Pemphigoid scarring type brunsting-perry L12.1

Rare variant of the scarring pemphigoid with solitary infestation of head, forehead and neck. No infestation of mucous membranes.

Prevalence (Central Europe): 1.0 per 1 million inhabitants.

The formation of IgG autoantibodies against the "bullous pemphigoid antigen 2" (BP180; bullous pemphigoid 180 kDa protein) and more rarely also against laminin 5 is discussed. The formation of the antigen-antibody complexes leads to complement-mediated chemotactic attraction of inflammatory cells or to the release of proteases that destroy hemidesmosomal structures.

A genetic predisposition to the disease is associated with the HLA-DQB1*0301 haplotype in Caucasians.

Occurs in men of advanced age. Less common in women.

The clinical picture is often misinterpreted as pyoderma or as zoster as in the patient shown in the picture; it superficially impresses as a chronic and therapy-resistant scarring alopecia with extensive erosions and crusts. Bubbles are only rarely registered and then only in an acute phase of relapse. Infection of the mucous membranes is absent in this variant of the scarring pemphigoid. S.a. scarring pemphigoid.

Clinic, histology, immunofluorescence. Recently, antibodies against LAD-1 were detected.

Corresponding to the scarring pemphigoid.

• The successful therapeutic approaches with dupilumab are to be regarded as (still) experimental (Raef HS et al. 2021).

A 72-year-old man presented with scarring alopecia on the vertex of the scalp, multiple crusted plaques at the hairline, and a history of vesicular rash on the face. The scalp had crusted plaques with loss of follicular structures. An initial orienting scalp biopsy showed perifollicular neutrophils, lymphocytes, and plasma cells along with dermal fibrosis. Focal epidermal/dermal and follicular/adventitial skin clefts were seen, but were considered to be due to fibrosis, and the biopsy result was interpreted as neutrophil-mediated scarring alopecia.

Direct immunofluorescence (DIF) analysis revealed linear deposits of IgG and C3.

A repeat targeted scalp biopsy from a previously untreated skin area revealed more pronounced epidermal/dermal clefts, fibrosis, a mixed infiltrate with neutrophils, lymphocytes, histiocytes, and plasma cells, and pronounced follicular/adventitial dermal clefts with perifollicular neutrophils.

In conclusion, it has been shown that a neutrophil-rich form of cicatricial pemphigoid can be masked as neutrophil-mediated cicatricial alopecia.

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