Dermatology

A persistent, eczematous dermatitis had developed in the perioral area during the winter months in this 10-year-old boy. Topical corticosteroid creams had been tried, and these seemed to help some, but the ondition never really cleared. Because of the failure of the corticosteroid creams, a topical antifungal cream had also been tried; however, this, too, was of limited effectiveness.

An 18-month-old white boy is brought to his well-care visit by his parents, who are concerned that for the past month he has been less social and active. He has appeared weak and has refused to walk or play with his siblings. He has also had a decreased appetite and has lost about 2 kg. He has vomited several times but with no bile or blood.

This infant was noted to have a giant melanocytic nevus at birth. Giant congenital melanocytic nevi (GCMN) can be recognized not only by their increased size (greater than 20 cm) but also by their increased cellularity and ability to affect deep dermal layers and other subcutaneous tissue. 1-4 Acquired nevi generally do not permeate the deeper dermal layers. Occasionally, GCMN may result in dysplasia of intraepidermal nevus cells, inflammatory spread of nevus cells, and proliferative dermal nodules.1

Pityriasis rosea had been diagnosed in this 10-year-old boy 3 months earlier. However, the condition had not resolved within that time as had been predicted. In addition, the patient’s parents noted that pictures of pityriasis rosea that they had found on the Internet did not resemble their child’s skin.

HISTORYBaby boy born at full term via forceps-assisted vaginal delivery to a 17-year-old primigravida was noted to have left arm and chest anomalies. Apgar scores 9 at 1 minute and 9 at 5 minutes. No pregnancy complications except for low amniotic fluid index. Second trimester ultrasonogram showed no malformations. At birth, infant was vigorous. He tolerated feeding well and passed urine and stool appropriately. Results of a hearing screen and newborn metabolic screen were normal. No family history of genetic disorders.PHYSICAL EXAMINATIONClinically, infant was asymptomatic. Vital signs normal. Weight, height, and head circumference (3.040 kg, 50.8 cm, and 33 cm, respectively) appropriate for gestational age. He had a flat left upper chest, an abnormal and caudally displaced left nipple, hypoplasia of the muscles of the upper left shoulder girdle and arm, significant shortening and bowing of the left forearm, and left hand cutaneous syndactyly. The left thumb resembled a finger and was not opposable. Heart sounds heard best to the right of midline, corresponding to dextroposition of the heart. No heart murmurs. Remaining physical findings unremarkable.WHAT’S YOUR DIAGNOSIS?(Answer and discussion on next page)ANSWER: POLAND SYNDROMEPoland syndrome, also known as Poland sequence, is a rare congenital anomaly characterized by unilateral chest wall hypoplasia and ipsilateral anomalies of the upper extremity. The condition, first described by Dr Alfred Poland in 1841, was officially named in 1962 by Dr Patrick Clarkson, who reported on 3affected children. The incidence of Poland syndrome is 1 per 20,000 to 30,000; however, it is believed to be underreported. The syndrome is 3 times more likely in males than in females; the right side is favored over the left.1ETIOLOGYDiminished blood supply to the affected side secondary to developmental defects of the subclavian artery is the suspected cause of Poland syndrome. Maternal drug abuse during pregnancy has been reported.2,3 Most cases of Poland syndrome arise sporadically. Familial cases have been described, which suggests some degree of genetic transmission. However, a case of Poland syndrome in one identical twin has been reported.4CLINICAL MANIFESTATIONSThe minimum criterion needed for diagnosis is unilateral absence or hypoplasia of the sternal portion of the pectoralis major muscle. Other classic features on the ipsilateral side include: •Hypoplasia or aplasia of the breast and nipple. •Deficiency of subcutaneous fat and axillary hair. •Abnormalities of the rib cage. •Upper extremity anomalies, such as short arm and fingers as well as syndactyly.2It is not rare for infants with Poland syndrome to have all the classic features. Intelligence is not impaired in children with this syndrome.

The most common chronic medical problem that we pediatricians treat is asthma. We do our best to manage our patients’ asthma by prescribing controller medications, providing asthma action plans, and guiding families through acute exacerbations. We often ask about possible environmental triggers, such as tobacco smoke and cockroaches, and we advise patients to reduce their exposure to those triggers.

The lesion on the left forearm of this 20-month-old girl developed shortly after birth and gradually worsened. The toddler scratched the lesion daily. It bled on occasion. The mother vigilantly kept the baby’s nails short and administered an oral antihistamine regularly. There was a family history of chronic eczema.

A review of several recent medical journal articles of interest to pediatricians.

An 11-year-old comes into the office with an asymptomatic slowly progressive eruption that began 3 years prior. The problem is finally resolved with topical and/or systemic treatment.

A 12-year-old is brought into the office for a 10-month-old generalized itchy skin eruption.

Sleep is an important, yet frequently underestimated component of adolescent health. Adequate sleep is essential for achieving maximal cognitive abilities as well as for maintaining the energy needed to meet the demands of a busy adolescent’s schedule. Lack of quality sleep can result in attention problems, cognitive dulling, various somatic complaints (such as headaches and abdominal pain), and mood disturbances.

A 3-year-old girl was with her family on vacation in Cancun, Mexico, when blisters developed on the fingers of both her hands. The family returned home early and sought immediate medical attention.

A 4-month-old boy was transferred to our center from a community care hospital because of persistent fever (temperature up to 39.4°C [103°F]) of 5 days’ duration. He also had decreased activity, increased irritability, occasional vomiting after feedings, and a few episodes of loose stool.

Case: The parents of this 5-month-old boy are concerned about recurring eruptions of blisters on the infant’s hands and feet. What question could you ask the parents that might quickly define the condition for you?

Hypertrichosis refers to the increased growth of vellus or other hair at inappropriatelocations beyond the normal variation for a patient’s reference group.1 The affectedareas have a greater number of hair follicles than is normal for the body site.1 The condition is unrelated to androgen excess and unaccompanied by virilism or menstrual abnormalities.

A 17-year-old teenager has developed fuzzy pale rings around several moles over the summer, the moles were present for years,possibly since birth. There is a family history of skin cancer.

The most common chronic medical problem that we pediatricians treat is asthma. We do our best to manage our patients' asthma by prescribing controller medications, providing asthma action plans, and guiding families through acute exacerbations. We often ask about possible environmental triggers, such as tobacco smoke and cockroaches, and we advise patients to reduce their exposure to those triggers.

Measurement of body mass index (BMI) is an effective way to screen for obesity and is an important part of the routine health evaluation of all children. A fasting blood sugar test is recommended for obese children 10 years and older who have a BMI above the 85th percentile for their age and sex and 2 high-risk criteria for diabetes (eg, positive family history or signs of insulin resistance). Patients with a BMI at the 85th percentile or higher also require screening for other comorbidities. Such screening includes measurement of waist circumference, blood pressure, lipid levels (specifically, levels of high- and lowdensity lipoprotein cholesterol and triglycerides, as well as total cholesterol), and liver transaminase levels.

A 13-year-old boy was brought to the emergency department (ED) with a generalized itchy rash of 2 days' duration. For the past 3 days, he had dry, itchy eyes with a purulent discharge (Figure 1) and nonbilious emesis 2 or 3 times per day, with some blood streaks in the vomitus on the third day of illness.

The parents of this 8-year-old girl have brought her for evaluation of areas of loss of pigment on her face. They are worried that these might represent the beginnings of vitiligo.

A previously healthy 15-year-old girl presents with right-sided chest pain that began a few hours earlier during Spanish class. The pain is nonradiating; it is alleviated by leaning forward and exacerbated by sitting still. Shortly after its onset, the patient had taken acetaminophen, which provided temporary relief. However, when she returned home from school, the pain resumed. She denies fever, shortness of breath, nausea, vomiting, and cough.

Four-month-old Hispanic boy brought for evaluation because of hypopigmented patches on his skin and a history of seizures. Infant was the product of an uncomplicated pregnancy; born at 40 weeks' gestation to a 16-year-old gravida 1, para 1 mother who received appropriate prenatal care. At birth, he had multiple hypopigmented patches on his face, torso, and extremities. At age 2 months, he was hospitalized for new-onset seizures.

An afebrile baby presents with disseminated pustules on the trunk, face, and extremities.

A 17-month-old girl awoke with drooling, cough, respiratory distress, and a muffled cry and was brought to the emergency department. She had no nausea, vomiting, or diarrhea and no history of choking, aspiration, or airway problems.

An 8-year-old girl is brought to the emergency department by her mother with a complaint of 5 days of worsening constipation and rectal bleeding. For the past week, the girl has had small stools 3 or 4 times a day and crampy abdominal pain. Yesterday, her stools were streaked with a small amount of blood. The mother notes that her daughter spends up to an hour in the bathroom with each bowel movement. In addition, the mother remarks that the girl has a rash in the rectal area and along the inner thighs.

An 11-year-old girl presented with a swelling on the left side of the chin of 1 month's duration; in the past 24 hours, following a bite by an unidentified insect, the swelling had rapidly enlarged and become painful (A). She was otherwise healthy and had no significant medical or family history.

Numerous brown macules were sparsely distributed over the torso, head, and extremities of an African American newborn. The infant also had a mongolian spot on the buttocks. The baby's mother, a great aunt on the father's side, and the great aunt's daughter and grandson had similar brown macules at birth. All family members were healthy.

A 4-month-old infant is brought in due to raised hyperpigmented lesions around his shins.

For 2 days, a 17-year-old boy had a widespread pruritic eruption that involved the trunk and extremities but spared most of the face. Many of the lesions were annular, and they would appear and resolve within 1 day. The patient denied shortness of breath, difficulty in swallowing, and periorbital swelling.

Practicing pediatricians commonlytalk with patients and parentsabout medical risks. Examples of suchrisks include those of a newborn havinga genetic disease, of a complication of anillness developing, and of a patient experiencingan adverse effect from a medicationor vaccine. Different ways of expressingand communicating risk mayhelp patients and parents understand themagnitude of a risk and make informed,thoughtful decisions about their medicalcare. It is important to be aware of theinfluence personal experience and concernshave on how risk is perceived andto recognize how the choice of a particularway of framing a risk may inadvertentlycommunicate a clinician's personalbiases in a situation.