FFA with Sterile Pustules: The Pustular FFA Variant

The Pustular Variant of Frontal Fibrosing Alopecia

Frontal fibrosing alopecia is a scarring alopecia that seems to be increasing in incidence worldwide. As we learn more and more about FFA and as more and more patients come to be diagnosed, it’s not surprising that more and more variants are uncovered. Variants refer to different clinical or histopathological forms of the disease

Lobato-Berezo et al, 2022

Authors of a new study describe a new pustular variant of FFA. Patients with FFA normally has redness and scale around their frontal hairs - but not usually pustules.

The patient in the report was a 65-year-old woman presented with a 10-year history of FFA. She had never received treatment for this condition. She had not had any local trauma or traction alopecia type forces that might account for infection or pustules. She was not receiving treatment for any medical condition. She also was not applying any routine facial cosmetics that might plug pores and give a pustular FFA appearance. The patient noted that the pustules were present throughout the period and progressively increasing. No other facial or body sites were involved with pustules.

Her physical examination showed many typical FFA features with hairline recession and perifollicular erythema and scale. However, she had multiple pustules around the hair follicles. A skin swab for bacterial and fungal culture was sterile meaning that it did not grow any organisms. A vertical section of a punch biopsy revealed typical FFA findings with a perifollicular lymphohistiocytic infiltrate in the middle and superior part of the dermis with follicular epithelial vacuolar changes and plasma cells. howeer, what was different in this case was the intrafollicular and intraepidermal pustules that were present! These were filled with abundant neutrophils Gram and periodic acid–Schiff stain ruled out bacterial and fungal infection.

The patient was treated with topical clobetasol cream was prescribed for 1 month with complete clearance of the pustular lesions. No flare-ups were observed after 3 months of follow-up.

The authors propose a new pustular variant of FFA.

Comment

This is interesting and something for us all to be on the lookout for. While the authors propose that this could be a variant of the LPP-FD phenotypic spectrum, I’m not so sure we can make this leap in this case. It certainly could be but this patient’s long history of these pustules and the fact they are sterile, certainly makes it possible that this is not related to folliculitis decalvans. In fact, one wonders whether the pustules are more related to other immune mediated neutrophilic processes like psoriasis.

Reference

Lobato-Berezo et al. Pustular frontal fibrosing alopecia: a new variant within the folliculitis decalvans and lichen planopilaris phenotypic spectrum? Br J Dermatol. 2022 May;186(5):905-907.