Skin Disease for Specialists Flashcards
T1 neurofibromatosis
AD disease, mutations in neurofibromin 1
cutaneous findings of neurofibromatosis
neurofibromas, cafe au lait, freckling, plexiform neurofibroma (like bag of worms)
systemic manifestations of NF1
ocular lisch nodules or gliomas
skeletal dysplasia
pheocrhomocytoma
learning disabilities
tx for NF
excision of changing or dx neurofibromas
optic and pheochromocytoma screening
patho of tuberous sclerosis
cutaneous lesions and peripheral CNS neoplasms
inheritance of tuberous sclerosis
AD w/ incomplete penetrance
mutation of TSC1 and TSC2
cutaneous clin pres of tuberous sclerosis
facial angiofibromas
hypomelanotic macules, shagreen patch (leather like, connective tissue nevi), periungual fibromas (toes)
systemic manifestations of tuberous sclerosis
oral, ocular, cardiac (myocardial rhabdomyoma), neurological (seizures), renal
pyoderma gangrenosum def
uncommon recurrent cutaneous ulcerative skin disease
related to IBD or heme malignancy, half idiopathic
dx of pyoderma gangrenosum
exclude infection w/ biopsy and culture
hx of undelrying disorder
rapid resposnse to systemic CS
pathergy
debridements w/ pyoderma
multi can be devastating- continual wound breakdown
tx for pyoderms
CS
immunomodulation
def lichen sclerosis
inflammatory disease leading to white scar atrophy, either anogenital or extragenital (less common)
pres of lichen sclerosis
pain, fissures, pruritis
can cause scarring, vaginal urethral stenosis
tx for lichen sclerosis
high potency topicals (clobetasol)
pres of delusions of parasitosis
usually 50s-60s
long term sx, multi physicians
hx of pesticide use, changing residence
all derm findings self induced
morgellons vs delusions of parasitosis
morgellons- “fibers” coming from skin
delusions- infested w/ parasites
helpful meds w/ delusions
antipsychotics- pimozide and risperidone have been helpful
pato of infantile hemangioma
benign growth of endothelial tissue, most common tumor in infants
risks for hemangioma
female, prematurity
deep vs superficial hemagioma
superficial bright red
deep is blue nodule
complications of hemangioma
ulceration, disfigurement
part of PHACES syndrome (cardiac, eye, sternal, arterial abnormalities)
risk w/ diffuse neonate hemangiomas
high output cardiac failure
tx for hemangiomas
observation if low risk
high risk or large: tx w/ propanalol, CS or vincristine second and third line
patho of port wine stain
congenital capillary malformaion, well demarcated red macules and patches at birth
clin assoc w/ PWS
surge weber syndrome- PWS in V1/V2 assoc w/ leptomeningeal angiomas (develop delay, seizures, ocular abnormalities)
midline lumbosacral assoc w/ spinal dysraphism
mistaken for PWS
nevus simplex- angel kiss or stork bite
more prominenet w/ crying, many fade on their own
PWS tx
latser therapy, better when treated young
define kaposi sarcoma
endothelial neoplasm linked to HHV8, most common in AIDS or transplant pts
pres of kaposi
dark, violaceous plaques or nodules
tx for kaposi
prevent w/ HAART
advanced tx w/ systemic chemo
patho of hidrenitis suppurativa
aka acne inversa
inflammatory disorder of hair follicle- causes abscesses nodules and sinuses (tunnels connected to skin surface)
not from infection or hygeine
tx for hidrenitis supp
long term abx
OCPs and spironolactone for women
systemic anti inflammatories- adalimumab
I and D acutely, sometimes wide excision
