ANGIONEUROTIC EDEMA (QUINKE=S Dz)
ANGIOEDEMA = DEEP DERMIS (VS URTICARIA (WHEAL) IN SUPERFICIAL DERMIS)
BRADYKININ INDUCES, SO IT TAKES ON THE ORDER OF 12 HOURS TO PEAK. IF ABDOMINAL CAN CAUSE “10” PAIN. CAN LEAD TO A COMPARTMENT SYNDROME IN EXTREMITIES
NON PITTING, LOCALIZED SWELLING, USUALLY ASSYMTRIC, NON DEPENDENT AREAS, RELATIVELY RAPID ONSET WITH SLOW 10-12 HOUR PEAK (BRADYKININ MEDIATED), INVOLVES SKIN OR MUCOSA, ONSET USUALLY IN CHILDHOOD
HEREDITARY
TYPE I (85%) COMMON— DEFICIENT CI INH (C1 ESTERASE INHIBITOR)
TYPE II (15%) VARIABLE—-DEFECTIVE CI INH, DECREASED C4
AQUIRED
B-CELL MALIGNANCY
CT Dz/AUTOIMMUNE Dz (SLE)
ALLERGEN? DRUG INDUCED (ACE INHIBITORS!)—-LATE ONSET (UP TO 1 YEAR) ARE COMMON—-CAPTOPRIL (1/1000)—ASSOC. COUGH AND DYSGUESIA—MECHANISM NOT WORKED OUT–PROBABLY RETARDS THE BREAKDOWN OF BRADYKININ
ALSO HAVE LOW TITERS OF C2 AND C4
ATTACKS ARE SELDOM PRIOR TO PUBERTY
DO NOT PRESENT WITH URTICARIA
Dx:
Hx, PE C4, C2, CI INH LEVELS
C4 AS A SCREENING TOOL!
Rx:
EVERYONE WITH AN ATTACK NEEDS IMMEDIATE TREATMENT
DECADRON 10 MG IV Q 6 HOURS, EPI, AIRWAY CONTROL
5000 U HEPARIN S.Q.
HI AND H2 BLOCKERS
AMINOPHYLLINE
SOME NEED PROPHYLACTIC TREATMENT
ANDROGENS VS CI-INH (CINRYZE)
ANDROGENS (PO) WITH HIGH SIDE EFFECTS, BP, LFTS
CINRYZE (IV) EVERY FEW DAYS, PLASMA DERIVED (HEPATITIS VACCINES) AND VENOUS PRESERVATION
(ANDROGEN)STANAZOL 2 MG/KG/DAY—WEAN TO 0.5 MG/KG/DAY
(ANDROGEN)DANAZOL 200 MG/KG/DAY—WEAN TO 200 MG Q D
AMICAR (EPSILON AMINOCAPROIC ACID)—PRIMARILY IN CHILDREN
FFP
CIq ESTERASE INHIBITOR CONCENTRATE IN 5% D5W OVER 10-45 MINUTES—IF UNAVAILABE– GIVE FFP
FOR MILD CASES: MEDROL, ZYRTEC, AND ZANTAC
URTICARIA = SUPERFICIAL DERMIS
MORE LIKELY TO BE A ALLERGIC PHENOM (TYPES I – IV)
MELKERSON-ROSENTHAL SYNDROME (1931)
RECURRENT OROFACIAL EDEMA
RECURRENT FACIAL PARALYSIS(POSSIBLY PROGRESSIVE)
LINGUAL PLICATA(FISSURED TONGUE)–POSSIBLY PERMANENT ORAL CAVITY/LIP DEFORMITY(CHELITIS)
+/- MIGRAINE HA
TRIAD
: CHRONIC RECURRENT UNI OR B FACIAL PARALYSIS, FACIAL SWELLING(LIPS AND TONGUE), FISSURED TONGUE (LINGUAL PLICAE/SCROTAL TONGUE)
COMPLETE TRIAD IN ONLY 25%–USUALLY SEQUENTIAL TRIAD OF SIGNS
USUALLY STARTS IN CHILDHOOD OR 2nd DECADE
FEMALE>MALE
UNILATERAL ON IPSI SIDE OF SWELLING
PROBABLY A LOCALIZED VARIANT OF ANGIONEUROTIC (QUINKE=S) EDEMA VS A VARIANT OF SARCOIDOSIS (HIGH ACE LEVELS/GRANULOMATOUS CHANGES)
Dx
: LIP BIOPSY-GRANULOMATOUS CHANGES-NON-CASEATING
Rx
: STEROIDS
ACYLOVIR? THALIDOMIDE
PROPHYLACTIC DECOMPRESSION(FACIAL PARALYSIS TENDS TO WORSEN)
MAY BE A COUSIN TO (A LOCALIZED VARIANT OF) ANGIOEDEMA
AUTO D
PEAKS AGE 20-30
HIGH [ACE] LEVELS WITH ATTACKS