Special Skin Cases Flashcards
what disease presents with abnormal skin findings and neurologic anomalies?
neurofibromatosis type 1
how is neurofibromatosis type 1 inherited
AD
name the four skin findings in neurofibromatosis type 1?
neurofibromas
cafe au lait
axillary and inguinal freckling
plexiform neurofibroma
what is a plexiform neurofibroma?
a big neurofibroma that feels like a sack of worms
big skin patch that is brown
what are cafe au lait macules?
brown patches that have regular borders and unifrom pigmentation
what are the 2 eye findings in neurofibromatosis type 1?
lisch nodules and optic gliomas
how do you manage the neurofibromas in neurofibromatosis type 1?
should excise any rapidly changing ones
what disorder presents with cutaneous lesions and peripheral/CNS neoplasms?
tuberous sclerosis
how is tuberous sclerosis inherited
AD
what are two genes in tuberous sclerosis?
TSC1 and 2
name the four skin findings in tuberous sclerosis
angiofibromas
hypomelanotic macules
shagreen patch
periungal fibromas
what is a shagreen patch?
connective tissue nevi that is hyper or hypopigmented
how do shagreen patches feel?
leather like
what other organs have issues in tuberous sclerosis?
cardiac renal oral optic neurological
what is a neuro finding in tuberous sclerosis/
seizures
what is a oral finding in tuberous sclerosis?
gingival fibromas and dental enamel pits
spontaneous quickly growing painful ulcers on legs are likely to be what?
pyoderma gangrenosum
what does pyoderma gangrenosum start as and what does it lead to?
papulopustule leads to a big ulcer
where is pyoderma gangrenosum commonly found?
lower extremities
what is pyoderma gangrenosum associated with?
IBD
what can trigger pyoderma gangrenosum?
surgery and wound debridement
what is treatment for pyoderma gangrenosum?
wound management and systemic steroids
what is lichen sclerosus?
inflamm disease that leads to ivory white scar like atrophy
where does lichen sclerosus commonly occur?
in genitals
what are symptoms of anogenital lichen sclerosus?
pain and pruritis…also can be asymptomatic
is anogenital or extragenital lichen sclerosus more common?
anogenital by far
how does lichen sclerosus appear on skin?
white scarring and atrophy
what is treatment for lichen sclerosus?
high potency steroid like clobetasol for 12-24 weeks
what is Morgellons Disease?
delusions of parasitosis…
what is delusions of parasitosis?
people think they keep getting infected with worms and try to pull them out and treat themselves with pesticides
infantile hemangiomas often occur in what locations?
face or in dermatome pattern
what is treatment for infantile hemangiomas?
beta blockers
what is a port wine stain? where is it found?
red macules and patches on the face that are present at birth
how do port wine stains change over time?
start red and get a deeper purple hue and then progress to having nodules
what causes port wine stain?
congenital capillary malformation
what are port wine stains associated with clinically?
SturgeWeber syndrome
how to treat port wine stain?
laser therapy
how does kaposi sarcoma appear on skin?
dark violaceous plaques and nodules
what causes kaposi sarcoma?
neoplasm of endothelial cells linked to HHV8``
who gets kaposi?
HIV
what should HIV patient be on to prevent kaposi?
HAART
what is treatment for Kaposi? when?
chemotherapy when extensive disease
what is a disease that presents with recurring bouts of incision and drainage?
hidradenitis supporative
what is another name for hidradenitis supporative?
acne inversa
explain the pathology behind acne inversa?
inflamm disorder of hair follicle causes abscesses and nodules and sinuses
what are sinuses in acne inversa?
epitheliazed tunnels with multiple connections to skin surface
who gets more acne inversa?
females..smokers..obesity
when does acne inversa usually start?
20 and 30s
what does early disease with acne inversa look like?
mainly just recurrent nodules and abscessed with no scarring
what does severe disease with acne inversa look like?
lots of sinuses and scarring
what are treatment options for acne inversa?
long term abx
systemic anti inflamm drugs
name the systemic anti inflamm drug for acne inversa?
adalimumab
what two drugs sometimes help acne inversa for women?
OCPs and spiranolactone
what is a key difference in morgellons disease and delusions of parasitosis?
morgellons has fiber extruding from skin
