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