Pemphigus

Klasifikasi
• pemphigus vulgaris
• pemphigus foliaceus
• paraneoplastic pemphigus
 Sex ratio
• Larger epidemiologic studies (i.e., those
identifying greater than 100 cases) have
shown that the sex ratio of pemphigus in
women versus men ranges from 1.33 or 2.25
to 1
 Age of onset
• the mean age of onset is approximately 40
years, rarely occurs in children.
• Autoantigens are desmogleins,
transmembrane desmosomal adhesion
molecules (cell-to-cell adhesion structures)
• antidesmoglein 1 and 3 antibodies in
pemphigus patients directly cause blisters and
hence are the etiologic agents of disease.
 Pemphigus vulgaris
• Erosions on mucous membranes and skin
• flaccid blisters on skin
• histology: suprabasal acantholysis.
• the blister occurs in the deeper part of the
epidermis, just above the basal layer
• patients with PV may present with more
localized disease
• one form of which is called pemphigus
vegetans of Hallopeau.
• The PV antigen is desmoglein 3
• All patients with PV have antidesmoglein 3
antibodies, and some of these patients also
have antidesmoglein 1 antibodies
• Patients with mucosal-dominant PV tend to
have only antidesmoglein 3 antibodies, whereas
those with mucocutaneous disease usually have
both antidesmoglein 3 and antidesmoglein 1
antibodies
 Pemphigus foliaceus
• crusted, scaly skin lesions
• histology: subcorneal acantholysis
• blister is in the granular layer.
• patients with PF may present with more
localized disease, represented by pemphigus
erythematosus
• The PF antigen is desmoglein 1, PF patients
typically have antibodies against only
desmoglein 1.