presence of a central lumen, chronic discharge, or failure to respond to traditional treatment should prompt the clinician to consider these rare umbilical anomalies The distinction is of great clinical significance because these problems may be associated with other congenital malformations, and surgical excision of the entire remnant is necessary to prevent sequelae, such as infection Diagnosis is most commonly made by ultrasound FIGURE 120.18 Large pedunculated umbilical granuloma that responded to suture ligation and repeated silver nitrate applications Granuloma Annulare The lesions of granuloma annulare are composed of infiltrates of lymphocytes and altered collagen within the dermis They appear first as raised nodules that gradually expand centrifugally to form annular rings with central clearing ranging from to cm in diameter They have a firm, fibrous, sometimes-lumpy consistency on palpation Overlying skin can range from normal, to erythematous, violaceous, or slightly hyperpigmented ( Fig 120.19 ) Although most are asymptomatic, patients occasionally report mild pruritus and present with superficial excoriation caused by scratching The lack of an active microvesicular border, firm consistency on palpation, deeper dermal location, and failure to respond to antifungals help distinguish these lesions from tinea corporis Lesions are commonly found on the extensor surfaces of the lower portions of legs and the dorsum of the hands and feet and, less often, on the trunk or abdominal wall Although granuloma annulare may present at any age, more than 40% of cases appear after infancy and before 15 years of age Because most lesions resolve within to years, reassurance is usually all that is necessary Reoccurrence of the lesion, typically at the same site, is common While rare, a subcutaneous, generalized, and perforating form of granuloma annulare exists in children and should prompt referral to a dermatologist FIGURE 120.19 Granuloma annulare Juvenile Xanthogranuloma Juvenile xanthogranuloma (JXG) present as nodular or plaque-like lesions with a firm or rubbery consistency Initially reddish in color, they evolve to have a distinct yellow or orange hue ( Fig 120.20 ) While many are noted at birth, others appear within the first several months They range in diameter from 0.5 to cm Like hemangiomas, they tend to grow rapidly in infancy, then spontaneously regress in early childhood leaving a hyperpigmented scar Common sites include the scalp and face, proximal extremities, groin, and occasionally, the subungual area of a digit or a mucocutaneous junction Histologically, xanthogranulomas are composed of lipid-laden macrophages or histiocytes within a granulomatous matrix The inciting source is unknown The majority of children have solitary lesions In rare cases, multiple, giant, or disseminated lesions may occur Patients who have multiple lesions are more likely to have systemic or ocular involvement The most common noncutaneous lesions are ocular lesions, specifically lesions of the iris that have been associated with spontaneous anterior chamber hemorrhage and glaucoma Unlike cutaneous and systemic lesions, ocular lesions not spontaneously regress and may require intervention On occasion, ocular lesions have been misdiagnosed as retinoblastoma Other noncutaneous lesions in systemic JXG may involve the gastrointestinal tract (most commonly the liver), central nervous system, bone marrow, lymph nodes, heart, kidney, adrenal glands, and lungs Not all children with systemic JXG have concomitant cutaneous lesions Children who have both JXG and neurofibromatosis are at a much higher risk for unusual forms of leukemia and thus should be appropriately monitored Unlike children with disseminated xanthomas, there is no relationship between JXG and lipid abnormalities All children with suspected xanthogranuloma warrant consideration of an ophthalmologic evaluation, and careful evaluation for evidence of systemic involvement FIGURE 120.20 Yellow nodular lesion of juvenile xanthogranuloma (Reprinted with permission from Burkhart C, Morrell D, Goldsmith LA, et al VisualDx: Essential Pediatric Dermatology Philadelphia, PA: Lippincott Williams & Wilkins; 2009.) Neurofibroma A localized neurofibroma may present as a solitary lesion in an otherwise normal patient or as a feature of neurofibromatosis type I Cutaneous neurofibromas arise from nerve sheath cells located in the dermis They appear as pink or fleshcolored nodules that are soft and range in size from 0.5 to cm They are painless, though at times can be mildly pruritic Most not appear until adolescence Multiple neurofibromas should raise the suspicion for neurofibromatosis type I Lesions may be confused with angiolipomas and hemangiomas; however, a distinguishing feature is the tendency of neurofibromas to be especially soft centrally and invaginate with digital pressure, described as “button-holing.” Elective excision is indicated only if the lesion is compressing a nerve causing nerve root pain, because excision is often followed by recurrence of an even larger lesion Keloid/Hypertrophic Scar Exaggerated proliferation of fibrous connective tissue in the process of cutaneous wound healing results in formation of hypertrophic scars and keloids Wounds involving areas of skin that are thick or under high tension (shoulders, back, chest, or chin) are at greatest risk The ear lobe is another commonly affected site Individuals with dark skin are much more susceptible to abnormal scarring, which has its highest incidence in adolescence and early adulthood Hypertrophic scars remain confined to the area of original injury They are rarely painful and tend to undergo slow regression over to 12 months In contrast, keloids extend beyond the original wound margins and rarely regress spontaneously Initially, keloids may be tender or pruritic They have a rubbery consistency on palpation and a smooth pink surface ( Fig 120.21 ) Ear piercing, tattooing, and elective cosmetic procedures should be avoided in persons who tend to form keloids If a family chooses to pursue ear piercing, they should be counseled to complete the procedure in the first decade of life where the risk of keloid is lower, though not absent Keloids can be treated with topical or intralesional steroid injections, laser therapy, cryotherapy, and surgical excision with postoperative pressure dressing Recurrence is common and often difficult to treat Patients with severe keloids should be referred to a dermatologist or plastic surgeon for further treatment ... (Reprinted with permission from Burkhart C, Morrell D, Goldsmith LA, et al VisualDx: Essential Pediatric Dermatology Philadelphia, PA: Lippincott Williams & Wilkins; 2009.) Neurofibroma A localized