PEMPHIGUS (THOST 1911)

VESICULAR Dz (<1CM)—–BULLOUS Dz (>1CM)

AN AUTOIMMUNE Dz AGAINST THE STRATUM SPINOSUM (INTRAEPIDERMAL)—PEMPHIGUS OR AGAINST THE B.M. (PEMPHIGOID)

PEMPHIGUS (MORE COMMON)

AGE 50—-MALE = FEMALE

HIGH INCIDENCE IN ASHKENAZIC JEWS (LIKE NIEMAN PICK AND TAY SACHS SPHINGOLIPIDOSIS)

INTRAEPITHELIAL—-2/3 OC +

ACANTHOLYSIS= SEPARATION/DISSOLUTION OF THE INTERCELLULAR BRIDGES IN “PRICKLE” CELL LAYER (STRATUM SPINOSUM) OF THE EPIDERMIS= +NICKOLSKY SIGN

NONSCARRING

IGG AND C3—-BASAL CELL LAYER REMAINS ATTACHED TO THE L.P.—-SUPRABASILAR CLEFTING—–“ROW OF TOMB STONES–+TZANC CELLS(LIKE HSV)

VEGETANS—-INDOLENT

VULGARIS

—RAPID ACUTE (33% FATAL IF ESOPHAGUS INVOLVED)

Rx:

STEROIDS, MTX, CYCLOPHOSPHAMIDE, AZATHIOPRIN

PEMPHIGOID (PEMPHIGUS LIKE)

AUTOIMMUNE Dz AGAINST THEB.M.—–LEADS TO SCARRING!

SUBEPITHELIAL CLEFTING

FEMALE > MALE

AGE 70

CICATRICIAL

AFFECTS THE MUCOSAL SURFACES—-O.C., OP, SUPRAGLOTTIC LARYNX, CONJUNCTIVA—SYNBLEPHARON

BULLOUS

MORE AFFECTS THE CUTANEOUS FLEXOR SURFACES

Rx:

DAPSONE, PENNICILLAMINE