Strange and Schafermeyer's Pediatric Emergency Medicine, Fourth Edition (Strange, Pediatric Emergency Medicine), 4th Ed.

CHAPTER 92. Newborn and Infant Rashes

Sarah Judd

HIGH-YIELD FACTS

• Nevus flammeus, or Port-wine stains, when present in the area innervated by the ophthalmic branch of the facial nerve is associated with Sturge–Webber syndrome, a neurocutaneous disorder with vascular malformations of the brain and intractable seizures.

• Multiple or clustered capillary hemangiomas may be associated with deep tissue and parenchymal involvement and further evaluation is necessary.

• Candida skin or oral infections may be secondary to excessive use of oral antibiotics. Treatment includes antifungal agents such as nystatin, ketoconazole, or clotrimazole as well as consideration of probiotics and hygiene.

• Dermal melanosis or Mongolian spots are dark blue–grey patches of melanocytes located in the dermis. Mongolian spots should not be confused with bruising.

• Seborrheic dermatitis can be recognized clinically by the presence of greasy scales and erythematous plaques.

• Diaper dermatitis is usually caused by irritation of the skin from prolonged contact with feces and urine. Sparing of the skin folds is diagnostic.

• Multiple café au lait spots of neurofibromatosis increase the risk for auditory and CNS tumors.

• Vascular malformations in a “beard distribution” on the face are associated with airway hemangiomas.

There is a tremendous burden on the newborn infant to abruptly adapt to the new extrauterine environment. Many changes occur, including those within the skin and dermal systems. Benign and transient rashes commonly occur and often cause significant parental concern.

Milia are 1 to 2 mm, discreet pearly papules usually limited to the face and scalp. They are superficial inclusion cysts that contain laminated keratinized material of sebaceous origin.1 No treatment is necessary, and the lesions resolve spontaneously.

Miliaria are lesions caused by obstruction of eccrine sweat glands, and are particularly common in warm climates. Very superficial sweat gland obstruction results in miliaria crystallina which results from sweat being trapped in the intracorneal layer of the skin producing tiny clear vesicles. Miliaria rubra or heat rash is common in febrile or overheated infants. These are erythematous small papules that are most commonly found on the upper trunk and head. Neonatal acne would present as open or closed comedones concentrated to the face and upper chest which is thought to be related to excess maternal or exogenous androgen and self-limiting.

Erythema toxicum is present in up to 50% of newborn infants. The lesions usually appear by the second day of life, last approximately 1 week, and resolve spontaneously. The rash presents as pinpoint, papulopustular lesions on an erythematous base that appear on the face, trunk, and extremities. The lesions are at the opening of sebaceous ducts. Characteristic findings with Wright stain will reveal collections of eosinophils with absence of infectious organisms.1

Pustular melanosis is found almost exclusively in African American infants, and the lesions are usually present at birth. It presents with a combination of fine pustules, areas of fine scale, and hyperpigmented macules where prior pustules were located.1 A Wright stain of the contents will show a predominance of neutrophils, and absence of bacteria. There are a variety of vesiculopustular lesions that can be confused with pustular melanosis that may have serious complications (Table 92-1).3

TABLE 92-1

Vesiculopustular and Bullous Lesions with Systemic Complications2

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Seborrheic dermatitis in infants usually has its onset within the first 4 weeks of life. The scalp is often the first to show signs of the characteristic greasy yellow scales, occasionally with loss of hair (Fig. 92-1). Erythematous plaques develop and skin creases are often involved. The retroauricular area may have weeping or scaly denuded areas of involvement. Overproduction of sebum causes the accumulation of greasy scales, but the cause is still unclear. Treatment consists of low-potency topical corticosteroids to treat inflammation. Mild tar or selenium shampoo and oatmeal baths often help. When severe, consider the possibility of histiocytosis (Letterer–Siwe disease) or Leiner disease with generalized erythroderma and failure to thrive.

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FIGURE 92-1. Seborrheic dermatitis in infant.

Diaper dermatitis encompasses all causes of skin irritation localized to the diaper area. Irritant dermatitis is the most common cause and may be related to prolonged skin contact with urine and feces or from soaps and chemicals present in the diaper. Absorbable disposable diapers have decreased the incidence of this problem. Irritant dermatitis can usually be recognized by erythema or raw appearance and scaling of the skin with sparing of the skin folds. Secondary infection with Candida can confuse the appearance with involvement of the skin folds and presence of papular satellite lesions extending beyond the area of erythema (Fig. 92-2). Candida skin or oral infections may be secondary to excessive use of oral antibiotics. Treatment for external infection includes topical antifungal agents such as nystatin, ketoconazole, or clotrimazole as well as consideration of probiotics and hygiene. Oral candida is often treated with nystatin or fluconazole for 14 days.

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FIGURE 92-2. Neurofibromatosis with axillary freckling. (Crowe sign).

DISORDERS OF PIGMENTATION

Dermal melanosis or Mongolian spots are dark blue–gray patches of melanocytes located in the dermis. They are most commonly found on the buttocks and sacral region of the lower back and slowly resolve over years. When they are present on the extremities, shoulders, and face they may persist. Mongolian spots should not be confused with bruising.

Neurofibromatosis is a group of inherited neurocutaneous disorders manifested by multiple café au lait spots. Presence of six or more lesions measuring >5 mm in an infant or the presence of axillary “freckling” (Fig. 92-3) is diagnostic. These disorders are associated with a high incidence of optic gliomas, acoustic neuromas, and a variety of other CNS tumors.4 The patient with neurofibromatosis who presents with headache or focal neurologic signs must be evaluated for an intracranial mass lesion. Other causes of café au lait spots include McCune–Albright syndrome, which is characterized by polyostotic fibrous dysplasia and multiple endocrine abnormalities.

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FIGURE 92-3. Irritant dermatitis with Candida overgrowth.

Urticaria pigmentosa can present in the first few months of life as red–brown macules. Type 1, a solitary mastocytoma is of no consequence. Type 2, can present with multiple pigmented macules or papules that when scraped with a blunt object will produce a wheal and flare termed Darier sign (Fig. 92-4). This condition is caused by populations of mast cells in the cutaneous tissue. When irritated by scratching, they release histamine, thereby, producing the characteristic wheals and occasionally blisters. With temperature changes, metabolic insults, such as fever or viral illnesses, large numbers of the degranulating lesions can produce flushing and systemic symptoms of histamine release.

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FIGURE 92-4. Darier sign for urticaria pigmentosa.

Incontinentia pigmenti is an X-linked disorder occurring in females and is usually present at birth (Fig. 92-5). It is a neurocutaneous syndrome with four overlapping stages of skin presentation. The first stage is manifested by linear blisters surrounded by erythema. The second stage presents usually before 6 months of age as the blisters evolve into warty plaques. As the plaques disappear, they become whorls of hyperpigmentation during stage 3. The final stage occurs later in adulthood with hypopigmentation and loss of hair and sweat glands. Approximately 20% of patients will have seizures, various neurologic deficits, and mental retardation.

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FIGURE 92-5. Incontinentia pigmenti.

VASCULAR LESIONS OF INFANCY

Cutis marmorata is a vascular phenomenon noted in cold ambient temperature and may be present in an unclothed infant for the first few months of life. A reticulated, blanching rash is characteristic, and this may be confused with mottling. Acrocyanosis, that is cyanosis of the hands and feet, may also be present. Keeping the infant warm and in a neutral thermal environment will resolve the color changes in minutes.

Vascular malformations are common skin presentations at birth or in the first few months of life. They can be secondary to abnormalities of the capillary, venous, arterial, or lymphatic systems. Most are benign and self-limited, but a few may herald serious systemic consequences.5 The salmon patch is a common capillary malformation usually located on the forehead and upper eyelids and is present at birth. These usually resolve within 1 to 2 years, but if present on the back of the neck, may persist for life. Nevus flammeus, or Port-wine stains, are generally benign capillary malformations, but may persist for life. When present in the area innervated by the ophthalmic branch of the facial nerve (includes the upper eyelid and forehead), it is associated with Sturge–Webber syndrome, a neurocutaneous disorder with vascular malformations of the brain and intractable seizures.

Simple capillary hemangiomas are the most common vascular tumors in infancy. They may be unrecognized at birth, but appear early in the neonatal period as a small, red telangiectasia that rapidly grows to become the characteristic Strawberry hemangioma over the first 6 months to 1 year, then slowly regress and disappear over the first decade. Multiple or clustered capillary hemangiomas may be associated with deep tissue and parenchymal involvement and further evaluation is necessary. Vascular malformations of the skin over the “beard distribution” of the face in an infant presenting with stridor or upper airway involvement suggests airway hemangiomas.

There are a number of hereditary and congenital conditions with vascular, hyper- and hypopigmented lesions, bulla, or ichthyotic scale that are beyond the scope of this text.

ACKNOWLEDGMENTS

The author would like to thank prior contributors: Robert A. Wiebe and Malee V. Shah

REFERENCES

1. Kleigman RM, Stanton B, St Geme J, Schor N, Behrman R. Nelson Textbook of Pediatrics. Elsevier Health Sciences; 2011.

2. Eichenfeld LF, Frieden IJ, Esterly NB. Textbook of Neonatal Dermatology. Philadelphia, PA: WB Saunders Company; 2001.

3. Van Praag MG, Van Rooij RG, Folkers E, Spritzer R, Menke HE, Oranje AP. Diagnosis and treatment of pustular disorders in the neonate. Pediatr Dermatol. 1997;14:131–143.

4. Yohay K. Neurofibromatosis types 1 and 2. Neurologist. 2006;12(2):86–93.

5. Blei F. Basic science and clinical aspects of vascular anomalies. Curr Opin Pediatr. 2005;17(4):501–509.