Pemphigus.pptx

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.