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DIAGNOSIS: Brunsting-Perry Cicatricial Pemphigoid
HISTOPATHOLOGY: Revealed a subepidermal bulla with mild superficial perivascular lymphohistiocytic infiltrate and evidence of a dermal scar. Direct immunofluorescence showed linear granular basement membrane positivity for IgG, IgM, and complement (Cí3). (Fig 3-4)
COURSE: Initial evaluations by otolaryngology and ophthalmology were normal. Control of scalp lesions was achieved with a class I steroid ointment. The patient subsequently developed erosive gingival lesions approximately one month after her initial presentation, which have waxed and waned (Fig 5). Her oral lesions are well controlled topically as well. She will also receive annual ophthalmic monitoring for the development of ocular involvement. It remains to be seen if she will progress to the chronic scarring mucosal lesions and eye involvement characteristic of classic cicatricial pemphigoid.
DISCUSSION: Cicatricial pemphigoid is an autoimmune condition characterized by subepidermal blisters of the mucosal membranes, resulting in scarring at involved sites. The most common sites of involvement are the oral mucosa and the conjunctiva. The skin is involved in one-quarter to one-third of cases, although it rarely precedes mucosal involvement and often does not result in scarring. Patients with cicatricial pemphigoid have circulating antibodies specific for BPAg2, BPAg1, laminin-5, or EBA.
The Brunsting-Perry variant is a rare form of cicatricial pemphigoid first described in 1957, and is characterized by recurrent vesicles and/or bullae involving the skin of the head and neck region. Lesions are often described as pruritic, and they heal with superficial scarring and milia formation, which on the scalp is manifested as cicatricial alopecia. Occasionally in the Brunsting-Perry variant mucous membranes are involved, but it is often reported to be transient and nonscarring. However, 2-8 % of individuals with the Brunsting-Perry variant will progress to develop classic cicatricial pemphigoid with scarring mucosal and ocular involvement; this can occur up to 20 years after the appearance of the original skin lesions. Skin lesions on the scalp were the presenting symptom in 2 of 81 cicatricial pemphigoid cases in one large report.
Histopathology in the Brunsting-Perry variant shows subepidermal blisters like that seen in classic cicatricial pemphigoid, and direct immunofluorescence (DIF) uniformly shows linear staining for IgG. DIF has occasionally also been reported to be positive for complement and IgM. Although it was not performed in our patient, indirect immunofluorescence is only occasionally positive for anti-basement membrane zone antibodies, similar to what is seen in classic cicatricial pemphigoid. In the original report in 1957, several of the patients had peripheral eosinophilia, ranging from 2% to 5.5% (our patient had 7.5% eosinophils), and more than 50% of cells in the blister fluid were eosinophils in several cases.
Treatment of Brunsting-Perry cicatricial pemphigoid is aimed at controlling local disease with intralesional steroids or topical steroids. If more severe disease occurs with ocular and mucosal lesions, systemic treatment with immunosuppressants including corticosteroids or dapsone can be used. In general however, this disease tends to be recalcitrant to therapy.
REFERENCES:
1.Brunsting LA, Perry HO. Benign Pemphigoid? A Report of Seven Cases with Chronic, Scarring, Herpetiform Plaques About the Head and Neck. (1957) Arch Derm:75, 489-501.
2.Fleming TE, Korman NJ. Cicatricial Pemphigoid. (2000) JAAD:43, 571-591.
3.Hardy KM, Perry HO, Pingree GC, et al: Benign Mucous Membrane Pemphigoid. (1971) Arch Derm:104, 467-475.
4.Jacoby WD et al. Cicatricial Pemphigoid (Brunsting-Perry Type). Case Report and Immunofluorescence Findings. (1978) Arch Derm:114, 779-781.
5.Michel B et al. Cicatricial Pemphigoid of Brunsting-Perry. Immunofluorescence Studies. (1977) Arch Derm:113, 1403-1405.
6.Yancey, B: Dermatology in General Medicine, Fifth Edition: 674-679.
My thnks to Drs. Eva Hurst, Saadia Raza and Michael Heffernan for their assistance in the preparation of this case
