Spot the Rash

A neonate with white papules on the face

A 5-day-old healthy male presents to your clinic for a well-child examination. The parents report he is doing well but have some concerns about his skin. Shortly after birth, they noted several white papules scattered mainly on his cheeks and around his eyes. They do not seem to bother him. His mother reports that she occasionally develops similar lesions on her eyelids.

On physical examination, he is well appearing and alert. Lateral to his right eye and on his cheeks are a few discrete scattered 1-mm to 2-mm firm, round white papules (Figure 1). The remainder of a full skin examination is normal.

Can you spot the rash?

Diagnosis: Milia

Milia (singular – milium) are keratinaceous cysts that are commonly seen congenitally in full-term newborns. They are seen in nearly half of full-term newborns and occur most commonly on the face. These milia tend to resolve spontaneously during the first several weeks to months of life.

The pathogenesis of milia in newborns is not known. On histopathology, milia are small epidermal cysts surrounded by a wall of stratified squamous epithelium. In the case of primary milia, as in the congenital type, milia are thought to originate from sebaceous ducts. In secondary forms, they are thought to originate from the eccrine duct.

Marissa J. Perman

Marissa J. Perman

Besides newborns, children and adults may also develop primary milia on the face, especially the eyelids and cheeks. Other less common locations include the genitalia and nasal crease. Nasal crease milia are often seen in combination with small inflammatory papules and are referred to as “pseudo-acne of the nasal crease.” Milia in this setting may be seen more often in patients who are atopic and rub their noses frequently (known as the “allergic salute”).

Primary milia are also found in several genodermatoses, including but not limited to pachyonychia congenita type 2, basal cell nevus syndrome, Rombo syndrome, Brooke-Spiegler syndrome, Bazex-Dupre-Christol syndrome, atrichia with papular lesions, and orofaciodigital syndrome type I.

Secondary milia are usually due to trauma or rubbing and are also seen in certain genodermatoses with frequent skin trauma or blistering such as porphyria cutanea tarda and epidermolysis bullosa, particularly the dystrophic type. Milia may also occur in infants who undergo numerous needle sticks for IV placement.

In neonates, milia are commonly confused with sebaceous hyperplasia, which occurs on the nose, nasal ala and upper lip (sites of increased sebaceous glands), and is easy to differentiate on physical examination. Sebaceous hyperplasia presents as numerous 1-mm to 2-mm follicular, monomorphic, yellow-white soft papules.

Milia-like calcified nodules also occur in infants, may be referred to as heel-stick calcinosis, and are found in premature infants who have undergone multiple heel sticks.

A newborn with a discrete 2-mm firm white cystic papule on the cheek.

A newborn with a discrete 2-mm firm white cystic papule on the cheek.

Image: Perman MJ

Treatment is unnecessary in most patients because milia often resolve over time without therapy. For patients seeking removal of a single or few lesions, incision with a small needle or scalpel blade followed by gentle pressure with a comedone extractor is often successful. In addition, topical retinoids may be used to aid in resolution of multiple lesions.

Physicians should be familiar with milia and their associations because they are frequently encountered in pediatric practice.

References:
Berk DR. J Am Acad Dermatol. 2008;59:1050-1063.

Disclosure: Perman reports no relevant financial disclosures.

A 5-day-old healthy male presents to your clinic for a well-child examination. The parents report he is doing well but have some concerns about his skin. Shortly after birth, they noted several white papules scattered mainly on his cheeks and around his eyes. They do not seem to bother him. His mother reports that she occasionally develops similar lesions on her eyelids.

On physical examination, he is well appearing and alert. Lateral to his right eye and on his cheeks are a few discrete scattered 1-mm to 2-mm firm, round white papules (Figure 1). The remainder of a full skin examination is normal.

Can you spot the rash?

Diagnosis: Milia

Milia (singular – milium) are keratinaceous cysts that are commonly seen congenitally in full-term newborns. They are seen in nearly half of full-term newborns and occur most commonly on the face. These milia tend to resolve spontaneously during the first several weeks to months of life.

The pathogenesis of milia in newborns is not known. On histopathology, milia are small epidermal cysts surrounded by a wall of stratified squamous epithelium. In the case of primary milia, as in the congenital type, milia are thought to originate from sebaceous ducts. In secondary forms, they are thought to originate from the eccrine duct.

Marissa J. Perman

Marissa J. Perman

Besides newborns, children and adults may also develop primary milia on the face, especially the eyelids and cheeks. Other less common locations include the genitalia and nasal crease. Nasal crease milia are often seen in combination with small inflammatory papules and are referred to as “pseudo-acne of the nasal crease.” Milia in this setting may be seen more often in patients who are atopic and rub their noses frequently (known as the “allergic salute”).

Primary milia are also found in several genodermatoses, including but not limited to pachyonychia congenita type 2, basal cell nevus syndrome, Rombo syndrome, Brooke-Spiegler syndrome, Bazex-Dupre-Christol syndrome, atrichia with papular lesions, and orofaciodigital syndrome type I.

Secondary milia are usually due to trauma or rubbing and are also seen in certain genodermatoses with frequent skin trauma or blistering such as porphyria cutanea tarda and epidermolysis bullosa, particularly the dystrophic type. Milia may also occur in infants who undergo numerous needle sticks for IV placement.

In neonates, milia are commonly confused with sebaceous hyperplasia, which occurs on the nose, nasal ala and upper lip (sites of increased sebaceous glands), and is easy to differentiate on physical examination. Sebaceous hyperplasia presents as numerous 1-mm to 2-mm follicular, monomorphic, yellow-white soft papules.

Milia-like calcified nodules also occur in infants, may be referred to as heel-stick calcinosis, and are found in premature infants who have undergone multiple heel sticks.

A newborn with a discrete 2-mm firm white cystic papule on the cheek.

A newborn with a discrete 2-mm firm white cystic papule on the cheek.

Image: Perman MJ

Treatment is unnecessary in most patients because milia often resolve over time without therapy. For patients seeking removal of a single or few lesions, incision with a small needle or scalpel blade followed by gentle pressure with a comedone extractor is often successful. In addition, topical retinoids may be used to aid in resolution of multiple lesions.

Physicians should be familiar with milia and their associations because they are frequently encountered in pediatric practice.

References:
Berk DR. J Am Acad Dermatol. 2008;59:1050-1063.

Disclosure: Perman reports no relevant financial disclosures.