FIGURE 96.9 Nevus simplex (salmon patch) (Reprimted with permission from Goodheart HP Goodheart’s Photoguide of Common Skin Disorders 2nd ed Philadelphia, PA: Lippincott Williams & Wilkins; 2003:1.) Incontinentia Pigmenti This is an X-linked dominant disorder with both skin and systemic lesions affecting the eyes, CNS, and skeletal system The disease presents at birth or shortly after with an inflammatory vesicular or bullous rash that develops in crops over the trunk and extremities The cutaneous lesions have four phases (inflammatory vesicles or bullae, verrucous lesions, whorled hyperpigmentation, and hypopigmented patches) that may overlap and occur in irregular sequence Suspected cases should be referred to a dermatologist because of the potential for systemic involvement Vascular Lesions Salmon patch (nevus simplex ) is the most common vascular lesion of infancy It is a pale pink macular lesion that is found most commonly on the nape of the neck, forehead, nasolabial region, or upper eyelids Most resolve by the second year of life Lesions on the neck may persist for life ( Fig 96.9 ) Port-wine stains (nevus flammeus) present at birth as pink to purple macular lesions than can vary tremendously in size ( Fig 96.10 ) These lesions not fade with time Klippel–Trénaunay–Weber syndrome should be considered when the port-wine stain involves a lower limb Consider Sturge–Weber syndrome when infants present with unilateral facial port-wine stains in a trigeminal nerve distribution Because Sturge–Weber syndrome can involve seizures, intracranial calcifications, and hemiparesis, infants with unilateral facial port-wine stains in trigeminal nerve distribution need further assessment FIGURE 96.10 Nevus flammeus (port-wine stain) (Reprinted with permission from Nursing management of the newborn In: Ricci S, ed Essentials of Maternity, Newborn, and Women’s Health Nursing 4th ed Wolters Kluwer: Philadelphia, PA; 2017:585–643.) Hemangiomas Capillary hemangiomas (strawberry hemangioma) may be present at birth but usually develop during the first few weeks of life Lesions may present anywhere on the body, starting as small, well-demarcated telangiectasis or macules that develop into raised scarlet or purple tumors with distinct borders Most capillary hemangiomas grow rapidly the first months of life, enter into a static period and then recede, usually by years of age ( Fig 96.11 ) Cavernous hemangiomas are deep-seated capillary hemangiomas that usually present at birth as diffuse swelling with no color change or a bluish hue Most spontaneously involute over months to years Management Hemangiomas that require intervention in the neonatal period are those that may compromise vital structures, such as the eyes, nares, or auditory canal, or lesions that by their location are susceptible to trauma, ulceration, and secondary infection Kasabach–Merritt syndrome, which presents as large, rapidly enlarging hemangiomas associated with thrombocytopenia and consumption coagulopathy, should be referred to appropriate specialists for management Monitor serial CBC and if evidence of significant bleeding/coagulopathy, may require transfusions of red cells, platelets, or coagulation factors Pigment Changes Mongolian spots are poorly circumscribed lesions of bluish-gray maculae generally located over the lumbosacral region, buttocks, and lower limb Mongolian spots are more commonly noted in infants of African-American, Native American, Hispanic, and East Asian descent Lesions usually fade during the first year of life and not require intervention ( Fig 96.12 ) Café-au-lait macules are round or oval, brown macular lesions varying in size from less than cm to greater than 20 cm Though small, infrequent café-au-lait macules are common in the general population, increases in number or size may be a sign of neurocutaneous disease, such as neurofibromatosis The clinician should carefully examine these infants for other cutaneous or systemic signs of disease ( Fig 96.13 ) FIGURE 96.11 Capillary hemangioma (strawberry hemangioma) (Courtesy of Dean John Bonsall, MD In: Chung EK, Boom JA, Datto GA, et al., eds Visual Diagnosis in Pediatrics Philadelphia, PA: Lippincott Williams & Wilkins; 2006 With permission.) ... (Courtesy of Dean John Bonsall, MD In: Chung EK, Boom JA, Datto GA, et al., eds Visual Diagnosis in Pediatrics Philadelphia, PA: Lippincott Williams & Wilkins; 2006 With permission.)