Mucous membrane pemphigoid (MMP), also called cicatricial pemphigoid, indicates to a bunch of scare chronic autoimmune blistering diseases that mainly affect the mucous membranes, including the conjunctiva, and sometimes the skin. Patients with cutaneous involvement present with tense blisters and erosions, often on the head and also the neck or at sites of trauma. Scarring of the mucous membranes is common, consequently the designation cicatricial, which may cause decreased vision, blindness, and supraglottic stenosis with hoarseness or airway barrier.
Panel test:
– Immunogenetics
o Associated with HLA-DR3 and DR4.
o C4 null alleles are increased
– Immunopathology
o Pathology shows the presence of eosinophils, which may be accompanied by a peripheral blood eosinophilia.
o Staining on immuno-EM is localized to the epithelial lamina lucida.
o IIF is positive in only 30% of patients.
o Therefore, biopsy with DIF is the diagnostic test of choice.
– Autoantibodies
o Anti BPAg2 Ag autoantibody
Comments: DIF shows linear deposits of C3 at the dermo-epidermal junction in almost all cases, with IgG in up to 50%.
References:
- Baum S, Sakka N, Artsi O, et al. Diagnosis and classification of autoimmune blistering diseases. Autoimmun Rev 2014; 13:482.
- Chan LS, Ahmed AR, Anhalt GJ, et al. The first international consensus on mucous membrane pemphigoid: definition, diagnostic criteria, pathogenic factors, medical treatment, and prognostic indicators. Arch Dermatol 2002; 138:370.
- Marazza G, Pham HC, Schärer L, et al. Incidence of bullous pemphigoid and pemphigus in Switzerland: a 2-year prospective study. Br J Dermatol 2009; 161:861.
- Schmidt E, della Torre R, Borradori L. Clinical features and practical diagnosis of bullous pemphigoid. Dermatol Clin 2011; 29:427.
