pemphigus

YSMU Department of Dermatology, STI and CosmetologyPemphigusLecturer: Edgar Manukyan MD, MS

Bullous DiseasesHereditary epidermolysis bullosa

Familial benign pemphigus or Hailey-Haileys disease

Pemphigus Vulgaris

Bullous Pemphigoid

Cicatrical Pemphigoid

Dermatitis Herpetiformis

Linear IgA Dermatosis

Hereditary Epidermolysis BullosaJunctional epidermolysis bullosa (Herlitz variant) There are large eroded, oozing and bleeding areas that occurred intrapartum. When this newborn is lifted up, dislodgment ofepidermis as well as erosions occur due to manual handling.

Pemphigus VulgarisPemphigus vulgaris is a serious, acute or chronic, bullous, autoimmune disease of skin and mucous membranes that is often fatal unless treated with imunosuppressive agents. It is the prototype of the pemphigus family, a group of autoimmune acantholytic blistering diseases.

Age of Onset 40 to 60 years.

Sex Equal incidence in males and females.

Etiology Autoimmune disorder.

Pemphigus VulgarisLoss of normal cell-to-cell adhesion

IgG against pemphigus antigen (desmoglein 3, cadherin family)

Stratum spinosum

Acantholysis, rounded-up (acantholytic) keratinocytes.

Starts from oral mucosa

Pemphigus VulgarisPemphigus vulgaris flaccid bullae, erosions. The patient previously had erosive and very painful stomatitisfor over six months and was treated unsuccessfully with topical remedies by his dentist.

Pemphigus vulgaris Widespread, confluent erosions on the back of a patient who has a generalized eruption including the scalp and the mucous membranes.Pemphigus Vulgaris

Pemphigus Vulgaris

Pemphigus VulgarisSites of Predilection Scalp, face, chest, axillae, groin, umbilicus. In bed-ridden patients, there is extensive involvement of back.

Mucous Membranes Bullae rarely seen, erosions of mouth and nose, pharynx and larynx, vagina.

Diagnosis: biopsy, direct immunofluorescence, indirect immunofluorescence

Pemphigus Vulgaris

Pemphigus Vulgaris