A large scalp ulceration.

A 57-year-old Caucasian man presented with a 3-month history of an expanding irregularly shaped superficial ulcer on the vertex of the scalp with associated hair loss. The patient reported blisters and pustules prior to the onset of the ulcer and alopecia. He denied other skin or oral lesions and specifically denied picking at the scalp. At the time of presentation, physical examination revealed a superficial ulcer, measuring roughly 15 × 8 cm (Fig 1), on the vertex of the scalp. A shave biopsy (Fig 2) was initially performed, followed by 2 punch biopsies; one for hematoxylin and eosin staining, and one for direct immunofluorescence (Fig 3).

Fig 1

Fig 2

Fig 3

Question 1: What is the most likely diagnosis?

Bullous impetigo

Ulcerated basal cell carcinoma

Dermatitis artefacta

Pemphigus vulgaris

Brunsting-Perry cicatricial pemphigoid

Answers:

Bullous impetigo – Incorrect. Bullous impetigo would show a subcorneal neutrophil-rich bulla with associated Gram-positive cocci.

Ulcerative basal cell carcinoma – Incorrect. Basal cell carcinoma would show a basaloid neoplasm extending off of the epidermis, with peripheral palisading of nuclei and clefting artifact surrounding tumor nodules.

Dermatitis artefacta – Incorrect. Dermatitis artefacta typically presents with irregularly shaped erosions/ulcers with jagged borders. While the patient's clinical presentation revealed an asymmetric ulcer, dermatitis artefacta should test negative on direct immunofluorescence.

Pemphigus vulgaris – Incorrect. Pemphigus vulgaris would demonstrate a suprabasilar intraepidermal acantholytic bulla with potential follicular involvement. Additionally, it is not typically a scarring process on biopsy.

Brunsting-Perry cicatricial pemphigoid – Correct. This patient's presentation of a rapidly progressing ulcer on the head with loss of hair growth in addition to a subepidermal bulla with positive direct immunofluorescence findings suggests localized cicatricial pemphigoid, with the Brunsting-Perry variant being the most likely diagnosis.

Question 2: What separates this entity from other variants of cicatricial pemphigoid?

Target antigen

Lack of significant mucous membrane involvement

Lack of response to immunosuppressive therapy

Most cases clinically display one large focal area of cutaneous involvement

It has an acute presentation with spontaneous resolution

Answers:

Target antigen – Incorrect. Autoantibodies to BP180, type VII collagen, and laminin-332 have been detected. However, the exact antibody for Brunsting-Perry cicatricial pemphigoid is unknown.

Lack of mucous membrane involvement – Correct. Brunsting-Perry cicatricial pemphigoid typically does not affect the mucous membrane, though cases have been reported with mild mucous membrane involvement.

Lack of response to immunosuppressive therapy – Incorrect. Brunsting-Perry cicatricial pemphigoid can be treated with prednisone and nonsteroidal immunosuppressants such as azathioprine or mycophenolate.

Most cases clinically display one large focal area of cutaneous involvement – Incorrect. Most cases of Brunsting-Perry cicatricial pemphigoid present with multiple small areas occurring on the head and neck.

It has an acute presentation with spontaneous resolution – Incorrect. Difficulty diagnosing Brunsting-Perry cicatricial pemphigoid often leads to a protracted clinical course and requires long-term treatment.

Question 3: What is observed histologically and on direct immunofluorescence with this entity?

Subepidermal bulla with lymphocytes and eosinophils and focal dermal scar; linear basement membrane zone staining with anti-IgG and C3 antibodies

Subepidermal neutrophil-rich bulla; granular deposition of IgA in dermal papillae

Suprabasilar acantholytic bulla with possible follicular involvement; intercellular IgG or C3 within the epidermis

Subepidermal neutrophil-rich bulla; linear IgA staining along the basement membrane of the epidermis

Subepidermal bulla with mixed dermal infiltrate containing lymphocytes, eosinophils, and neutrophils; linear deposition of IgA or IgG along the conjunctival epithelium basement membrane

Answers:

Subepidermal bulla with lymphocytes and eosinophils and focal dermal scar; linear basement membrane zone staining with anti-IgG and C3 antibodies – Correct. This histologic and direct immunofluorescent pattern describes Brunsting-Perry cicatricial pemphigoid.

Subepidermal neutrophil-rich bulla; granular deposition of IgA in dermal papillae – Incorrect. This histologic and direct immunofluorescent pattern describes dermatitis herpetiformis.

Suprabasilar acantholytic bulla with possible follicular involvement; intercellular IgG or C3 within the epidermis – Incorrect. This histologic and direct immunofluorescent pattern describes pemphigus vulgaris.

Subepidermal neutrophil-rich bulla; linear IgA staining along the basement membrane of the epidermis – Incorrect. This histologic and direct immunofluorescent pattern describes Linear IgA bullous disease.

Subepidermal bulla with mixed dermal infiltrate containing lymphocytes, eosinophils, and neutrophils; linear deposition of IgA or IgG along the conjunctival epithelium basement membrane – Incorrect. This histologic and direct immunofluorescent pattern describes mucous membrane cicatricial pemphigoid.

Conflicts of interest

None declared.