Palmar Crease Pallor

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Skin color in newborns is related partially to the amount of fat present. Preterm infants generally appear redder because they have less subcutaneous fat than do term infants.

The newborn has vasomotor instability, and the color of the skin may vary greatly from moment to moment and from one area of the body to another. It is often noted that when the infant is lying on one side for a time, a sharp color demarcation appears: The lower half of the body becomes red, and the upper half is pale. Seen more commonly in preterm than in term infants, this has been termed the harlequin color change and is benign. The episodes may persist from 30 seconds to 30 minutes.

Inspect for cyanosis or acrocyanosis. Acrocyanosis is a benign condition in which the hands and feet are cyanotic and cool but the trunk is pink and warm. This condition is common among newborns. In central cyanosis, the tongue and gums are also blue. Persistent central cyanosis can be suggestive of respiratory abnormalities or the presence of cyanotic congenital heart disease.

Is plethora present? Plethora is a condition marked by an excess of blood and a marked redness of the complexion. Plethora in the newborn usually indicates high levels of hemoglobin.

Is pallor present? Pallor may be associated with anemia or, more commonly, with cold stress and peripheral vasoconstriction. Pallor may also reflect asphyxia, shock, sepsis, or edema. It should be recognized that the presence of pallor may mask cyanosis in a newborn with circulatory failure.

Are there any skin findings from birth trauma, manifested by petechiae, ecchymoses, or lacerations?

Physiologic jaundice is found in almost 50% of all term newborns by the third or fourth day after birth. This finding, which is even more prevalent among preterm infants, results from delay in the maturation of enzymatic processes in the liver. In most cases, jaundice is a self-limited condition, resolving after 96 hours of age. However, very high levels of bilirubin in the newborn's serum can lead to a condition known as kernicterus, in which permanent neurologic damage may occur. As such, it is important to monitor the serum bilirubin level and to treat elevated levels with phototherapy.

Icterus appearing before the third day may indicate a pathologic condition. Disorders that must be considered are hemolytic anemia, caused by blood group incompatibility or by

Figure 24-6 Mongolian spot.

bacterial or viral infections, and galactosemia, an inborn error of galactose metabolism. Visible jaundice in newborns does not appear until the serum bilirubin is approximately 5 mg/dL. When the level exceeds this threshold, the jaundice spreads in an orderly manner, from the top of the head to the soles of the feet. When the soles become yellow, the serum bilirubin level has usually reached 12 mg/dL. Many centers now use a transcutaneous bilirubinometer to facilitate recognizing potentially dangerous levels of jaundice.

Observe the pigmentation. Large, slate-blue, well-demarcated areas of pigmentation in the sacrogluteal area or elsewhere are called mongolian spots and are normal variants. Ninety percent of all mongolian spots are in the buttock area. These spots fade and disappear by 5 to 6 years of age in 98% of children who have them. Mongolian spots are present in more than 90% of African-American newborns and 70% of Asian-American newborns but in fewer than 10% of white newborns. Figure 24-6 shows a classic mongolian spot.

Telangiectases on the eyelids, glabella, or nape of the neck are common and frequently disappear during the first few years of life. They are often referred to as ''stork bites'' or ''angel kisses.''

Vascular nevi may be isolated defects or part of a syndrome and can be classified as malformations or hemangiomas. Hemangiomas are the most common tumors of infancy. They may be flat and are commonly caused by dilated capillaries, or they may be mass lesions and consist of large, blood-filled cavities. The port wine stain, also known as the nevus flammeus, consists of dilated capillaries and appears as a pink to purple macular lesion of variable size. It can be as large as half the body. It manifests at birth and represents a permanent defect. Figure 24-7 shows a port wine stain involving the ophthalmic division of the trigeminal nerve. Often, children with port wine stains in this area have associated capillary hemangiomas of the ipsilateral meninges and occipital portion of the cerebral cortex, a condition known as Sturge-Weber syndrome. Mental retardation, seizures, hemiparesis, contralateral hemianopsia, and glaucoma are often seen in the first few years of life. Figure 24-8 shows a child with Sturge-Weber syndrome (see also Fig. 10-24).

The strawberry nevus, or capillary hemangioma, is a bright red, protuberant lesion seen commonly on the face, scalp, back, or anogenital area. It may be present at birth, but it more commonly develops within the first 2 months of life. Girls are affected more often than boys. The lesion may expand rapidly, reach a stationary period, and then regress. More than 60% of capillary hemangiomas become involuted by the time the child is 5 years of age, and 95% become involuted by 9 years of age. Figure 24-9 shows a strawberry nevus.

The cavernous hemangioma is more deeply situated and is a cystic, often compressible lesion that is more diffuse and ill defined than the capillary hemangioma. The overlying skin may appear normal in color or may have a bluish hue. Like the capillary hemangioma, the cavernous hemangioma has a growth phase followed by a period of involution. If it is located near the trachea, life-threatening compression may result when the hemangioma enlarges. The child pictured in Figure 24-10 has a combination of a strawberry nevus and a cavernous hemangioma. The strawberry lesion overlies the cavernous hemangioma. The child pictured in Figure 24-11 has a mixed hemangioma;it has both superficial capillary and deep

Palmar Crease Pallor Test
Figure 24-7 P°rt wine stain. F¡gure 24-8 Sturge-Weber syndrome.

cavernous components. This lesion involuted by the child's third year of life. Although these lesions usually resolve by the seventh year of life, they may leave scarring, loose skin, and telangiectases. Also, as part of the Klippel-Trénaunay syndrome, they may be associated with overgrowth of a portion of the body (a complete side, an arm, a leg, or a smaller portion.)

Other birthmarks that may suggest the presence of an underlying genetic disorder include café an lait spots (see Fig. 8-44) and hypopigmented macules. One or two cafe au lait spots, the color of coffee with milk, are not uncommon in the newborn. In dark-skinned individuals, the macule is darker than the surrounding skin and should be described as ''cafe sans lait'' (coffee without milk). The presence of more than six of these spots, measuring greater than 0.5 cm,

Child Dermatology Physical ExamOverlying Skin Appears Intact

is the hallmark of neurofibromatosis type I, an autosomal dominantly inherited condition that combines cafe au lait spots, axillary or inguinal freckles (see Fig. 8-47), pigmented hamartomas in the iris (Lisch nodules), and bony abnormalities such as scoliosis and pseudarthrosis with benign tumors of the Schwann cells called neurofibromas. In this condition, multiple cafe au lait spots are frequently the presenting feature.

The presence of hypopigmented macules suggests a condition known as tuberous sclerosis complex. In this disorder, the hypomelanotic macules (Fig. 24-12) are often described as ash-leaf shaped, with one side smooth and the other side jagged, and are associated with other derma-tologic manifestations (facial angiofibromas known as adenoma sebaceum, and shagreen patches, as seen in Fig. 24-13), the presence of benign tumors in the brain (cortical ''tubers,'' subependymal nodules), kidney (angiomyolipomas and cysts), and heart (rhabdomyomas), seizures, and mental retardation. Because of the significance of some of these findings, the presence of multiple hypomelanotic ash-leaf spots noted during an initial evaluation should trigger a full evaluation for associated features.

Is a rash present? Bullous lesions may be present at birth. One or two blisters on the lips or hands may represent ''sucking blisters.'' Widespread blisters, many of them ruptured and leaving a collarette of scale with underlying pigmentation, represent transient neonatal pustular melanosis, which is benign. This condition, present at birth, is of unknown origin and is most commonly found on the trunk and extremities. It is seen in 5% of African-American newborns and in 0.5% of white newborns. Figure 24-14 shows transient neonatal pustular melanosis in a newborn. Notice the intact pustule and the ruptured pustule with a collarette of scale. The vesicopustules last 48 to 72 hours, and the pigmented macules may last 3 weeks to 3 months.

Erythema toxicum is a common rash among newborns. It is a self-limited, benign eruption of unknown cause, consisting of erythematous macules, papules, and pustules. The condition is

Hypopigmented Macules InfantsPallor Palmar Creases

seen in 40% of otherwise healthy term newborns; it is not seen in premature newborns. The lesions may appear anywhere on the body except on the palms and soles and have the appearance of flea bites. It is most commonly seen during the first 3 to 4 days after birth but may be present at birth. The lesions may last 2 to 3 weeks. Erythema toxicum is pictured in Figure 24-15.

Milia on the face are seen in almost 50% of all newborns. Milia appear as tiny whitish papules on the cheeks, nose, chin, and forehead and usually disappear by 3 weeks of age.

Blisters which first appear at 3 to 4 days of life may be staphylococcal pustulosis, which manifests as pustular or bullous skin lesions found mainly around the groin and umbilicus. The vesicular lesions of herpes simplex also usually appear after the third day after birth, up to the third week.

Transient Neonatal Pustular Melanosis
Figure 24-14 Transient neonatal pustular melanosis.

Figure 24-15 Erythema toxicum.

Herpes Simplex Rash Birth
Figure 24-16 Congenital rubella with a blueberry muffin rash.

Prenatal or transplacental infections of the fetus may manifest with cutaneous symptoms. If a pregnant woman contracts rubella in the first trimester, there is a 20% chance that the infant may have the congenital rubella syndrome. A cutaneous sign of congenital rubella is the blueberry muffin lesion. Such lesions, which represent sites of extramedullary hematopoiesis, are bluish-red macular/papular lesions ranging in size from 2 to 8 mm. They are noted at birth or within the first 24 hours and appear on the face, neck, trunk, or extremities. Other features of the congenital rubella syndrome include eye defects, cardiac defects such as ventriculoseptal defects and valvular defects, deafness, bone lesions, hepato-splenomegaly, jaundice, thrombocytopenia, interstitial pneumonitis, and, later, mental retardation. Figure 24-16 shows a child with the classic blueberry muffin rash of congenital rubella.

Congenital syphilis may present as an erythematous maculopapular rash that later turns brown or becomes a hemorrhagic vesicular rash. This is a rash that commonly affects the palms and soles.

Is hair present? A newborn's skin may be covered with fine, soft, immature hair, known as lanugo hair. Lanugo hair frequently covers the scalp and brow in premature infants but is usually absent in term infants, except perhaps on the ears and shoulders. Inspect the lum-bosacral area for tufts of hair. Tufts of hair (hypertrichosis) in this area are suggestive of the presence of an occult spina bifida or a sinus tract, anomalies that may be an external sign of tethering of the spinal cord. Figure 24-17 shows sacral hypertrichosis in a child who had a tethered spinal cord. Examine the fingernails. In a post-term infant, the fingernails are long and may be stained yellow if meconium was present in the amniotic fluid. Hypoplastic fingernails may be a marker for fetal alcohol syndrome.

Examine the dermatoglyphics of the fingers, palms, and soles. In addition to their value for identification purposes, these patterns are important indicators of genetic abnormalities. Normal finger dermatoglyphic patterns are the loop, whorl, and arch. The loop is normally the most prevalent pattern. The arch is the least prevalent pattern, and the presence of more than four arches is usually abnormal, often signaling the presence of congenital abnormalities. A single transverse palmar crease, known as a simian crease, is found in more than 50% of individuals with chromosomal abnormalities such as trisomy 21;however, simian creases are present in 10% of individuals who are normal.

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