Incontinentia pigmeti
X-linked dominant disorder. Partial deletion of the gene IKBKG. Blaschko lines (brown whorls), which correspond to embryonic mesodermal and epidermal cell migration pathways. Neonates present in the vesicular stage, which then can progress to subsequent verrucous, hyperpigmented, and hypopigmented stages. Skin biopsy specimens of vesicles will show spongiosis with eosinophils and dyskeratotic keratinocytes
Hair collar sign
ring of darker terminal hairs surrounding the defect, signifies higher risk of underplaying neurodevelopmental abnormality, such as a defect of nueral tube closure
Cafe au lait spots
Associated with NF1. 6 or more usually meet criteria
Psoriasis
neonatal - well-defined red plagues with adherent silver-white scale, typically located on arms, legs, umbilicus, gluteal cleft and scalp
Large scalp lesions of aplasia cutis are at higher risk of?
Hemorrhage. An eschar can form over the dura at the peripheral borders of the lesion. As the eschar dries, it can pull away form the dura, thus leading to tears and significant hemorrhage.
Nevus of Ota
nevus fuscoceruleus opthalmomaxillaris - unilateral, patchy, blue to bluish-gray to brown patch of skin on the face
Propanolol
nonselective betablocker. lipophilic. metabolized by lier. Half life of 3 to 6 hours and demonstrates peak effect at 1 to 4 hours. The dose response is usually most pronounced after the first dose
Fetus papyraceus
bilateral large stellate defects of truncal skin and sometimes the extremiteis. most cases occur as the result of a monochorionic twin gestation during which 1 twin dies during the first trimester. In these cases, an ischemic or thrombotic event is thought to be the cause
Leopard Syndrome
familial lentiginosis syndrome. Acronym for lentigines, EKG conduction defects, ocular hyperteloriss, pulmonary stenosis, abnormal genitalia, retardation fo growth, and deafness
Relative contraindications to propanolol
asthma and cardiogenic shock
Nevus anemicus
pale macule or macules that coalesce into patches of varying size. may be an irregular border. caused by decreased blood flow through the capillaries in the dermal papillae because of increased hypersensitivity of the blood vessels to catecholamines
Period of most rapid growth for hemangiomas
For superficial hemangiomas the most rapid growth occurs between 5.5 and 7.5 weeks of age. Infantile hemangiomas typically reach ~80% of their final size by 3 to 5 months of age
markers of higher risk cutis aplasia
> 2.0 cm, hair collar, membranous, midline
