Clinical Manifestations of Mucous Membrane Pemphigoid in a Tertiary Center

Abstract

Mucous membrane pemphigoid (MMP) is a progressive inflammatory disease of autoimmune etiology. We performed a retrospective analysis of clinical signs and treatment on 16 patients. Conjunctival biopsies were performed in all patients and showed typical immuno-deposits at the basement membrane zone. The mean age at presentation was 69 years, 60 % were female.12 patients demonstrated ocular involvement (11 bilaterally). At the time of referral to our hospital, 92 % had reached an advanced stage III or IV. All patients presented conjunctival fibrosis with resultant fornix foreshortening. Trichiasis and symblepharon were found in 11 patients. Keratitis was found in 11 patients resulting in ulceration in 5 cases. Complications required surgical interventions included: entropion surgery (n = 2), tarsorrhaphy (n = 1), amniotic membrane transplantation (n = 2), keratoplasty (n = 1). Systemic immunomodulatory therapy is the treatment of choice. Dapsone (n = 8), steroids (n = 8), azathioprine (n = 5), cyclophosphamide (n = 2), mycophenolate mofetil (n = 4) and methotrexate (n = 1) were used concomitantly or consecutively. Early diagnosis can prevent ocular complications. Immunomodulatory therapy has provided an avenue for preserving vision. The management of MMP requires a multidisciplinary approach.

Zusammenfassung

Das vernarbende Schleimhautpemphigoid ist eine progressive, entzündliche Autoimmunerkrankung. Wir führten bei 16 Patienten eine retrospektive Analyse der klinischen Zeichen und der Therapie der Krankheit durch. Eine Bindehautbiopsie wurde bei allen Patienten durchgeführt und zeigte typische Immunablagerungen im Bereich der Basalmembrane. Das Durchschnittsalter betrug 69 Jahre, 60 % der Patienten waren weiblich. 12 Patienten zeigten eine Augenbeteiligung (11 bilateral). Bei Erstvorstellung erreichten 92 % das fortgeschrittene Stadium III und IV. Alle Patienten hatten eine Bindehautfibrose mit Fornixverkürzung; 11 zeigten Symblepharon und Trichiasis. Keratitis war bei 11 Patienten vorhanden, 5 Fällen davon zeigten Hornhautulzeration. Komplikationen benötigten einen chirurgischen Eingriff: Entropion-Operation (n = 2), Tarsorrhaphie (n = 1), Amniontransplantat (n = 2), Keratoplastik (n = 1). Eine systemische immunmodulatorische Therapie ist die Behandlung der Wahl. Eine Therapie mit Dapson (n = 8), Steroide (n = 8), Azathioprin (n = 5), Cyclophosphamid (n = 2), Mycophenolat mofetil (n = 4) und Methotrexat (n = 1) wurde in Folge oder Kombination eingesetzt. Frühe Diagnose kann Augenkomplikationen vermeiden. Immunmodulatorische Therapie hilft, das Visus zu erhalten. Die Betreuung sollte möglichst in enger interdisziplinärer Kooperation erfolgen.

Key words

MMP - mucous membrane pemphigoid - entropion - symblepharon - dapsone

Schlüsselwörter

vernarbendes Schleimhautpemphigoid - Entropion - Symblepharon - Dapson

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