Intensive inflammatory infiltration was present under the epithelium and around vessels composed of plasma cells, lymphocytes, and eosinophils. Direct immunofluorescence (DIF) study of peribullous skin demonstrated linear deposits of IgG, IgA as well as complement C3 fraction with a moderate intensity along the dermoepidermal junction. and milia. The paper also provides a review of previous reports on this item as well as a comprehensive differential diagnosis of facial blistering lesions. Keywords: mucous membrane pemphigoid, Brunsting-Perry pemphigoid, laser scanning confocal microscopy Introduction Mucous membrane pemphigoid (MMP) is an autoimmune inflammatory disorder characterized by blistering lesions of mucous membranes and skin.1 In classic MMP, oral mucosa and conjunctiva are typically affected and may cause significant dysfunction, including Tubastatin A HCl blindness.2,3 Skin involvement is observed in 25C35% of MMP patients with well-tense blisters localized mainly on the head, neck, and upper torso that heal, leaving atrophic scars and milia.1,4,5 They result from subepidermal split without acantholysis accompanied by mixed-cell infiltrates of lymphocytes, plasmacytes, histiocytes, neutrophils, and eosinophils.5 The exact etiopathogenesis is still poorly understood but associated with autoantibodies against basement membrane zone (BMZ) proteins that are mirrored by linear deposition of immunoglobulin G, A, or complement C3 along the basement membrane. The main target antigens are bullous pemphigoid antigen 180 kDa (BP180) and laminin 332 (laminin 5).4,6 The clinical presentation of MMP is very diverse and may cause diagnostic difficulties. In the literature, there are scarce reports of rare type Tubastatin A HCl of MMP confined to the skin, termed Brunsting-Perry type (MMP-BP). This type of pemphigoid has histopathologic and immunofluorescence microscopic features similar to those observed in MMP; however, mucous membranes are generally spared. It may also mimic other vesiculobullous diseases, such as bullous pemphigoid (BP), epidermolysis bullosa acquisita (EBA), dermatitis herpetiformis (DH), linear IgA bullous dermatosis (LABD) or bullous systemic lupus erythematosus (BSLE), as well as impetigo.7 Since MMP-BP is also heterogeneous in terms of immunological findings, it may be a real diagnostic challenge. Herein, we present the case of Brunsting-Perry MMP in a 36-year-old female with negative indirect immunofluorescence and diagnosis established using fluorescence overlay antigen mapping by laser scanning confocal microscopy (FOAM-LSCM). Additionally, we provide a review of previously reported data on MMP-BP in terms of clinical, immunological, and therapeutic aspects as well as a comprehensive differential diagnosis of facial blistering diseases. Case Presentation A 36-year-old woman with a 2-month history of pruritic, vesiculobullous eruption located on her face and neck has Tubastatin A HCl been admitted to the Department of Dermatology in November 2020 for diagnosing. In outpatient settings, the patient was treated with topical steroids without clinical effect. Initial physical examination revealed numerous, well-tense blisters and erosions arranged in a herpetiform pattern at the margins of well-outlined Foxd1 oval or polycyclic erythematous and slightly atrophic plaques located in the central zone of the face, left cheek, and mandibular area. Blisters varied in size with the largest less than 1 cm in diameter (Figure 1A). Additionally, a few similar lesions were scattered over the frontal portion of the patients neck and upper back (Figure 1B). The blistering plaques enlarged slowly peripherally and healed with slightly atrophic scars and milia (Figure 1C). The patient complained about pruritus and burning of the lesional skin. Other areas of the skin were not affected, and no mucosal involvement was found. Open in a separate window Figure 1 (A) Before treatment: polycyclic erythematous and slightly atrophic plaques located in the central zone of the face; (B) well-tense blisters and erosions arranged in a herpetiform pattern on the frontal portion of patients neck and upper back; (C) One month after 1 pulse of intravenous methyloprednizolon: slightly atrophic scars, hypopigmentation and sparse milia; (D) 6 months after dapsone and prednison treatment: a few milia and slightly anthropic scars, no new blisters. The patient was in good general health without any chronic disorder, pharmacological therapy, or supplements intake. Her family history was negative for either vesiculobullous or other autoimmune diseases, and the condition was not affected by sunlight exposure. No other evident triggering factor was found. Reports of routine laboratory tests were of Tubastatin A HCl normal values. In the blood, eosinophils constituted 5.4% of total white blood cells. Antinuclear antibodies (ANA), extractable nuclear antigens (ENA), double-stranded DNA (dsDNA), and anti-transglutaminase-1 (TG1) antibodies were all Tubastatin A HCl negative. Neither bacterial nor fungal growth was found in the lesional smear. UVA testing was negative, but MED-UVB was 0.03 J/cm2. The Tzanck test showed a trace of protein masses, neutrophils, and eosinophils but no acantholytic cells. Microscopic examination of a lesional skin biopsy showed a subepidermal blister filled with proteinaceous fluid and neutrophils without acantholysis. Intensive.