Mucous membrane pemphigoid (MMP) with ocular involvement, also referred to as ocular cicatricial pemphigoid, is a rare, autoimmune blistering disease that can have devastating effects for patients. The resulting corneal neovascularization, recurrent corneal abrasions, and ulceration can ultimately result in severe vision loss. We present a 64-year-old man with recalcitrant ocular MMP and consequently advanced corneal involvement that received bilateral keratoprostheses. This case highlights an aggressive but successful placement of bilateral keratoprostheses in recalcitrant ocular MMP, which restored his vision and ultimately his quality of life.

Proliferative verrucous leukoplakia (PVL) is a rare, aggressive form of oral leukoplakia with a substantial risk of malignant transformation. The slowly progressive course and the lack of a single defining histopathologic characteristic for PVL make this entity a diagnostic challenge. We report on a patient who presented with a 7-year history of worsening oral lesions.

Background

Several European countries recently developed international diagnostic and management guidelines for pemphigus, which have been instrumental in the standardization of pemphigus management.

Objective

We now present results from a subsequent Delphi consensus to broaden the generalizability of the recommendations.

Methods

A preliminary survey, based on the European Dermatology Forum and the European Academy of Dermatology and Venereology guidelines, was sent to a panel of international experts to determine the level of consensus. The results were discussed at the International Bullous Diseases Consensus Group in March 2016 during the annual American Academy of Dermatology conference. Following the meeting, a second survey was sent to more experts to achieve greater international consensus.

Results

The 39 experts participated in the first round of the Delphi survey, and 54 experts from 21 countries completed the second round. The number of statements in the survey was reduced from 175 topics in Delphi I to 24 topics in Delphi II on the basis of Delphi results and meeting discussion.

Limitations

Each recommendation represents the majority opinion and therefore may not reflect all possible treatment options available.

Conclusions

We present here the recommendations resulting from this Delphi process. This international consensus includes intravenous CD20 inhibitors as a first-line therapy option for moderate-to-severe pemphigus.