Pathology
Images
Atlas of dermatopathology
Josef Feit, Hana Jedličková, Günter Burg, Luděk Matyska, Spasoje Radovanovic et al.
×
+ Introduction
+ Literature
+ Support
+ Skin biopsy
+ Non-tumorous skin diseases
+ Skin tumors
+ Cysts of the skin and subcutis
+ Soft tissue lesions
+ Some lesions of the oral cavity
+ Dermatovenerology
+ Collection of histological slides by prof. Günter Burg
+ Collection of histological slides by prof. Werner Kempf
+ Collection of histological slides of dr. Ram Chandra Adhikari
Pemphigus vulgaris
Etiology, pathogenesis
  • autoimmune disease: circulating IgG antibodies directed against surface antigens on stratified squamous cells, which are associated with desmosomes
  • antibody binding causes desintegration of epidermis and blister formation
Clinical signs
  • adults, usually 40 – 60 years old
  • occurs first in the mouth, later affects the entire body
  • flaccid, easily broken blisters, without inflammation
  • erosions, crusts
  • sometimes pain, bleeding
  • heals without scars
  • life threatening disease
  • loss of fluids, proteins, secondary infections
  • test Tzanck is used in clinical diagnostics: cytologic identification of acantholytic keratinocytes within the smear of underside of the fresh bulla.
  • Nikolski sign: pressure of the finger causes formation of a new bulla; pressure of the finger in the vicinity of the bulla causes extension of the bulla and acantholysis (pseudonikolski sign)

Pemphigus vegetans is an longstanding vulgar pemphigus of the intertriginous areas with pustules and vegetations. pemphigus foliaceus (subcorneal acantholysis).

pemphigus erythematosus Senear-Usher, (similar to p. foliaceus, dermo-epidermal deposits similar to those in lupus erythematodes may be present), pareneoplastic and drug induced pemphigus are separate diseases, characterized by acantholysis as well.

Pemphigus herpetiformis is a rare variant of vulgar pemphigus, clinically resembling dermatitis herpetiformis (vesicles, papules, strong pruritus) and histologically is characterized by acantholysis and positive immunofluorescence finding. Inflammatory infiltrate is of variable density, with admixture of eosinophils and/or neutrophils. May affect oral mucosa as well.

Pemphigus vulgaris (864)

Pemphigus vulgaris (863)

Pemphigus vulgaris (1978)

Pemphigus vulgaris, autopsy (1978)

Histology

Intraepidermal suprabasal blisters; separated acantholytic cells; “chickenwire” immunofluorescence in stratum spinosum (very important diagnostic sign); eosinophils.

Pemphigus vulgaris (1808)

Pemphigus vulgaris (1809)

Pictures

Another case: Pemphigus vulgaris, HE 20x (71) Pemphigus vulgaris, HE 40x (72)

Another case: Pemphigus vulgaris, HE 10x (73) Pemphigus vulgaris, HE 20x (74) Pemphigus vulgaris, HE 40x (75) Pemphigus vulgaris, roof of the bulla, HE 40x (76) Pemphigus vulgaris, HE 100x (77)

The acantholysis is sometimes inconspicuous: Pemphigus vulgaris, old bulla, HE 20x (84)

Another case: Pemphigus vulgaris, HE 20x (78)

Another case: Pemphigus vulgaris, HE 40x (6025)

Another case: Pemphigus vulgaris, HE 60x (6414)

Incipient vulgar pemphigus with tiny acantholytic vesicle: Pemphigus vulgaris, incipient, HE 40x (79) Pemphigus vulgaris, incipient, HE 40x (80) Pemphigus vulgaris, HE 100x (81) Pemphigus vulgaris, HE 100x (82)

Network like fluorescence positivity of the stratum corneum: Pemphigus vulgaris, net-like intraepidermal anti-IgG positivity, anti-IgG, FITC 40x (83)

Acantholysis may not be present in healing, older blisters: Pemphigus vulgaris, old bulla, HE 20x (84)

Another case: Pemphigus vulgaris, HE 20x (1566)

Partially regenerated bulla, pemphigus vulgaris: Pemphigus vulgaris, HE 40x (5035)

Another case: Pemphigus vulgaris, HE 10x (1808) Pemphigus vulgaris, HE 40x (1569) Pemphigus vulgaris, HE 40x (1809)

Pemphigus vulgaris: Pemphigus vulgaris, HE 60x (13446)

Pemphigus vulgaris: Pemphigus vulgaris, HE 60x (11008)

Pemphigus vulgaris: Pemphigus vulgaris, HE 60x (12020)

Pemphigus vulgaris: Pemphigus vulgaris, HE 60x (12515)

Pemphigus vulgaris: Pemphigus vulgaris, HE 40x (13230)

Fissurated granuloma caused by the pressure of glasses in a patient with pemphigus: Granuloma fissuratum caused by glasses in patient with pemphigus, HE 20x (4254)

Pemphigus vulgaris, oral mucosa: Pemphigus vulgaris, mucous membrane, HE 40x (4295) Pemphigus vulgaris, mucous membrane, HE 40x (4296)

Another case of pemphigus vulgaris of oral mucosa: Pemphigus, oral cavity, HE 60x (6210)

Another case of pemphigus vulgaris of oral mucosa: Pemphigus vulgaris, oral mucosa, HE 60x (13231) Pemphigus vulgaris, oral mucosa, HE 60x (13232)

Pemphigus herpetiformis: Pemphigus herpetiformis, HE 20x (4924)

Pemphigus vulgaris, esophagus: Pemphigus vulgaris, esophagus, HE 60x (73953)