Neonatal acne (neonatal cephalic pustulosis) occurs in more than 20% of healthy newborns (see Chapter 36). Lesions appear at about 2 weeks of age and generally resolve within the first 3 months of life. Typically, small, inflamed papules arise on the cheeks and across the nasal bridge. The classification of neonatal acne is being re-examined (at the time of this writing) because of reports that implicate Malassezia furfur in its pathogenesis⁹. It is now widely held that these early papular lesions are the result of direct infection by this ubiquitous yeast. Additional support comes from the clinical response to treatment with topical 2% ketoconazole cream. Previously, benzoyl peroxide had also been shown to be effective. Given the transient and benign nature of this eruption, parental reassurance alone is usually adequate.

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Infantile acne

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If acne presents at 3-6 months of age, it is classified as infantile (Fig. 38.13). Clinically, comedo formation is much more prominent than in the neonatal form and may lead to pitted scarring. Deep cystic lesions and suppurative nodules are occasionally seen. The pathogenesis of infantile acne reflects the hormonal imbalances intrinsic to this stage of development, and maternal hormones play only a minor role. During the first 6-12 months of life, infant boys have elevated levels of luteinizing hormone (LH) and its stimulatory product testosterone¹⁵, with levels transiently equivalent to those measured during puberty. In addition, the infantile adrenal gland is immature in both boys and girls, leading to elevated levels of DHEA (see Pathogenesis). At approximately 12 months, these levels normally decrease and remain at nadir levels until adrenarche, around 9 or 10 years of age. Testicular androgen is also minimal throughout most of childhood²⁹. Infantile acne typically resolves within 1-2 years and remains quiescent. In unusual cases, however, the acne may persist well into and throughout adolescence. Topical tretinoin or benzoyl peroxide is usually prescribed for comedonal infantile acne to obviate the risk of permanent scarring. Oral isotretinoin is reserved for severe nodulocystic presentations³⁰.

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Endocrinologic abnormalities

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Although most patients with acne do not have overt endocrinological abnormalities, hyperandrogenism should be suspected in female patients with hirsutism (see Chapter 70) or irregular menstrual periods. Their acne is generally severe or more difficult to treat, and the onset can be fairly abrupt. Other signs and symptoms of an increased androgen state include coarsening of the voice, muscular habitus, androgenetic alopecia, clitoromegaly with variable posterior labial fusion, and increased libido. Insulin resistance and acanthosis nigricans can occur in association with hyperandrogenism in the HAIR-AN syndrome. These patients are at increased risk for accelerated cardiovascular disease and diabetes mellitus and should be followed by appropriate medical specialists.

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The evaluation of patients suspected of having hyperandrogenism includes a thorough history and physical examination; the age of the patient and pubertal status are also important to take into account. Prepubertal boys and girls and postpubertal women with signs should undergo appropriate evaluation. Laboratory studies should be performed approximately 2 weeks prior to the onset of menses, during the luteal phase of the menstrual cycle. Initial tests should include serum total and free testosterone, DHEAS, and an LH/follicle-stimulating hormone (FSH) ratio.

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Understanding the hormonal pathway is essential in the evaluation of hyperandrogen states. For example, an elevated serum DHEAS or 17-hydroxyprogesterone indicates an adrenal source of excess androgen production. The levels of these hormones are then useful in discerning an etiology. DHEAS values in the range of 4000-8000 ng/ml or 17-hydroxyprogesterone levels >3 ng/ml may be indicative of congenital adrenal hyperplasia. Defects in the adrenal enzymes 21-hydroxylase or 11-hydroxylase lead to this condition (Fig. 70.6). Patients with severe deficiencies of these enzymes will present during infancy, whereas the majority of affected individuals have partial deficiencies and can present into adolescence. If the serum DHEAS is greater than 8000 ng/ml, with or without an elevated testosterone level, an adrenal tumor should be suspected.

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If the total testosterone level is elevated, an ovarian source is likely. Polycystic ovarian syndrome (PCOS) is the most common condition associated with an elevated serum testosterone, with levels ranging from 150 to 200 ng/dl. An increased LH/FSH ratio greater than 2-3 can also be observed. Symptoms of PCOS include irregular menstrual periods, hirsutism, obesity, insulin resistance and reduced fertility. When levels of serum testosterone exceed 200 ng/dl, an ovarian tumor should be considered. As androgen levels vary significantly in each individual, any abnormal laboratory studies bear repeating to confirm an abnormality before further evaluation or initiation of treatment.