skin is one of the most common extraintestinal organ system affected in patients with inflammatory bowel disease (IBD), including both Crohn's disease and ulcerative colitis. The skin manifestations associated with IBD are polymorphic and can be classified into 4 categories according to their pathophysiology: (1) specific, (2) reactive, (3) associated, and (4) induced by IBD treatment. Cutaneous manifestations are regarded as specific if they share with IBD the same granulomatous histopathological pattern: perianal or metastatic Crohn's disease (commonly presenting with abscesses, fistulas or hidradenitis suppurativa-like features) is the prototype of this setting. Reactive cutaneous manifestations are different from IBD in the histopathology but have close physiopathological links: pyoderma gangrenosum, a neutrophil-mediated autoinflammatory skin disease typically manifesting as painful ulcers, is the paradigm of this group. Among the cutaneous diseases associated with IBD, the most commonly seen are erythema nodosum, a form of panniculitis most commonly involving bilateral pretibial areas, and psoriasis, a T helper 1/T helper 17-mediated erythematous squamous inflammatory disease. Finally, the number of cutaneous adverse reactions because of IBD therapies is progressively increasing. The most frequent drug-induced cutaneous manifestations are psoriasis-like, eczema-like, and lichenoid eruptions, as well as cutaneous lupus erythematosus for biologics, and nonmelanoma skin cancer, mainly basal cell and squamous cell carcinomas for thiopurines.

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“…Mycophenolate mofetil (2 g/day) was administrated to two patients who were resistant to oral prednisolone and azathioprine treatment. Remission was achieved in one patient, but relapse was noted after the transient improvement of symptoms, and the drug was subsequently ineffective in another patient . Cases in which the p.o.…”

Section: Eba‐cq9: Is the Concomitant Use Of Oral Steroid And Mycophenmentioning

confidence: 98%

Japanese guidelines for the management of pemphigoid (including epidermolysis bullosa acquisita)

Ujiie

Iwata

Yamagami

et al. 2019

The Journal of Dermatology

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The pemphigoid group is a category of autoimmune subepidermal blistering diseases in which autoantibodies deposit linearly at the epidermal basement membrane zone (BMZ). The main subtypes of pemphigoid mediated by immunoglobulin G autoantibodies are bullous pemphigoid (BP), mucous membrane pemphigoid (MMP) and epidermolysis bullosa acquisita (EBA). To establish the first guidelines approved by the Japanese Dermatological Association for the management of pemphigoid diseases, the Committee for Guidelines for the Management of Pemphigoid Diseases (Including EBA) was founded as part of the Study Group for Rare Intractable Skin Diseases under the Ministry of Health, Labor and Welfare Research Project on Overcoming Intractable Diseases. These guidelines aim to provide current information for the management of BP, MMP and EBA in Japan. Based on evidence, the guidelines summarize the clinical and immunological manifestations, pathophysiologies, diagnostic criteria, disease severity determination criteria, treatment algorithms and treatment recommendations. Because of the rarity of these diseases, there are few clinical studies with a high degree of evidence, so several parts of these guidelines were established based on the opinions of the Committee. To further optimize these guidelines, periodic revision in line with the new evidence is necessary.

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Mycophenolate Mofetil in Epidermolysis bullosa acquisita

Cited by 20 publications

References 2 publications

Premonitory Epidermolysis Bullosa Acquisita Mimicking Eyelid Dermatitis

Premonitory Epidermolysis Bullosa Acquisita Mimicking Eyelid Dermatitis

Cutaneous Manifestations in Patients With Inflammatory Bowel Diseases

Japanese guidelines for the management of pemphigoid (including epidermolysis bullosa acquisita)

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