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Summary Background Frontal fibrosing alopecia (FFA) has become one of the most common causes of cicatricial alopecia worldwide. However, there is a lack of clear aetiology and robust clinical trial evidence for the efficacy and safety of agents currently used for treatment. Objectives To enable data to be collected worldwide on FFA using common criteria and assessment methods. Methods A multicentre, international group of experts in hair loss was convened by email to create consensus recommendations for clinical trials. Consensus was defined at > 90% agreement on each recommended part of these guidelines. Results Standardized diagnostic criteria, severity rating, staging, and investigator and patient assessment of scalp hair loss and other clinical features of FFA were created. Conclusions These guidelines should allow the collection of reliable aggregate data on FFA and advance efforts in both clinical and basic research to close knowledge gaps in this condition.

A man in his 20s presented with a 1-month history of extensive and painful vegetating plaques on the lips and buccal and nasal mucosa. His medical history was notable for Crohn disease treated for the previous 6 years with high doses of sulfasalazine (3000 mg/d). A physical examination revealed multiple friable pustules coalescing into vegetativeanderosiveplaqueswithyellowexudateandhemorrhagiccrust on the upper and lower labial mucosa. Similar exophytic plaques were noted to involve the nasal mucosa, resulting in complete obstruction of the nostrils and shortness of breath (Figure). The genital mucosa, intertriginous areas, and scalp were spared. General physical examination results and a review of systems were otherwise normal, and findings from laboratory studies were remarkable for peripheral blood eosinophilia. There was no evidence of active gastrointestinal disease at the time of presentation.A punch biopsy from the lower labial mucosa was performed and revealed epithelial acanthosis, epithelial spongiosis, and intraepithelial neutrophilic and eosinophilic abscesses. Focal suprabasilar clefting and a mixed inflammatory infiltrate within the dermis contained lymphocytes, eosinophils, and neutrophils. There was no evidence of granulomatous inflammation. Results of a direct immunofluorescence study were negative for immunobullous dermatosis. The patient was diagnosed as having a severe and extensive form of pyodermatitis pyostomatitis vegetans (PPV). Treatment with systemic corticosteroids (1 mg/kg/d) was rapidly initiated. Follow-up 1 week later showed a dramatic improvement in the lesions.First described in 1898 by François Hallopeau, PPV is a rare and chronic mucocutaneous dermatosis. Some researchers consider PPV an oral variant of pyoderma gangrenosum, belonging to the spectrum of neutrophilic dermatoses. 1 A strong association with inflammatory bowel diseases is well documented in the literature, particularly with ulcerative colitis. 1,2 Cases associated with primary sclerosing cholangitis have also been reported. Presentations with no underlying systemic disease may be seen. The oral mucosa is the most frequently affected site, but all of the mucosal surfaces can be involved. Clinical presentation is characterized by multiple coalescing pustules on an erythematous base, giving rise to the classic snail

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