Number II
Pemphigus vulgaris

Abstract

Pemphigus is a group of potentially life-threatening autoimmune diseases characterized by cutaneous and/or mucosal blistering. Pemphigus vulgaris (PV), the most common variant, is characterized by circulating IgG antibodies directed against desmoglein 3 (Dsg3), with about half the patients also having Dsg1 autoantibodies. There is a fairly strong genetic background to pemphigus with linkage to HLA class II alleles and ethnic groups such as Ashkenazi Jews and those of Mediterranean and Indian origin, are especially liable. Oral lesions are initially vesiculobullous but readily rupture, new bullae developing as the older ones rupture and ulcerate. Biopsy of perilesional tissue, with histological and immunostaining examination are essential to the diagnosis. Serum autoantibodies to either Dsg1 or Dsg3 are best detected using both normal human skin and monkey oesophagus or by enzyme-linked immunosorbent assay. Before the introduction of corticosteroids, PV was typically fatal mainly from dehydration or secondary systemic infections. Current treatment is largely based on systemic immunosuppression using corticosteroids, with azathioprine or other adjuvants or alternatives but newer therapies with potentially fewer adverse effects, also appear promising.

Pemphigus Vulgaris with Tense Bullae

“…It is characterized by involvement of the oral mucosa, such as painful oral erosions that often precede painful, cutaneous flaccid vesicles and bullae. 2 Involvement of the oral mucosa is usually accompanied by severe pain that can lead to weight loss and malnutrition. Although it is a rare disease, with incidence rates between 0.1 and 0.5 per 100,000 people per year, PV has the potential for lifethreatening complications, and treatment is always indicated at the time of disease onset.…”

Juvenile Pemphigus Vulgaris

“…In children and adolescents, PV should be differentiated from erythema multiforme, acute herpetic gingivostomatitis, impetigo, linear IgA disease, epidermolysis bullosa, cicatricial pemphigoid, bullous pemphigus, and paraneoplastic pemphigus. 6 Histopathological and immunofluorescence examinations are very important in the diagnosis of pemphigus. Acantholysis and intraepidermal blister formation are characteristic findings on histopathological examination.…”

“…6 Since pemphigus is rare in pediatric population, evidence-based treatment guidelines have not been reported yet. Immunosuppression is the mainstay of therapy, and systemic corticosteroids are the treatment of choice (prednisolone 1-2 mg/kg/day) to control the disease during the acute phase.…”

Diagnosis and management of common non-viral oral ulcerations

“…This results in epithelial separation above the basal cell layer, causing bullae on the skin and mucosae. 10 The oral lesions precede cutaneous involvement in 75% of affected patients. Although lesions may be confined to the oral cavity, the skin lesions usually develop in the subsequent months.…”