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CONGENTINAL LESIONS

Common skin conditions in the newborn

A variety of skin conditions are common incidental findings in the newborn. In general, no treatment is needed (Figs 19.1 – 19.8).

Figure 19.1  Sebaceous hyperplasia. The sebaceous glands of the newborn may be temporarily enlarged at birth secondary to stimulation by maternal hormones. Note the pinpoint yellow papules on this infant's nose.

Figure 19.2  Milia. Small, white milia are common on the face of newborns. Spontaneous resolution is expected.

Figure 19.3  Lanugo hair. The presence of increased hair in an infant, as shown here on the forehead, is common and temporary.

Figure 19.4  Erythema toxicum. Erythematous macules (1–3 cm in diameter) with a central papulopustule in a newborn is characteristic of this benign and transient condition.

Figure 19.5  Erythema nuchae. A congenital vascular patch on the nape occurs very commonly and may persist throughout life.

Figure 19.6  Salmon patch. A vascular blanching red patch or streak across the forehead or glabella occurs in many newborn infants. It usually fades, in contrast to port wine stains or vascular patches on the nape, which persist.

Figure 19.7  Scalp: electrode laceration. Superficial lacerations may be seen in the newborn, caused by the scalp fetal monitor. Herpes simplex infection has also been associated. Rarely, scalp abscesses may occur.

Figure 19.8  Sucking pad

Figure 19.9  Supernumerary digit: (a) a solitary lesion; (b) bilateral lesions in a typical location

Congenital and early-onset vascular anomalies

Congenital vascular anomalies are divided into two broad categories.

These conditions and their classification are discussed in Chapter 15.

Common infantile lesions such as nevus flammeus, ‘stork bite', and ‘salmon' patch are excluded from modern classifications of vascular anomalies. They occur mainly on the nape of the neck and forehead, and are viewed as dermal vascular ectasias that will fade. However:

Supernumerary digit

A supernumerary digit is congenital and usually located at the ulnar side of the base of the fifth finger. It may range in size from a small papule to a large (sixth) digit (Fig.19.9). Surgery is appropriate if needed.

Accessory tragus

A congenital, firm papule or multilobular nodule occurring in the preauricular area is characteristic (Fig.19.10). The lesion may consist of skin only, or skin and cartilage. It presumably developed from the auricular hillocks of the first branchial arch. These lesions may occasionally be bilateral or familial, or occur in association with other facial abnormalities. Congenital ear defects may be associated with kidney abnormalities, as both are formed at the same embryologic stage.

    The preauricular tag may be simply excised. However, if there is any question of a sinus or deep extension, excision should be done by a pediatric otolaryngologist.

Figure 19.10  Accessory tragus. This congenital lesion is thought to represent a remnant of the first branchial arch.

Congenital preauricular sinus

A preauricular pinpoint opening (Fig.19.11) may indicate an underlying sinus or fistula. Acute swelling and pain indicate a bacterial infection. This sinus may be familial (autosomal dominant). A sinus is much more common than a fistula and usually ends blindly, joining with the periosteum of the auditory canal. Rarely, there is an auricular fistula that connects with the tympanic or intratonsillar cleft. There are rare associations, most commonly with deafness and branchio–oto–renal (BOR) syndrome. If other anomalies are present, auditory and renal testing should be considered. An oral antibiotic should be given for any infection. A pediatric otolaryngologist may perform a surgical excision on the uninfected sinus.

Accessory nipple

Also known as polythelia, this lesion appears as a small, brown papule that may resemble a nipple, along the milk line that stretches from the axilla through the normal nipple to the groin. Only if there is breast tissue can conditions such as fibrocystic disease, abscess, or cancer develop. An association with urinary tract abnormalities has been reported. In one study, kidney and urinary tract malformations (e.g. adult polycystic kidney disease, unilateral renal agenesis, cystic renal dysplasia, familial renal cysts, and congenital stenosis of the pyeloureteral joint) occurred in 7.5% of patients with polythelia versus 0.7% of controls. If an analysis of the urinary system is indicated, an ultrasound examination may be done.

Figure 19.11  Congenital preauricular sinus. This preauricular hole most often ends blindly. The main risk it poses is secondary infection.

Figure 19.12  Accessory nipple. (a) Also known as polythelia, this lesion appears as a small, brown papule that may resemble a nipple. One, two (as shown), or more may occur. If two occur, they often appear on opposite sides at the same level. (b) Close-up view.

Figure 19.13  Wattle. (Courtesy of Tanya Forman, M.D.)

    One, two, or more lesions may occur. If two occur, they often appear on opposite sides at the same level (Fig.19.12). There is an equal sex and side incidence. No treatment is needed. Simple excision may be done.

Wattle

Also known as cutaneous cervical tag, the wattle presents as a pedunculated, flesh-colored tag on the neck (Fig.19.13). Onset is usually by 3 years of age. A branchial sinus may be associated. The wattle probably represents either ectopic auricular cartilage or a remnant of the branchial arch system. Simple excision may be done if no sinus or fistula is associated.

Absent fingerprints

Rarely, a person may be born without fingerprints (Fig.19.14).

Midline sacral lesions

Clinical

Lipomas, sinus tract, pigmented lesions, tufts of hair (e.g. fawn tail nevus), and vascular patches are some of the congenital, sacral, cutaneous abnormalities that may be associated with an underlying spinal malformation (Fig.19.15), also known as spinal dysraphism. Potential associations include tethered cord, spina bifida, and vertebral anomalies. In one study, dimples or pits (defined as a depression whose base could not be visualized with or without traction) alone were not associated with an underlying abnormality. It appears that the presence of two or more lesions makes finding an underlying abnormality more likely.

Figure 19.14  Absent fingerprints. This patient was on record with the FBI for being born without fingerprints (or so he said!).

Figure 19.15  Meningomyelocele. (Courtesy of O. Dale Collins III, M.D., Ph.D.)

    If occult spinal dysraphism is suspected, magnetic resonance imaging (MRI) is the imaging method of choice. Plain radiographs may be negative. If a dermal sinus tract is not suspected, MRI may be delayed until 4 months of age to allow for better imaging. Until approximately 6 months of age, when the posterior vertebral elements ossify, ultrasound is an excellent screening tool. If plain radiographs and spinal ultrasound are unremarkable in an infant with a suspicious cutaneous sacral lesion who is otherwise normal, MRI may not be necessary.

    Biopsy or surgical removal of a cutaneous midline lesion should not be attempted before a non-invasive work-up has been done.

Embryonic tail

A congenital, pedunculated, tail-like lesion attached to the sacrum is characteristic of a ‘human tail' (Fig.19.16). It may contain adipose, collagen, skeletal muscle fibers, blood vessels, and nerves, but it does not contain bone, cartilage, or spinal cord elements. Some reports have documented movement and contraction. Associated abnormalities are common, including spina bifida, meningocele, cleft palate, and skeletal defects.

    After careful physical examination and radiographic imaging of the area, surgery may be done.

Figure 19.16  Embryonic tail. (Courtesy of Mary M. Dobry, M.D.)

Figure 19.17  Multiple congenital milia or small epidermoid cysts along the raphe. (Courtesy of O. Dale Collins III, M.D., Ph.D.)

Median raphe cyst

There is a barely raised line or fold, termed the raphe, that runs along the midline from the urethra, along the penis, scrotum, and perineum to the anus, in a male. Congenital cysts that are found along this line are called median raphe cysts. These cysts are thought to arise as a fusion defect or entrapment phenomenon occurring during the embryonic development of the raphe. An asymptomatic (unless infected) cyst, which may form along the raphe from the glans to the anus, is characteristic. It may present with acute inflammation and enlargement at puberty or soon after due to infection with staphylococcus, Neisseria gonorrhoeae , or simple trauma from intercourse. Culture for gonorrhea should be done. Inquiry about penile discharge or other signs of sexually transmitted disease is appropriate. Multiple congenital milia along the median raphe have been reported (Fig.19.17).

    Simple surgical excision may be performed. Observation alone may be appropriate, with intervention anticipated if symptoms arise.

Nasal glioma

Etiology and pathogenesis

The nasal glioma (also known as nasal glial heterotopia) falls under the category of heterotopic brain tissue (Fig. 19.18). There is no connection with the central nervous system, in contrast to an encephalocele or meningocele, and thus nasal gliomas do not change size or shape as a result of crying or straining, nor do they distend with jugular vein compression (Furstenberg sign).

Clinical

A congenital, firm, non-transilluminating, blue or red nodule just lateral to the nasal root (extranasal glioma) is characteristic. It may also protrude from the nasal cavity (intranasal glioma) or be mixed. Some degree of nasal obstruction may occur.

Differential diagnosis

This includes other congenital nodules of the face, notably the following.

Treatment

Preoperative imaging and surgical excision by a neurosurgical specialist is recommended. Surgery should be performed in childhood to prevent the deforming effects on developing facial bones.

Dermoid cyst

Etiology and pathogenesis

The dermoid cyst is present at birth and is composed of epithelium and appendageal elements; it may contain bones, hair, or teeth.

Clinical

A round, subcutaneous, asymptomatic congenital nodule at the tail of the eyebrow (Fig.19.19), periorbitally, or on the nose or scalp is characteristic. In a recent retrospective review of orbitofacial dermoids, those of the brow–frontotemporal region were the most common. In none of the 54 was there a bony attachment or transcranial communication. The second most common location was in the orbit. In this group of 21 orbital dermoids, none had an intracranial connection. However, of nine nasoglabellar dermoids included in the analysis, several showed an intracranial connection.

    Preoperative MRI is recommended, although the yield for brow– frontotemporal lesions is low. An ophthalmologist should be consulted for orbital lesions.

Differential diagnosis

Lesions discussed in the differential diagnosis of nasal glioma should be considered.

    Patients with a dermoid cyst may be referred at an older age with a diagnosis of epidermoid cyst, but the site, congenital nature, and lack of punctum should rapidly distinguish the two.

Figure 19.18  (a,b) Nasal glioma. The nasal glioma represents a congenital deposit of brain tissue. There is no connection with the central nervous system, in contrast to an encephalocele or meningocele.

Figure 19.19  Dermoid cyst. This round, subcutaneous, asymptomatic, congenital nodule at the tail of the eyebrow was presumed to be a dermoid cyst.

Treatment

Dermoids may be removed surgically. For isolated dermoids in the brow–frontotemporal region, simple excision may be done, although incision through the frontalis muscle is needed. After removal of lesions of the orbit, volume replacement may be needed for the larger lesions. Finally, those in the nasoglabellar region may show transcranial extension, and craniotomy may be needed.

Median nasal dermoid fistula

The median nasal dermoid fistula is a dermoid cyst with a connection to the surface. A midline pit or fistula, often with hairs emanating from it, on the dorsum of the nose is characteristic (Fig.19.20). Sebaceous material may emanate spontaneously or on compression. The fistula communicates with a dermoid cyst, which usually occurs posterior to the nasal bones but may also occur in the nasal septum, ethmoid, or sphenoid bones, or attached to the dura. A firm, painless swelling may be noted anywhere from the glabella to the tip of the nose. Intermittent drainage, abscess formation, meningitis, or osteomyelitis may occur.

    Surgical excision is recommended by a trained specialist able to remove the entire lesion.

Figure 19.20  (a) Median nasal dermoid fistula. (b) Dermal fistula. (Panel a courtesy of Michael O. Murphy, M.D.)

 

Figure 19.21  Nevus sebaceus. (a) A round or oval, red to yellow, smooth plaque on the scalp at birth is characteristic. The face is the second most commonly affected site. (b) At puberty, under hormonal influence, the plaque may become verrucous or studded with multiple papules. (c) The systematized variant. Note the diffuse yellow plaques, causing alopecia. Some pigmented areas are associated. This is a variant of an epidermal nevus.

PRACTICE POINTS

White/Cox: Diseases of the Skin, 2ed.(c) 2006, Elsevier Inc. All rights reserved.