Dermatology Flashcards
What is aplasia cutis and what is it associated with?
congenital absence of skin
20% of those on the scalp have underlying skull abnormalities
if it occurs in multiple places on the scalp: trisomy 13
if it occurs as a midline defect, look for spinal dysraphia
midline scalp lesions with darker hair: cranial dysraphism
What is a complication of infantile hemangiomas?
Ulceration, particularly lip and perineum and if the lesion is large.
Name the locations where an infantile hemangioma causes complications and name the complications.
Periorbital lesions: 80% risk of periorbital complications, astigmatism, amblyopia, refractive errors
Beard lesions (mandible, chin, submental): subglottic hemangioma resulting in stridor, cough, difficulty swallowing
Ear: if obstructs external canal affects hearing
Nose and lip: tendency to ulcerate and affect cosmetic appearance of the child
Midline lumbosacral region: increased risk of spinal dysraphism, possible GU anomalies (evaluate with US / MRI)
Multiple cutaneous hemangiomas: >5, can have visceral hemangiomas, most are self limited but some result in high output cardiac failure, GI hemorrhage, thyroid abnormalities
what is PHACE syndrome
Posterior fossa abnormalities Hemangioma (large, facial involving V1) Arterial anomalies (intracerebral arterial anomalies) Cardiac defects (coarctation) Eye abnormalities
What is Nevus Simplex
a capillary malformation known as an angel kiss or stork bite. Facial lesions usually lighten as the child gets older
What syndrome is associated with port wine stain or nevus flammeus that involves the V1 distribution?
Sturge-weber syndrome
ipsilateral cerebral vascular malformation: seizures, intellectual disabilities, contralateral hemiplegia, ophthalmologic (glaucoma, choroidal vascular anomalies)
How should children with nevus flammeus (port wine stain) in the V1 distribution be screened?
ophthalmologic exam head imaging: leptomeningeal vascular malformaion calcifications of the leptomeninges and the underlying white matter cerebral atrophy enlarged choroid plexus
What is the concern if a child has nevus flammeus in the V2 distribution
risk for glaucoma (need ophthalmologic exams)
possible orthodontic challenges
What syndromes are associated with nevus flammeus in the lower extremities?
Klippel-Trenaunay syndrome
capillary-venous-lymphatic vascular malformation, with soft tissue or limb overgrowth
Parkes-Weber syndrome:
associated with more marked limb overgrowth in both length and girth
What is a nevus sebaceous and how should it be managed?
yellow to salmon colored plaque with a waxy texture
texture enlarges during puberty
due to risk of basal cell carcinoma, more advise removal prior to puberty
What is a nevus spilus?
well demarcated tan or light brown macule with multiple small dark macules and papules
like a chocolate chip cookie
benign
How are congenital melanocytic nevi classified?
based on size of lesions as an adult
small < 1.5 cm
medium 1.5 cm to 20 cm
large > 20 cm
What are some complications associated with congenital melanocytic nevi
with large nevi: 6-8% chance of melanoma, especially in first 5 years of life
large lesions over the scalp or midline spine can have leptomeningeal involvement = neurocutaneous melanosis
What is a nevus of ota? And what can be a complication?
African and Asian infants
unilateral irregularly speckled areas of bluish-gray discoloration on the face, periorbital area and sclera
do not fade with age
cutaneous and ocular melanoma can occur in these lesions
need skin and ophthalmic evals periodically
What is a nevus of Ito
Similar to nevus of ota but on shoulders, neck and upper extremity
