What is Polymorphous Light Eruption?

Patient Presentation
A 17-year-old female came to clinic because of a pruritic skin rash for 3 days.
She was on a spring break vacation to a beach and noticed the rash on her arms and top of her legs.
She said she felt her skin become ‘tingly’ towards the end of one afternoon that increased in intensity. She noticed that her skin looked liked a sunburn in that it was ‘bumpy’ and seemed to itch more than be painful.
She used some moisturizing lotion on the rash but she said that didn’t seem to make a difference. Over the evening she said that the intensity of the itchiness gradually faded.
In the morning of the second day, the bumpiness was still there and it would itch if she accidentally bumped the areas. When she went outside the itchiness increased almost immediately. She put on long-sleeved shirt and a long pair of shorts that covered the rashes which seemed to help some.
She had been using the same sunscreen during this vacation that she had used previously. She denies any new soaps, lotions, deodorants, clothing, detergents, etc.
The past medical history was non-contributory.
The review of systems was negative including fever, joint complaints, difficulty breathing or swallowing.
The pertinent physical exam showed a well-appearing female with mild sunburn to her face, back of neck and ears. On the extensor surfaces of her forearms, upper arms and top-front of her legs bilaterally, she has an intensely red, blanching rash that has fine confluent papules, some of which look like they may be vesicular.
Palpation causes her to have a mildly painful pruritus.
The diagnosis of polymorphous light eruption was made. The patient was instructed to avoid the sun as much as possible and to use protective clothing and sunscreen. For her comfort she was told that she could use diphenhydramine. She was also told that this could improve within a few days to two weeks but could recur.

Discussion
Photodermatosis is any dermatosis caused by exposure to light. These photodermatoses can be normal responses to excessive sunlight or could be a photosensitivity. Photosensitivity is a general term describing an abnormal reactivity to sunlight by the skin.
Idiopathic photosensitivity reactions include:

  • Actinic Prurigo – is also called hydroa aestivale and Hutchinson’s summer prurigo. It is commonly seen in Indians and mixed ancestry people of Mexico, Central American and South America. Intensely pruritic papules, plaques and nodes occur with excoriations and scars. It generally affects exposed areas but can also be seen in covered areas particularly the lower back and buttocks. It can be seen year round but is worse in the spring and summer with improvement in the fall.
  • Juvenile spring eruption – is a photosensitivity where there are edematous, red papules mainly on the helix of the ears that can become vesicular and crusted. Dorsum of the hands and the trunk can also be involved. It is third more common in boys than girls and usually resolves within 1 week.
  • Polymorphous light eruption – see below
  • Solar urticaria – is a Type 1 IgE-mediated hypersensitivity reaction that causes localized urticarial flare reaction during or within 30 minute of light exposure. The skin returns to normal after a few hours and will not recur within 12-24 hours even with more sunlight exposure. It occurs often in females in the 3rd to 4th decade but can be seen as early as age 2 years.

Learning Point
Polymorphous light eruption is the most common idiopathic photosensitivity reaction. It is sometimes referred to as “sun poisoning” or “sun allergy”. It is most often found in females in the second or third decade of life and occurs in 10-15% of the U.S. population.
An autosomal-dominant form has been described in North and South American natives. It appears to be an immune-mediated, delayed type hypersensitivity reaction to UV rays in the 290-480 nm range papules but its exact etiology is unknown.

The skin is described a having pleo- or poly-morphic lesions that range from small papular, urticarial, vesicular or eczematous lesions to large papules and plaques that can look like erythema multiforme. The lesions generally occur 1-2 days after intense sunlight.
They are located on sun exposed areas such as the face, arms, hands and sides of the neck. The pruritus can be quite severe and the lesions resolve within 1-2 weeks if no additional sunlight exposure occurs. For some people the lesions can occur in the spring and persist, but improve as the skin gets used to the sun exposure over the summer.

The diagnosis is made clinically, but phototesting can be done. With phototesting, the expected reaction occurs in hours, lasts days and is not urticarial. Sometime systemic lupus erythematosus may present with similar lesions and serological testing is necessary.

Images of polymorphic light eruption can be found in the eMedicine and Google Image references below in To Learn More.

Questions for Further Discussion
1. What medications commonly cause photosensitivity?
2. What is phytophotodermatitis?

Related Cases

To Learn More
To view pediatric review articles on this topic from the past year check PubMed.

Information prescriptions for patients can be found at MedlinePlus for this topic: Sun Exposure

To view current news articles on this topic check Google News.

To view images related to this topic check Google Images.

Scheinfeld NS, Shirin S, DelRosario R, Winkielman A. MDPolymorphous Light Eruption. eMedicine.
Available from the Internet at http://www.emedicine.com/derm/topic342.htm (rev. 3/9/06, cited 4/9/07).

Paller AS, Mancini AJ. Hurwitz Clinical Pediatric Dermatology. Elsevier Inc. 2006;505-507.

ACGME Competencies Highlighted by Case

  • Patient Care
    1. When interacting with patients and their families, the health care professional communicates effectively and demonstrates caring and respectful behaviors.
    2. Essential and accurate information about the patients is gathered.
    3. Informed decisions about diagnostic and therapeutic interventions based on patient information and preferences, up-to-date scientific evidence, and clinical judgment is made.
    4. Patient management plans are developed and carried out.
    5. Patients and their families are counseled and educated.

  • Medical Knowledge
    10. An investigatory and analytic thinking approach to the clinical situation is demonstrated.

  • Practice Based Learning and Improvement
    13. Information about other populations of patients, especially the larger population from which this patient is drawn, is obtained and used.

    Author
    Donna M. D’Alessandro, MD
    Associate Professor of Pediatrics, Children’s Hospital of Iowa

    Date
    April 30, 2007

  • Can Lyme Disease be Transmitted by Breast Milk?

    A 9-month-old female came to clinic for her health supervision visit.
    The mother had just been diagnosed with Lyme disease after having fatigue, mild headache, muscle aches, and joint aches that were initially attributed to being a new mother. She did not remember having a rash.
    She had been started on antibiotics a few days ago, and wanted to know if she could pass the infection to her daughter because she was breastfeeding.
    The child herself did not have a history of a tick bite or rash, but did have a couple of colds in the past. She was doing well per her mother.
    The pertinent physical exam showed a smiling, interactive female. Growth parameters were between 5th and 25th percentiles, and her examination was normal.
    The diagnosis of a healthy infant was made. The physician reviewed Lyme disease in the American Academy of Pediatrics RedBook Online® which said that Lyme disease is not transmitted by breast milk.
    The mother was reassured with this information.

    Discussion
    Lyme disease, also referred to as Lyme borreliosis, is caused by the spirochete Borrelia burgdorferi. It is spread by the tick Ixodes scapularis which is about the size of a pencil point.
    In the United States it occurs mainly in the New England states, upper Midwest especially in Wisconsin and Minnesota, and in northern California. Cases occur during warm months (April-October) with ~ 50% of the cases occurring in June and July.
    All ages can be affected but incidence is highest in 5-9 year olds and 45-54 year olds.

    For children, the most common symptoms are erythema migrans, arthritis, facial nerve palsy, aseptic meningitis, and carditis. Symptoms depend on the timing though.

      Early localized disease

      • Erythema migrans at site of lesion – red macule or papule that appears to migrate over time forming a painless, non-pruritic, annular, erythematous lesion sometimes with central clearing.
        The spreading occurs over days to weeks. This can appear 1-55 days after the tick bite, with a median of 11 days.

      • Arthralgia
      • Fever
      • Headache
      • Lymphadenopathy, regional
      • Malaise
      • Neck stiffness, mild
      • Myalgia

        Recommended treatment:
        Doxycycline 100 mg orally, twice a day for 14-21 days is recommended for all children 8 years or older. Alternatively for all ages, Amoxicillin 50 mg/kg/day orally, divided three times per day for 14-21 days (maximum dose is 1.5 g/day)
        or Cefuroxime 30 mg/kg/day orally, divided twice per day for 14-21 days (maximum dose 1000 mg/day) or Cefuroxime 1.0 g/day orally for 14-21 days.

      Early disseminated disease

      • Erythema migrans, multiple lesions
      • Arthralgia
      • Arthritis
      • Carditis
      • Conjunctivitis
      • Cranial nerve palsies, especially cranial nerve VII
      • Encephalitis
      • Fever
      • Headache
      • Lymphadenopathy, regional
      • Meningitis, aseptic
      • Myalgia
      • Neuritis

        In untreated children, 50% will develop arthritis, 10% will develop central nervous system involvement, and 5% will develop cardiac involvement.

        Recommended treatment depends on symptomatology:

      • For multiple erythema migrans, follow early localized disease treatment for 21 days
      • For Isolated facial palsy follow early localized disease treatment for 21-28 days
      • For arthritis, follow early localized disease treatment for 28 days
      • For arthritis that is persistent or recurrent, Ceftriaxone sodium 75-100 mg/kg intravenously or intramuscularly, once per day for 14-28 days (maximum dose 2 g/day)
        or Penicillin 300,000 Units/kg/day intravenously, given in divided doses every 4 hours for 14-28 days (maximum 20 million Units/day)
        or same regimen as early localized disease

      • For carditis, follow persistent or recurrent arthritis treatment
      • For encephalitis or meningitis, follow persistent or recurrent arthritis treatment for 14-28 days

      Late disease – can occur months to years later

      • Arthritis, particularly large joints and is recurrent
      • Peripheral neuropathy

    Treatment usually prevents development of later stages but successfully treated patients may have persistent symptoms for weeks.

    The diagnosis is made by history and physical examination, particularly the characteristic erythema migrans rash.
    A step-wise approach to laboratory testing is recommended. The first step is screening by an enzyme immunoassay or immunofluorescent antibody assay. Positive or equivocal results should then be confirmed by Western immunoblot for IgG and IgM if confirming early disease and for IgG for late disease.
    Other testing can be done but can be complex or is not necessarily better than the step-wise approach.

    The risk of developing Lyme disease after a tick bite even in an endemic area is low enough that prophylactic antibiotics are not indicated for most people.
    Some experts recommend treatment after 72 hours of suspected attachment to the skin by the tick using Doxycycline 200 mg orally once or 4.4 mg/kg for body weight <45 kg in people older than 12 years of age. A vaccine was licensed by the FDA from 1998-2002, but is not currently available.

    Learning Point
    Lyme disease is not transmitted by breast milk. The American Academy of Pediatrics RedBook® states that no causal relationship between maternal Lyme disease and pregnancy complications or congenital abnormalities has been conclusively documented.

    Questions for Further Discussion
    1. What are other tick-borne diseases?
    2. Why is it called Lyme Disease?

    Related Cases

    To Learn More
    To view pediatric review articles on this topic from the past year check PubMed.

    Information prescriptions for patients can be found at MedlinePlus for these topics: Lyme Disease and Tick Bites
    and at Pediatric Common Questions, Quick Answers for this topic: Lyme Disease

    To view current news articles on this topic check Google News.

    DePietropaolo DL,
    Powers JH,
    Gill JM,
    Foy AJ. Diagnosis of lyme disease. Am Fam Physician. 2005;72(2):297-304. Available from the Internet at http://www.aafp.org/afp/20050715/297.html (rev. 7/15/2006, cited 3/8/07).

    American Academy of Pediatrics. Lyme Disease, In Pickering LD, Baker CJ, Long SS, McMillan JA, eds. Red Book: 2006 Report of the Committee on Infectious Diseases. 27th edit. Elk Grove Village, IL: American Academy of Pediatrics; 2006;428-433.

    ACGME Competencies Highlighted by Case

  • Patient Care
    1. When interacting with patients and their families, the health care professional communicates effectively and demonstrates caring and respectful behaviors.
    2. Essential and accurate information about the patients is gathered.
    3. Informed decisions about diagnostic and therapeutic interventions based on patient information and preferences, up-to-date scientific evidence, and clinical judgment is made.
    5. Patients and their families are counseled and educated.
    6. Information technology to support patient care decisions and patient education is used.
    8. Health care services aimed at preventing health problems or maintaining health are provided.

  • Medical Knowledge
    10. An investigatory and analytic thinking approach to the clinical situation is demonstrated.
    11. Basic and clinically supportive sciences appropriate to their discipline are known and applied.

  • Practice Based Learning and Improvement

    15. Information technology to manage information, access on-line medical information and support the healthcare professional’s own education is used.

    Author
    Donna M. D’Alessandro, MD
    Associate Professor of Pediatrics, Children’s Hospital of Iowa

    Date
    April 23, 2007

  • What Are The Physical Examination Signs that May Indicate A Developmental Dysplasia of the Hip?

    Patient Presentation
    A 5-day-old male came to clinic for follow-up after discharge from the newborn nursery. He was a full-term baby whose mother had prenatal care and had an uneventful pregnancy and normal vaginal delivery.
    He was breastfeeding well and his parents had several routine newborn infant questions.
    The pertinent physical exam showed an alert, non-dysmorphic male with a weight of 3290 grams (50% – down 130 grams since birth), length of 48 cm (25%), and head circumference of 37 cm (90%).
    On his examination he had a dislocatable left hip. The right hip was normal. The rest of his physical examination was normal.
    The patient was referred to orthopaedics who confirmed the diagnosis of developmental dysplasia of the hips and placed him in a Pavlik harness to be taken off only when bathing.
    The patient’s clinical course showed that at 5 weeks of age, he was re-evaluated and still had a dislocatable hip.
    The radiologic evaluation at that time showed the left hip to be fairly well located when the patient wore the harness but there was still dysplasia of the left hip. It was decided to keep him in the harness constantly for the next 2 weeks and to re-evaluate him.
    At 7 weeks of age, his hip was stable but repeated ultrasound showed continued dysplasia of the left hip. At 3 months of age his hip was completely stable on physical examination and was minimally dysplastic on ultrasound examination also.
    The harness was discontinued and at 6 months of age he had a normal examination and was discharged from the orthopaedic service.


    Figure 48 – Coronal ultrasound images of the left hip (left-sided image) and right hip (right-sided image) were obtained after 5 weeks of therapy in a Pavlik harness with the patient located in the Pavlik harness during the exam. The coronal view is meant to simulate an anterior-posterior radiograph of the hip. The images show the left hip to still be dysplastic with there still being a shallow acetabulum and decreased alpha angle even though the left hip appears fairly well located in the left acetabulum. The left hip alpha angle measured 45 degrees (normal is usually greater than 60 degrees). The right hip was normal in appearance.

    Discussion
    Developmental dysplasia of the hip (DDH) is a group of abnormalities regarding the improper alignment and growth of the femoral head and acetabulum.
    Without tight alignment of the femoral head and acetabulum, the hip joint may grow abnormally.
    It was previously called congenital dysplasia of the hip (CDH)but it has become clear that some children have normal hips at birth but are later found to have dysplasia.
    Therefore DDH is the preferred term.

    Most patients have no risk factors, but risk factors include female gender, family history of DDH, in utero postural deformities and breech positioning.
    DDH can lead to pain, difficulty ambulating and degenerative joint disease including potentially avascular necrosis of the femoral head and growth arrest.
    DDH is usually unilateral (75%).

    Recently the U.S. Preventive Services Task Force (USPSTF) concluded that there is insufficient evidence to recommend routine screening for DDH to prevent adverse outcomes.
    Evidence shows that screening leads to earlier identification of DDH, but that there is a high rate of spontaneous resolution (60 – 80 % of the newborn hips identified by physical examination and > 90% identified as suspicious or abnormal by ultrasonography).
    The American Academy of Pediatrics recommends serial physical examination of the hips but not general ultrasound imaging of the hips. It also recommends ultrasound imaging of all female infants born in the breech position or with a family history of DDH and optional imaging of male infants born in the breech position.

    As the femoral heads do not ossify until 4-6 months of age, plain radiographs are not useful in newborns. Hip ultrasonography can image the cartilagenous structures of the hip and therefore is the modality of choice for hip imaging in infants < 6 months.

    Children are often treated non-surgically with devices that flex and abduct the hips into position. One commonly used method is the Pavlik harness which looks similar to a marionette puppet. The infant has straps around the chest and over the shoulders for support. The infant also has straps on the calves. The chest/shoulder and calve straps are connected by ‘strings’ (usually made out of the same strap-like material) which holds the hips in a flexed and abducted position.
    Long-term Pavlik harness treatment results (e.g., nonoperative treatment) show a 95 percent success rate for acetabular dysplasia and subluxation, but the rate decreases to 80% for frank dislocation.
    Surgical treatment is used for unsuccessful non-surgical treatment, severe disease or some patients diagnosed late.

    Learning Point
    Physical examination signs to look for DDH in an infant include:

    • Beginning position: Patient lying supine with both hips flexed to 90° and holding flexed knees in the palm of the hands with thumbs along medial aspect of thigh and fingers along lateral aspect of thigh.
    • Barlow maneuver – gentle force on the femur posteriorly to try to dislocate the hip posteriorly. If positive, the femoral head can be felt to clunk or slip out of position. Some people remember this maneuver as “Barlow = Back” or pushing the femoral head back.
    • Ortolani maneuver – gentle abduction and lifting of the femur. If positive, the femoral head can be felt to clunk or slip over the posterior rim of the acetabulum back into position. Some people remember the maneuver as “Ortolani = Out” or replacing the “out” femoral head.
    • Other physical examination signs that may indicate a possible DDH are:
      • Galeazzi or Allis sign – heights of the knees are uneven when bilateral hips and knees are flexed with the feet flat on the table. The shorter side is the affected side because the shorter femoral segment.
      • Posterior leg creases – the creases of the buttocks, femur and knees are assymmetric because of the shorter femoral segment on one side.

    Older children can present with gait abnormalities and decreased hip abduction.

    Questions for Further Discussion
    1. What surgical operations are performed for DDH?
    2. What congenital syndromes are associated with DDH?

    Related Cases

    To Learn More
    To view pediatric review articles on this topic from the past year check PubMed.

    Information prescriptions for patients can be found at MedlinePlus for this topic: Hips Injuries and Disorders
    and at Pediatric Common Questions, Quick Answers for this topic: Developmental Dysplasia of the Hips

    To view current news articles on this topic check Google News.

    Mier RJ, Brower TD. Pediatric Orthopedics A Guide for the Primary Care Physician. Plenum Publishing Co. New York, NY. 1994;21-25.

    American Academy of Pediatrics. Clinical practice guideline: early detection of developmental dysplasia of the hip. Pediatrics. 2000;105:896-905. Available from the Internet at: http://aappolicy.aappublications.org/cgi/content/full/pediatrics;105/4/896(rev. 4/2000, cited 3/5/07).

    U.S. Preventive Services Task Force Recommendation Statement. Screening for Developmental Dysplasia of the Hip. Available from the Internet at http://www.ahrq.gov/clinic/uspstf06/hipdysp/hipdysrs.htm (rev. 3/2006, cited 3/5/07)

    Storer SK, Skaggs DL. Developmental dysplasia of the hip. Am Fam Physician. 2006 Oct 15;74(8):1284-5.

    ACGME Competencies Highlighted by Case

  • Patient Care
    1. When interacting with patients and their families, the health care professional communicates effectively and demonstrates caring and respectful behaviors.
    2. Essential and accurate information about the patients is gathered.
    3. Informed decisions about diagnostic and therapeutic interventions based on patient information and preferences, up-to-date scientific evidence, and clinical judgment is made.
    4. Patient management plans are developed and carried out.
    7. All medical and invasive procedures considered essential for the area of practice are competency performed.
    8. Health care services aimed at preventing health problems or maintaining health are provided.
    9. Patient-focused care is provided by working with health care professionals, including those from other disciplines.

  • Medical Knowledge
    10. An investigatory and analytic thinking approach to the clinical situation is demonstrated.
    11. Basic and clinically supportive sciences appropriate to their discipline are known and applied.

  • Systems Based Practice
    24. Cost-effective health care and resource allocation that does not compromise quality of care is practiced.
    26. Partnering with health care managers and health care providers to assess, coordinate, and improve health care and how these activities can affect system performance are known.

    Author
    Donna M. D’Alessandro, MD
    Associate Professor of Pediatrics, Children’s Hospital of Iowa

    Date
    April 16, 2007