Chapter 22: Café Au Lait Macule

INTRODUCTION

Café au lait macules (CALMs) are benign well-demarcated, light brown macules that typically present in early childhood. The pigmentation is typically uniform. Lesions may be multiple or isolated. They grow in proportion to the growth of the child. They are present in as many as 20% of the population and, rarely, can be associated with a host of genodermatoses.

EPIDEMIOLOGY

Incidence: 10% to 20% of the population

Age: birth and early childhood

Race: more common in African Americans than Caucasians

Sex: none

Precipitating factors: most commonly these are benign, isolated findings in healthy children. Multiple CALMs can be associated with genodermatoses such as neurofibromatosis, tuberous sclerosis, Bloom syndrome, McCune–Albright syndrome, Russell–Silver syndrome, Watson syndrome, and Westerhof syndrome

PATHOGENESIS

Unknown.

PATHOLOGY

Increased melanin in basal keratinocytes. Clinically darker lesions contain more melanocytes than lighter ones.

PHYSICAL LESIONS

Lesions are well demarcated, uniformly pigmented macules that vary in color from hues of tan to light brown to brown. They can present anywhere on the body but spare mucous membranes. Their size can range from a few millimeters to over 20 cm.

DIFFERENTIAL DIAGNOSIS

Postinflammatory hyperpigmentation, Becker’s nevus, melasma, lentigines, ephelides, berloque dermatitis, and congenital nevus.

LABORATORY EXAMINATION

Biopsy is not indicated. Additional laboratory workup may be appropriate in the event of suspicion of an underlying systemic disorder.

COURSE

They grow in proportion to the growth of the child. Once a child has fully grown, CALMs do not change in size or color. There is no increased risk of malignant transformation.

KEY CONSULTATIVE QUESTIONS

• Time of onset

• Failure to meet milestones

• Photosensitivity

• Intellectual impairment

• History of multiple fractures

• Central nervous system disorders or tumors

• Poor growth

• Scoliosis

• Ophthalmologic impairment

MANAGEMENT

CALMs do not require treatment unless their appearance is disfiguring or distressing to the patient or parents. Multiple lesions may suggest an underlying systemic disorder. If there is any indication of underlying systemic abnormalities in the setting of multiple CALMs, referral to appropriate pediatric specialists is indicated. Laser therapy is often employed as a treatment. CALMs tend to be more difficult to treat than other benign pigmented lesions such as ephelides and lentigines. They require multiple treatments and complete resolution can be challenging. Recurrence is common. Cryotherapy and surgical excision are alternatives to laser therapy but carry the risk of pigmentary alterations, poor cosmesis, pain, and scarring.

LASER TREATMENT