SCLERODERMA = PSS (PROGRESSIVE SYSTEMIC SCLEROSIS)
AN IDIOPATHIC SYSTEMIC AUTOIMMUNE Dz WITH A VARIABLE COARSE
FEMALE 3:1
ONSET 30-50 (USUALLY < 40)
WORSE Px IN BLACKS AND MALES
BILAT SYMMETRICAL STIFFNESS AND THICKNESS IN THE SKIN
BASICALLY PT DIES BACK FROM A PERIPHERAL VASCULAR INSUFFICIENCY (VASCULITIS)
ABNL RATE OF SYNTHESIS AND DEPOSITION OF NL COLLAGEN—PRIMARILY AFFECTS CT AND BV’S—LOSS OF 80% OF CAPILLARIES IN INVOLVED SKIN ANDORGANS
NAIL FOLD CAPILLARY CHANGES
RAYNAUD’S PHENOM
NEPHROCALCINOSIS
HTN—ARTERITIS
50% ABNL EKG’S
TELANGIECTASIA—RARE EPISTAXIS
-TIGHT FACIAL SKIN–TIGHT SKIN –THIN LIPS–INABILITY TO OPEN THE MOUTH IS THE INITIAL COMPLAINT IN 20%–VERTICLE PERIORAL FURROWS– DRY SKIN—RESORBTION OF MANDIBULAR ANGLES
TRISMUS—DYSPHAGIA
PROGRESSIVE DYSPHAGIA—WT LOSS (52%)—-LOSS OF CONTRACTION OF DISTAL 2/3 OF ESOPHAGUS(VS DERMATOMYOSITIS-POLYMOSITIS = UPPER 1/3)—DILATED AND PATULOUS LES—LEADS TO REFLUX AND SUBSEQUENT BREAK DOWN—–BLEEDING, STRICURE FORMATION (HOARSENESS AND PYROSIS)—INCREASED INCIDENCE OF BARRETT’S—-ADENOCA
LES INCOMPETENCE—AEROPHAGIA (BURPING) = “SCLERODERMA BARK”
OTHER CAUSES OF ESOPHAGEAL DYSMOTILITY = ETOH/DM–NEUROPATHY, SLE, RAYNAUD’S, CHAGAS DZ, PRESBYESOPHAGUS
LAB: HIGH ESR, MILD ANEMIA, ANA, HYPERGAMMAGLOBULINEMIA, POS RF, EKG CHANGES, POS, ANTI-SCL-70 DNA TOPOISOMERASE I, ANTI-CENTROMERE AB
VARIANTS:
CREST VARIANT
CALCINOSIS
RAYNAUD’S (TONGUE BLANCHING WITH DYSARTHRIA)
ESOPHAGEAL DYSMOTILITY
SCLERODACTALY
TELANGIECTASIA
THIBIERGE-WEISSENBACH SYNDROME
EXTENSIVE SUB-Q CALCINOSIS
MORPHEA OR LINEAR SCLERODERMA(LIMITED TO SKIN)
LOCALIZED/DISCRETE WELL DEMARKATED IVORY PLAQUES WITH VIOLACEOUS BORDERS ON THE SKIN
Rx: PRIMARILY SUPPORTIVE MANAGE HTN AGGRESSIVELY, OBSTAIN FROM TOBACCO, ELEVATE HOB(REFLUX PRECAUTIONS), ABX TO COMBAT BACTERIAL OVERGROWTH AND MALABSORBTION, D-PENICILLAMINE TO PREVENT COLLAGEN CROSS-LINKING, INTRA-ARTERIAL RESERINE TO COMBAT PERIPHERAL VASOCONSTRICION, AND OTHER SYMPTOMATIC TREATMENTS
STEROIDS ARE LARGELY UNEFFECTIVE
SCLEREDEMA ADULTORUM OF BUSCHKE—AN ACCUMULATION OF MUCOPOLYSACCHARIDES IN THE DERMIS (DIFFUSE DERMATOFIBROSIS)—-OFTEN SECONDARY TO DIABETES OR AT LEAST ASSOC WITH IT
USUALLY ON THE BACK, SHOULDERS AND NECK
DIFF DX AMYOIDOSIS, SCLEROMYXEDEMA, SCLERODERMA
BENIGN AND OFTEN SELF LIMITING—-SEND TO DERM
Bx: ACCUMULATION OF MUCOPOLYSACCARIDES IN THE DERMIS