Skin (Feinberg)

Key Concepts:

Terms in this set (73)

Cardiopulm + vascular dz
sarcoidsois
granulomatous vasculitis
sarcoidosis
inflamm dz affects multiple organs - esp lungs + lymph glands, granulomas!!

- lupus pernio = noncaseating granulomas --> translucent purple plaques - ear, cheek, nose, CHRONIC sarcoidosis
- erythema nodosum = on ANT SHINS, ACUTE + BENIGN sarcoidosis
granulomatous vasculitis - granulomatosis w/ polyangiitis (Wegener)
"C dz!!!! = upper resp tract, lower resp tract, renal"

triad:
- focal necrotizing vasculitis
- necrotizing granulomas in LUNG/UPPER AIRWAY
- necrotizing GLOMERULONEPHRITIS

palpable purpura
- saddle nose
granulomatous vasculitis - lymphomatoid granulomatosis
EBV --> B cell lymphoprolif dz --> erythematous papules/nodules (vs granulomatosis w/ polyangiitis - NO UPPER RESP INVOLVEMENT!)
granulomatous vasculitis - Churg-Strauss syndrome (EOSOINPHILIC granulomatosis w/ polyangiitis)
~Wegner (granulomatous, necrotizing vasculitis) -- but w/ EOSINOPHILS + ASTHMA

biopsy = leukocytoclastic vasculitis
hyperlipoproteinemia
xanthomas (nodules) - eyelids, planar, tuberous (=attach underlying tendons), tendinous (=fixed to underlying tendons), eruptive (=sudden elevated TG)
KAWASAKI DZ
medium vessel vasculitis that causes flu-like symptoms and can cause coronary artery anuerysms

Asian kid <4yo
mucocutaneous lymph node syndrome:
"CRASH + burn"
Conjuntival injection
Rash (polymorphous --> desquamating)
Adenopathy (cervial)
Strawberry tongue
Hand-foot changes
FEVER

complications:
--> coronary artery aneurysms --> thrombosis/rupture --> death
(pseudoxanthoma elasticum)
AR disorder of elastic tissue - mutation in ABCC6 transporter protein

yellow xanthoma macules, extra skin folds
RHEUMATIC FEVER
erythema marginatum
subcutaneous nodules

Group A beta hemolytic Strep infection
J<3NES:
Joint (migratory polyarthritis)
<3 (carditis)
Nodules in skin (subcutaneous)
Erythema marginatum
Sydenham chorea
(yellow nail syndrome)
abnormal lymphatics
Endocrine dzs
DM
Graves dz
hypothyroidism
DM
acanthosis nigricans
internal malignancies (hyperinsulinemia, GI adenocarcinoma)
necrobiosis lipoidica (legs)
diabetic nephropathy/retinopathy
scleredema (=induration of skin on back/neck, improves w/ DM control)
Graves dz
hyperthyroidism
exophthalmos
PRETIBIAL MYXEDMEA (often develop after tx)
hypothyroidsim
MYXEDEMA (FACIAL/PERIORBITAL)
LOSE LATERAL 1/3 OF EYEBROW (specific alopecia)
yellow skin (dec beta carotene metabolism)
GI dzs
carcinoid syndrome
IBD
carcinoid syndrome
malig prolif neuroendocrine cells --> carcinoid tumors (in small bowel)

CUTANEOUS FLUSHING!!!
IBD (inflamm bowel dz)
Crohn dz = noncaseating granulomas [**fat GRANny + old CRONE skip down cobblestone road away from wreck]

UC = look like cancer sore...

BOTH: PYODERMA GRANGRENOSUM = purple undermined borders, craterlike holes, PUSTULES, PURULENT DRAINAGE
(Cowden dz)
AD disorder, GI polyps, wartlike papules
dermatitis herpetiformis
celiac dz
Peutz-Jeghers Syndrome
pigmented macules on lips/oral mucosa
recessive dystrophic epidermolysis bullosa
congeital bullous dz --> recurrent blistering + scarring on hands/feet

scarring --> pseudosyndactyly --> mittenlike hands

epidermolysis bullosa simplex = more superficial bullae, not life threatening

junctional epidermolysis bullosa = widespread blistering skin + mucous membs, FATAL
hematologic dz
amyloidosis
mastocytosis
congenital erythropoietic porphyria
porphyria cutanea tarda
(amyloidosis)
rare + serious dz caused by accumulation of proteins as abnormal insoluble fibers (amyloids) - local or systemic

a/w multiple myeloma --> get amyloid fibrils (immunoglobulin light chain deposits) deposits in skin

shiny translucent papules on eyelids
mastocytosis
mast cell activation disorder --> get too many mast cells

infilatration of mast cells into skin/other organs

-urticaria pigmentosa = skin lesions, reddish-brown macules + papules (look ~ nevi)
- Darier sign = stroking of indiv lesions --> get uticarial wheals

Dx = mast cells on skin biopsy
porphyrias
grp of dzs caused by build up of porphyrins --> affect skin/CNS

rare autosomal recessive disorder, mutation in gene for uroporphyrinogen III synthase (defective Hb synth)
congenital erythropoietic porphyria
pophyria charac by

SEVERE PHOTOSENSITIVITY
sun exposure --> vesicles + bullae develop

erythrodontia = red-stained teeth
porphyria cutanea tarda
porphyria charac by

photosensitivity vesicle formation + hypertrichosis (excess hair growth)

a/w HEP C
AIDS
human immunodeficiency virus (HIV) infection causing immune sys interference

a/w opportunistic infections:
viral
fungal
bacterial
scabies

Karposi sarcoma
oral hairy leukoplakia
AIDS - viral: Herpes simplex
virus infections become chronic erosive --> large nonhealing ulcers
AIDS - viral: Herpes zoster
vesicles in a dermatomal distribution
AIDS - fungal/bacterial
bacillary angiomatosis = many benign tumors of vascular walls caused by Bartonella henselae

purple papules + nodules
(mistaken for Kaposi sarcoma = lymphatic infiltrate vs bacillary angiomatosis = neutrophils)

tx = oral abx
AIDS - Kaposi sarcoma
tumor cause by infection by HHV8

dysreg VEGF prolif --> angiogenesis --> purple lesions on nose, extremities, mucous membs

can occur in any body surf - esp mucous membs

most common on HARD PALATE
AIDS - oral hairy leukoplakia
hairy white lesion on side of tongue caused by EBV usu in immunocompromised pts, CAN'T BE SCRAPED OFF!! (vs. oral thrush/candidiasis)
immunodeficient states
corticosteroids = used long term --> cause vascular fragility --> steroid purpura

cyclosporine = a/w hypertrichosis (excess hair)

aphthous stomatitis = many immunosuppressive drugs --> suppress bone marrow
infectious dz
infective endocarditis
staph toxic shock syndrome
staph scalded skin syndrome
necrotizing fasciitis
meningococcemia
scarlet fever
vibrio inefection
Lyme dz
rock mountain spotted fever
INFECTIVE ENDOCARDITIS
inflamm of endocardial surf of heart caused by bacterial infection, septic emboli/vasculitis --> cause skin lesions

bacteria FROM JANE <3

Fever
Roth spots (retina white spots surrounded by hemorrhage)
Osler nodes (TENDER raised lesions on fingers/toepads)
Murmur

Janeway lesions (microembolus --> small PAINLESS erythematous lesions on palm/sole)
Anemia
Nail bed splinter hemorrhage
Emboli
NECROTIZING FASCIITIS
bacterial skin infection usu caused by anaerobic bacteria/S. pyogenes (flesh-eating dz/bacteria)

rare infection of deeper layers of dermis + subcutaneous tissue (hypodermis)

dx = CREPITUS (presence of locules of gas on CT scan - to confirm)

tx = surgical debridement
meningococccemia
infections caused by Neisseria meningitidis causing flu-like symptoms

if untx --> PETECHIAL RASH, DIC, EXTENSIVE HEMORRHAGE, HYPOTENSION, DEATH

LOS --> inflamm --> leaky cpaillaries + consum platelets --> PETECHIAL RASH

leaky capillaries --> hypovolemia/SHOCK --> peripheral vasoconstriction goes to max to try to maintain BP --> adrenals poorly perfused + infarct --> Waterhouse Friederichsen syndrome

dx = CSF - N. meningitidis
tx = abx
scarlet fever
condn caused by erythrogenic exotoxins of GAS (grp A beta hemolytic Strep pyogenes), result of pharyngitis/wound infection

generalized rash 1-2days after onset pharyngitis

strawberry tongue (vs. Kawasaki dz)
Pastia lines = linear petechial macules in body folds
VIBRIO INFECTION
infection by vibrio vulnificus from minor trauma swimming in lake or while cleaning seafood

in pts w/ hepatic cirrhosis

after eat raw oysters
LYME DZ
infectious dz caused by Borrelia burgdorferi --> cause erythema chronicum migrans (chronic migrating redness, bull's eye rash) w/ flu-like symptoms

spirochete
transmit by deer tick Ixodes scapularis
rock mountain spotted fever
infectious dz caused by Rickettsia rickettsii --> cause HA, fever, myalgias, RASH BEGIN AT EXTREMITIES + SPREAD CENTRALLY

dx = skin biopsy
tx = IV chloramphenicol/tetracycline
neuro dz
basal cell nevus syndrome
epidermal nevus syndrome
incontinentia pigment/hypomelanossi of Ito
neurofibramatosis
Sneddon syndrome
tuberous sclerosis
nevus
benign neoplasm of melanocyte
(basal cell nevus syndrome)
rare genetic condn, autosomal dom disorder, mutation inactivate PTCH gene --> basal cell carcinoma
(epidermal nevus syndrome)
extensive epidermal nevi w/ systemic manifestations

large pigmented linear/whirled streaks
(incontinentia pigmenti)
genetic disorder affects skin + nervous sys, mutation in NEMO gene

linear pattern vesiculobullous lesions

verrucous paplues --> pigmented whirls
(hypomelanosis of Ito)
whirls of hypopigmentation --> pigmentary mosaicism

a/w neuro symptomss
NEUROFIBROMATOSIS
neurocutaneous disorder chrac by involvement of structs derived from neural crest cells

mutation NF1 tumor suppressor gene on chrm 17 ("17 looks like an n")

cafe au lait macules = flat evenly pigmented patches up to several cm diam

Lisch nodules = pigmented iris harmatomas

cutaneous neurofibromas

plexiform neuromas = larger deeper tumors a/w hypertrophy of bony + soft tissues
neurofibromatosis - variants
segmental neurofibromatosis = SEGMENTAL DISTRIB of cafe au lait spots + cutaneous neurofibromas

NF2 = bilat vestibular schwannomas, schwannomas, meningiomas, NO Lisch nodules, FEVER cafe au lait macules
SNEDDON SYNDROME
dz of skin + nervous sys, caused by occlusion of small/med arteries

livedo reticularis = lace-like purple discoloration of skin

strokes
tuberous sclerosis
neurocutaneous disorder a/w mutations of 2 tumor suppressor genes:

TSC1 gene = codes for hamartin - regulates cell cycled + neuronal synapse formation
TSC2 gene = ocdes for tuberin - GTPase activating activity, regulate protein transl + cell growth

adenoma sebaceum = skin colored papules on face
ash-leaf spots
confetti macules = small hypopigmented lesions
perifungual, subungual fibromas = skin colored nodules on finger/toenails
shagreen patch = skin colored plaque made of dermal CT

HAMARTOMASSSS
Harmatomas in CNS + skin
Angiofibromas
Mitral regurg
Ash-leaf spots
cardiac Rhabdomyoma
(Tuberous sclerosis)
autosomal dOminant inheritance
Mental retardation (intellectual disability)
renal Angiomyolipoma
Seizures
Shagreen patches
Subependymal giant cell astrycytoma
Subungual/periungual fibromas
renal dzs
Fabry dz
polyarteritis nodosa
perforating disorders
claciphylaxis
(Fabry dz)
rare genetic X-linked recessive lysosomal storage dz

deficiency in lysosomal enxyme alpha-galactosidase A --> accumulate ceramide trihexoside --> deposition of glycosphingolipids in body tissues

severe pain in extremities, burning of palms + soles

angiokeratomas (skin lesions) = pinpt red/purple papules (look like cherry hemangiomas)
polyarteritis nodosa
med vessel vasculitis, usu involves renal + visceral vessels, not pulm arteries, inflamm of MUSCULAR arteries

PAINFUL NODULES on LOWER EXTREMITIES
(perforating disorders)
extrusion of dermal material thru epidermis , a/w renal failure/DM

hyperkeratotic papules w/ central white craters
(calciphylaxis)
renal failure --> vascular calcification --> localized areas of skin become necrotic

painful purpuric pathes (~ livedo reticularis) ---> become indurated ulcerating plaques --> necrotic
rheumatologic dz
dermatomyositis
scleroderma/scleroderma-like dz
SLE
DERMATOMYSOSITIS
inflamm disorder of skin + skeletal muscle, progressive bilateral symmetric proximal muscle + MALAR RASH (vs SLE)

cutaneous manifestations:
- Gottron papules = erythematous scaling macules + papules on knuckles
- heliotrope erythema = periorbital erythema + edema

juveline variant = calcification of skin/muscle

vasculitic form (in KIDS) = cutaneous infarcts + ulceration + GI vasculitis w/ abd pain, bleeding, perforation
scleroderma (systemic sclerosis)
autoimmune disorder charac by SCLEROSIS OF SKIN + visceral organs

sclerosis (harden) of skin --> puffy taut skin, fingertip pitting

traid:
autoimmunity
noninflamm vasculopathy
collagen deposition w/ fibrosis

scleroderma-like dzs -
COMMON FEATURE = INDURATION OF SKIN

DIFFUSE type = widespread skin involvement + early visceral involvement

LIMITED scleroderma = limited skin involvement (hands + face) + late visceral involvement
- CREST syndrome =
Calcinosis/anti-Centromere antibodies
Raynaud phenomenon
Esophageal dysmotility
Sclerodactyly = localized thickening + tighness of digit skin, atrophy of underlying tissue
Telangiectasis of skin = dilation of capillaries --> spidery

+Raynaud phenomenon = dec blood flow to skin due to arterioloar (small vessel)vasospasm in response to cold/stress
MORPHEA
LIMITED/ localized scleroderma

sharply demarcated patches indurated skin

vs. systemic scleroderma
(graft vs host dz)
organ transplant --> sclerodermatous skin changes (teangiectasia)
(eosinophilic fasciitis)
inflamm of fascia limited to arms + legs

scleroderma-like hardening of skin, pucker skin, NO Raynaud phenomenon
SLE
anticardiolipin antibody syndrome
livedo vasculitis
neonatal lupus
SLE
chronic systemic autoimmune dz

RASH OR PAIN:
Rash (malar, discoid)
Arthritis
Soft tissue/serositis
Hematologic disorder
Oral/nasopharyngeal ulcers
Renal dz, Raynaud pheonomenon
Photensensitivty, Positive VDLR/RPR
Antinuclear antibodies (ANA)
Immunosuppressants
Neuro disorders

cutaneous manifestations:
discouid lupus = round scarred skin lesions w/ central hypopig + rim hyperpig + malar erythema
anticardiolipin antibody syndrome
autoimmune hypercoagulable state caused by antiphospholipid antibodies
--> thrombosis

can occur in SLE pts who suffer repeated throboses

livedo reticularis

false + serology for syphilis
(livedo vasculitis)
painful recuurent purple ulcers w/ reticular pattern on lower extremities
(neonatal lupus)
SLE symptoms in infant born from mom w/ SLE

discoid lupus erythematosus
heart block

due to transplacental passage of anti-Ro
(vit A deficiency)
blindness
"toad skin" = thick dry
vit B3 (niacin) deficiency = PELLAGRA
role in cell metabolism (ox-red rxns)

pellagra = disorder from chronic lack niacin
-4 D's = diarrhea, dermatitis, dementia, death

cutaneous sympt:
skin lesions in symmetric distribution at pressure pts + sun-exposed area

glove-like/boot-shaped

casal necklace = erythematous rash in broad collar + butterfly face distrib
VIT B6 deficiency**
ANGULAR CHEILITIS = lip scaling/cracks

seborrheic dermatitis

important cofactor in many enzymatic reactions of amino acid, lipid and glucose metabolism. important role in hemoglobin synthesis and function.
(vit B12)
role in RBC formation, metabolism (DNA synth, FA, AA), nervous sys funct

pernicious anemia --> get megaloblastic anemia

cutaneous sympt:
pallor + brown-gray fingernail
toenail discoloration
(vit C deficiency = scurvy)
cofactor in numerous collagen synthesis reactions and plays an essential role in collagen and amino acid formation.
antioxidant that supports immune function, essential to wound healing

general weakness, anemia, gum dz, skin hemorrhages

defect in CT develop --> skin probs

rough skin
follicular hyperkeratosis
coiled hair
perifollicular hemorrhages
easy bruising
petechiae
skin tears

poor wound healing, secondary infections
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