- Derieved : Greek word pemphix meaning bubble.
- Named by Wichman in 1791.
- Pemphigus refers to a group of autoimmune blistering diseases of the skin and mucous membranes characterized by formation of flaccid bullae.
- Mediated by circulating autoantibodies directed against keratinocyte cell surfaces.
- Binding of IgG autoantibodies to keratinocyte desmosomes and to desmosome-free areas of the keratinocyte cell membrane.
- Loss of cell-cell adhesion,acantholysis..
PV antigen: desmoglein 1 and desmoglein 3.
Risk factors: Genetic, Age factor, And Associated with other diseases like Thymoma and Myasthenia Gravis.
Sex: Male=Female .More in adolescent girl than boys.
Age:Adults (50-60 years)
General Health: Poor
Antibodies: IgGin Intracellular substance directed against Keratinocytes.
Site: Flexural surfaces, trunk, back, axilla, abdomen
The 3 primary subtypes:
- Pemphigus Vegetans
- Pemphigus Foilaceous
- Paraneoplastic Pemphigus.
Others: P. Erythematoides
- 1. Skin:
- Superficial and flaccid blisters, which usually arises on normal-appearing skin but may be found on erythematous skin.
- New blisters usually are flaccid or become flaccid quickly and are filled with clear fluid that arises on normal skin or on an erythematous base. The blisters are fragile; therefore, intact blisters may be few. The contents soon become turbid, or the blisters rupture producing painful erosions, which is the most common skin presentation. Erosions often are large.
- Erythematous halo may be present around the lesion.
- Mucous membranes: Commonly Involved. Oral lesions in 50-70% of patients, and almost all patients have mucosal lesions. Sometimes it is sole manifestation of this disease.Intact bullae are rare in the mouth. Ill-defined, irregularly shaped, gingival, buccal or palatine erosions, painful and slow to heal. The erosions extend peripherally with shedding of the epithelium. (Conjunctiva,esophagus,labia, vagina, cervix, penis, urethra, and anus.)
- Drug-induced PV: penicillamine, captopril, Rifampin and emotional stress have recently been reported as triggers for PV.
- Pregnancy Induced.
Mucous membranes are usually affected first in PV. Mucosal lesions may precede cutaneous lesions by months.
- Nikolsky sign: In patients with active blistering, firm sliding pressure with a finger separates normal-appearing epidermis, producing erosion. This sign is not specific for PV.
- Asboe-Hansen sign: (Bulla Spreading sign) Lateral pressure on the edge of a blister may spread the blister into clinically unaffected skin.
- Giemsa Staining of Skin Sample
- Direct and Indirect Immunofluorescence Assay.
Topical: Steroids and Antibiotics
Systemic: High Dose Prednisolone 80-120 mg Oral or Parenteral dose
If no new lesions appear, decrease dose by 5-10 mg/week
If new lesions appear, increase dose up to 200mg.
- Superadded Infections
By Dr. Sujit Shrestha