How are Personality Disorders Clustered?

Patient Presentation
A 15-year-old male came to clinic for his health supervision visit. He was known to be a high achiever in school and his mother expressed pride in his academic accomplishments. However, since starting 10th grade, he had stopped his sports and music activities stating he wasn’t as good at them, and wanted to “concentrate on what I’m good at.” He had continued math team and an academic knowledge team. His mother said that he was spending more and more time by himself everyday studying for math and knowledge competitions. “And I have to do that so I can win them. Remember last time I was only 5th place,” he interrupted. “But you’re also not sleeping or eating well and I’m worried about you,” she countered.

The past medical history showed that he was identified for the school’s gifted and talented program in first grade and had been involved with competitive soccer, swimming and had been taking private music lessons since age 5. These activities had stopped over the past year.

The pertinent physical exam showed a thin male with normal vital signs and his weight was 50% and tracking for age. He examination was normal and he had Tanner IV genitalia. The diagnosis of of a teen with concerning behaviors was made.

In a private interview with the adolescent, he acknowledged that he had stopped the activities because he felt he “had to get perfect grades,” and “had to win the competitions.” He would go to bed at night but then set an alarm to wake up in the middle of the night to do more studying and math problems or general knowledge quizzes. His friend group had diminished to only a few people but he interacted with them only at club meetings and would be studying during lunchtime at school. He said it really bothered him when he got any homework or math problems wrong. He talked about his study schedule and how he had it organized to the minute using a calendar tool and timer on his phone. He organized his books and pens/pencils on his desk before starting his work and would re-organize them several times while working. “If I don’t organize it, it really bothers me and I can’t get anything done until I get my highlighters and pens in order and check the timer,” he noted. The teen said that he did feel some pressure from his parents to perform, but most of what he did, he felt he had to do because of himself. The pediatrician was concerned about his perfectionistic tendencies along with what might be compulsions with his schedule and tools. He also seemed to be becoming more obsessed with his homework and math competitions and was becoming more isolated. The pediatrician was concerned for a possible obsessive-compulsive disorder. The teen agreed that he was having more anxiety if he couldn’t adhere to his schedule and therefore he was becoming less efficient. He also agreed that he missed spending time with some of his friends. After discussion with the parent and patient, the teen agreed to see a counselor who could help him to continue to achieve his goals but to help him learn to do it in a way that would let him see his friends, and do other activities he might want to do. At 6 month follow up, he was seeing a therapist but was still struggling.

Discussion
Personality traits (PT) represent patterns of thinking, perceiving, reacting, and relating that are relatively stable over time. There are 5 major PT:

  • Extraversion (e.g. tendency to be sociable)
  • Neuroticism (e.g. susceptibility to negative thoughts and distress)
  • Conscientiousness (e.g. self-regulation and being able to look at long term goals)
  • Agreeableness (e.g. self-regulations and relationship maintenance)
  • Openness to experience (e.g. imaginative, creative, curiosity).

Personality disorders (PD) occur when these personality traits become so conspicuous, rigid and maladaptive that they cause impairment in life and work. PDs are “enduring patterns of inner experience and behavior that deviates markedly from the expectations of the individual’s culture, with onset in early adulthood, leading to pervasive, stable and inflexible behavior over time, and causing stress or impairment.” PDs classification is evolving and the DSM-V currently has 10 different ones.

PDs have a prevalence in the US of ~9% in adults. They are highly co-morbid conditions (~40-80%) with other mental illness especially with major depressive disorder. People with PDs have more social dysfunction and an increased risk of disability including risky sexual behavior, violence, suicide and problems in school, work and relationships.

Diagnosis includes that the traits are persistent, maladaptive and involve at least two areas of functioning including affectivity, cognition, impulse control, and interpersonal functioning. There must also be significant impaired functioning or distress caused by the maladaptive traits, and the traits must be relatively stable with onset at least by adolescence or early adulthood. PDs are generally not diagnosed in children and youth < 18 years but can be if present for more than 1 year.
An adolescent study found that PD prevalence is estimated at 6-17% with median of 11%. PT do vary over time and are considered moderately stable by adolescence. The stability of PD diagnosis in adolescence though is modest. However the more persistent the PD symptoms, the more likely the patient is to have impairment in adulthood.

Learning Point
PDs are grouped based on similar characteristics but if these clusters are clinically useful has not been established. While patients should be considered as individuals, an aide-memoire for remembering the clusters is: Mad, Bad, Sad.

  • Cluster A are PDs where the patients are considered eccentric or odd.
    • Paranoid – distrusts and is suspicious, intentions of others are interpreted as malevolent
    • Schizoid – disinterested in other people, has perceptual or cognitive distortions
    • Schizotypal – has eccentric behavior and ideas, social relationships are detached and have limited range of emotions
  • Cluster B are PDs where patients are characteristically emotional, dramatic or erratic. Is characterized by appearing dramatic, emotional, or erratic.
    • Antisocial – has marked disregard for others, is socially irresponsible, will manipulate and be deceitful of others for personal gain
    • Borderline – has intolerance of being alone and has emotional dysregulation, marked impulsivity, unstable self image and relationships
    • Histrionic – seeks attention
    • Narcissistic – here is overt grandiosity with a fragile self-esteem and/or underlying dysregulation, lacks empathy, needs admiration
  • Cluster C are PDs where the person is anxious or fearful.
    • Avoidant – very sensitive to rejection so will avoid interpersonal contact, feels inadequate, socially inhibited
    • Dependent – is submissive, and needs others to take care of them, clingy behaviors
    • Obsessive-compulsive – can be rigid, have obstinacy and perfectionism, must have order and control

In a study of adults within a primary care setting, the most common PDs in order were borderline PD, dependent PD, obsessive-compulsive PD, and unspecified PD.

Questions for Further Discussion
1. What are psychoses and how common are they in the pediatric population?
2. What are the diagnostic criteria for different depressive illnesses?
3. How are personality disorders treated?

Related Cases

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

Evidence-based medicine information on this topic can be found at SearchingPediatrics.com and the Cochrane Database of Systematic Reviews.

Information prescriptions for patients can be found at MedlinePlus for these topics: Personality Disorder and Topics.

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

To view images related to this topic check Google Images.

To view videos related to this topic check YouTube Videos.

Shiner RL. The development of personality disorders: Perspectives from normal personality development in childhood and adolescence. Development and Psychopathology. 2009;21(3):715-734. doi:10.1017/S0954579409000406

American Psychiatric Association. Diagnostic and Statistical Manual of Mental Disorders. Fifth Edition (DMS-5). Washington, DC: American Psychaitric Association; 2013.

Angstman KB, Seshadri A, Marcelin A, Gonzalez CA, Garrison GM, Allen J-S. Personality Disorders in Primary Care: Impact on Depression Outcomes Within Collaborative Care. J Prim Care Community Health. 2017;8(4):233-238. doi:10.1177/2150131917714929

Bach B, Sellbom M, Skjernov M, Simonsen E. ICD-11 and DSM-5 personality trait domains capture categorical personality disorders: Finding a common ground. Aust N Z J Psychiatry. 2018;52(5):425-434. doi:10.1177/0004867417727867

Overview of Personality Disorders – Psychiatric Disorders. Merck Manuals Professional Edition. Accessed July 14, 2020. https://www.merckmanuals.com/professional/psychiatric-disorders/personality-disorders/overview-of-personality-disorders

Author
Donna M. D’Alessandro, MD
Professor of Pediatrics, University of Iowa

What are Potential Complications of Ichthyosis?

Patient Presentation
A 2-month-old male came to clinic for his health maintenance examination. The family needed some help with home health supplies and identifying some respite care. The past medical history was significant for epidermolytic ichthyosis diagnosed at birth but suspected as the father also had this problem. In the intensive care setting, he had blistering in various areas along with erythema and hyperkeratotic areas. He had some difficulty with feeding and was having problems with consistent weight gain and needed a high-caloric feeding regimen. He also had some problems with thermoregulation in the first few days of life and required 2 courses of antibiotics because of possible sepsis. He was tapering off pain medications.

The pertinent physical exam showed a fussy infant with copious amounts of lubricant over his body. His weight was 4453g (5th %) and he was gaining 12 grams/day. He would calm more when not handled, would look around and seem curious. His skin showed a few scattered open blisters with healthy tissue that was not infected among the plaque-like scales. He had no contractures. The rest of his examination was normal.

The diagnosis of epidermolytic ichthyosis with lower weight gain was made. The pediatrician reviewed the general home going plan with the family which included copious amounts of petroleum jelly to the skin and watching it closely. They were limiting his handling, including feeding him while he was propped up. They were monitoring him for potential infection and knew when to call dermatology for more help. He had a followup appointment with palliative care to continue to wean his pain medication. Infectious disease consultation had agreed that he should receive his regular vaccines at this time. The pediatrician offered to have his social worker meet with the family to discuss the supplies and respite care issues. “I know that palliative care can also help you with these problems, but my social worker can also help with coordinating your appointments and other help you may need in the future,” he stated. The mother stated that she would be very grateful for the help as a lot of his daily care was overwhelming her.

The patient’s clinical course revealed that over the next 16 months, the blistering resolved. He still had a lot of hyperkeratotic scale. As he got older and the blistering improved, his need for pain medication decreased and his general disposition improved, although he still had problems with sleep. He received live-virus vaccines at appropriate intervals as he did not have open skin during those visits.

Discussion
Ichthyosis is a large group of dermatological disorders which are caused by abnormal epidermal differentiation. The term ichthys means fish and refers to the scale-like dermatological pattern seen in these disorders.

Acquired ichthyosis is rare but more common in adults than children and has a rough, dry skin with prominent scaling, distributed symmetrically on the trunk and limbs (especially extensor surfaces), and is associated with autoimmune, infectious diseases, metabolic and malignant causes. Congenital or inherited ichthyosis is divided into non-syndromic (more common) and syndromic forms (generally very rare, e.g. Netherton syndrome or Sjogren-Larsson syndrome, Refsum syndrome, etc.). Specific diagnosis in the newborn period is not necessarily helpful, but with aggressive support, over time the specific clinical entity often becomes apparent. Treatment is also similar.

Some examples include:

  • Ichthyosis vulgaris
    • Most common and mildest form
    • “…xerosis, scaling, pruritis and eczema, and it is strongly associated with atopic manifestations.” Back and lower legs are more affected.
    • Starts after 2 months of age, better in the summer
  • Recessive X-linked ichthyosis
    • Second most common
    • Dark brown scale on entire body
    • Starts around birth and does not improve with age
  • Harlequin ichthyosis
    • Autosomal recessive due to ABCA12 gene, very rare
    • Has thick, armor or plate-like scales which can cause contractures
    • Babies are often born premature
  • Collodion baby
    • Autosomal recessive – various genes
    • This includes several forms but has “…a shiny, taut, cellophane-like membrane”. The membrane is shed over time and the clinical manifestations of the specific entity become more apparent.
    • Babies are often born premature
  • Epidermolytic ichthyosis
    • Keratinopathic ichthyosis that is autosomal dominant due to KRT1 or KRT10 genes
    • Presents at birth with blistered or denuded skin but usually this diminishes and hyperkeratotic skin becomes more prevalent. The hyperkeratosis continues into adulthood.

Learning Point
The skin is a very important organ system, therefore complications of ichthyosis are because of the disruption of these vital functions:

  • Fluid and electrolyte balance maintenance
    • Ichthyosis has increased risk of water loss, dehydration and electrolyte imbalance
    • Treatment includes close monitoring of weight, input/outputs, electrolytes and replacement with fluids and electrolytes as appropriate.
      Nutrition consultation may be necessary because of increased metabolic needs
  • Barrier maintenance
    • Ichthyosis causes loss of barrier integrity (e.g. fissuring and denudation) including risk of infection and potential for absorption of substances, and can affect thermoregulation.
    • Treatment includes monitoring for infection and aggressive treatment for suspected infection, use of emollients to prevent barrier break down, minimal handling of patient/area, avoiding tape and using gauze or self-adherent wraps, avoiding topical substances which could lead to absorption to potentially toxic levels (e.g. silver sulfadiazine, salicylic acid, lactic acid, etc.), isolation of patient, and monitoring of temperature regulation and pain management
  • Movement
    • Ichthyosis can cause contractures of various body parts which can restrict chest wall/lung movement, sucking and feeding, joint contractures and compartment syndrome
    • Treatment includes emollients to promote resolution or improvement and flexibility of the skin, respiratory support, nutritional support including nasogastric feedings, consultation with appropriate surgical services such as orthopaedics or ophthalmology (contractures can cause ectropion of the eyes) and otolaryngology/dental (for contractures of the mouth or other head and neck organs).

Questions for Further Discussion
1. What else can be considered in the differential diagnosis of blistering skin lesions at birth?
2. What are indications for a dermatology consultation? When would it be considered an emergency?

Related Cases

    Symptom/Presentation: Rash

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

Evidence-based medicine information on this topic can be found at SearchingPediatrics.com and the Cochrane Database of Systematic Reviews.

Information prescriptions for patients can be found at MedlinePlus for this topic: Skin Conditions

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

To view images related to this topic check Google Images.

To view videos related to this topic check YouTube Videos.

Craiglow BG. Ichthyosis in the Newborn. Semin Perinatol. 2013;37(1):26-31. doi:10.1053/j.semperi.2012.11.001

Takeichi T, Akiyama M. Inherited ichthyosis: Non-syndromic forms. The Journal of Dermatology. 2016;43(3):242-251. doi:10.1111/1346-8138.13243

Yoneda K. Inherited ichthyosis: Syndromic forms. The Journal of Dermatology. 2016;43(3):252-263. doi:10.1111/1346-8138.13284

Glick JB, Craiglow BG, Choate KA, et al. Improved Management of Harlequin Ichthyosis With Advances in Neonatal Intensive Care. Pediatrics. 2017;139(1). doi:10.1542/peds.2016-1003

Author
Donna M. D’Alessandro, MD
Professor of Pediatrics, University of Iowa

What Are the Symptoms of Herpes Simplex Keratitis?

Patient Presentation
A 6-year-old female came to clinic for her well child examination. She had recently moved to the area and her mother was concerned because of a “spot” on her eyelid. She had been diagnosed with herpes simplex of the right upper eyelid about 1 year previously. Her mother described it as a vesicular rash that was very painful and she took an oral medication for it. It resolved but the mother was concerned about hyperpigmentation on the right eyelid. She had not seen an eye doctor and had been well except for a left forearm fracture from falling off playground equipment which had resolved with casting. The review of systems was non-contributory.

The pertinent physical exam showed a healthy female with normal vital signs and growth parameters were in the 50-90%. HEENT showed no obvious hyperpigmentation but what might have been a tiny area where the mother pointed it out on the right upper eyelid. Visual acuity was 20/20-30 for both eyes and her visual examination was normal as was the rest of her physical examination.

The diagnosis of a healthy child with a history of herpes of the right upper eyelid. As there was no history of ocular involvement and it had been around a year since the original occurrence, the pediatrician only counseled the mother to monitor closely for recurrences including pain, tingling etc. of the eyelid, any visual problems or any concerns about the eyes. She said, “herpes infections are really common and can be hard to diagnose, but they can also cause real problems in the eyes if not treated right away or properly. Herpes tends to happen again especially in children and therefore if something seems to be happening with her eyes, I want to know about it so I can get you some help.”

Discussion
Herpes simplex virus (HSV) infections are common with an estimated 50% of the US population being infected by age 30, and with latent infection harboring in the trigeminal nerve in 100% of people by age 60 years. HSV infections can cause a vesicular or pustular skin rash that is painful, burning or pruritic and also flu-like symptoms with fever, chills, headache, and fatigue. HSV can also be asymptomatic.

To laymen, herpes simplex viruses cause “cold sores,” but to health care personnel, herpes causes many systemic infections including eczema herpeticum, folliculitis, herpes gladiatorum, whitlow, encephalitis and ocular HSV. Ocular HSV has many forms including primary or recurrent disease and involvement of all ocular tissues including “…blepharitis, conjunctivitis, epithelial keratitis, stomal keratitis, endothelialitis, iritis, trabeculitis, and retinitis.”

Ocular HSV infection is usually caused by HSV-1 but HSV-2 can cause keratitis. Neonatal primary HSV keratitis is caused by HSV-2 because of antenatal, intrapartum and postpartum exposure. HSV-2 seroprevalence in pregnancy is estimated to be 20-30%. Ocular HSV infections can cause significant complications including being the most common cause of corneal blindness in developed countries, and causing some form of visual disability in 1 million people/year globally. Children have worse outcomes with ocular HSV than adults “…includ[ing] recurrence (50%), corneal scarring (80%) and corneal neovascularization (>30%).” Risk factors for recurrence include trauma, eye surgery, atopic dermatitis, diabetes, and immune compromised status.

Learning Point
HSV is primarily a clinical diagnosis, but viral culture (gold standard) and polymerase chain reaction methods can detect HSV when there is active viral replication. Symptoms of HSV keratitis include burning, tearing, swollen eyelids, decreased visual acuity, and photophobia. However, a significant number of primary infections may not cause symptoms (up to 2/3rds) or not be recognized/reported by the children or adult caregivers. It can also be easily misdiagnosed. Therefore patients with potential HSV infection near the eyes usually are discussed or evaluated with ophthalmology.

Complications are caused by viral infectivity and activity and the immunological response of the host. Therefore treatment is both antiviral and steroid medications. Medications are systemic and/or topical. Antiviral medications current including acyclovir, valacylovir and ganciclovir. Patients are often started on prophylatic antiviral medication and monitored for recurrence. If there is no recurrence after a period of time (~1 year) then prophylaxis is stopped and patients continue to be monitored. For recurrence, patients are treated again with antiviral and/or steroids. Antiviral resistance has been documented particularly in patients with severe disease and taking topical steroids.

Questions for Further Discussion
1. What are the differences between herpetic whitlow and acute paronychia? A review can be found here
2. How are neonates evaluated and treated for potential HSV encephalitis?
3. How are outbreaks of herpes gladitorum contained?

Related Cases

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

Evidence-based medicine information on this topic can be found at SearchingPediatrics.com and the Cochrane Database of Systematic Reviews.

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

To view images related to this topic check Google Images.

To view videos related to this topic check YouTube Videos.

Tsatsos M, MacGregor C, Athanasiadis I, Moschos MM, Hossain P, Anderson D. Herpes simplex virus keratitis: an update of the pathogenesis and current treatment with oral and topical antiviral agents. Clin Experiment Ophthalmol. 2016;44(9):824-837. doi:10.1111/ceo.12785

Pinninti SG, Kimberlin DW. Neonatal herpes simplex virus infections. Semin Perinatol. 2018;42(3):168-175. doi:10.1053/j.semperi.2018.02.004

Vadoothker S, Andrews L, Jeng BH, Levin MR. Management of Herpes Simplex Virus Keratitis in the Pediatric Population. Pediatr Infect Dis J. 2018;37(9):949-951. doi:10.1097/INF.0000000000002114

Valerio GS, Lin CC. Ocular manifestations of herpes simplex virus. Curr Opin Ophthalmol. 2019;30(6):525-531. doi:10.1097/ICU.0000000000000618

Author
Donna M. D’Alessandro, MD
Professor of Pediatrics, University of Iowa

What Are the Clinical Signs and Symptoms of Congenital Syphilis?

Patient Presentation
A 5-day-old male came to clinic for his well-child examination. He was taking formula every 2-3 hours and his mother complained that she was quite tired from feeding him. The past medical history showed his 19-year-old G3P1 mother had received prenatal care but was living in transitional housing. She had been working until his birth at a restaurant. She had been positive on several occasions for sexually transmitted infections including syphilis before conception and had been treated. During pregnancy she was treated at his first appointment for chlamydia and tested negative for other sexually transmitted infections including syphilis and HIV. She had been re-screened at different points during pregnancy and was negative.

The patient’s clinical course showed that he had no complications and a normal physical examination. Neonatology had been consulted and as the mother had been adequately treated before and during pregnancy, no specific evaluation or treatment for any sexually transmitted disease was recommended.

The pertinent physical exam showed a healthy male who was down 3% from his birth weight. Growth parameters were around the 25th percentile.

The diagnosis of a healthy male was made. The mother had been offered social work support during their admission and asked for additional help with transportation and food programs. The resident and the staff physician both discussed that they knew that the incidence of syphilis was increasing but had not treated anyone for it in a long time.

The patient’s clinical course showed that several health supervision appointments were missed or rescheduled, however by his 12 month appointment he had received all of his vaccines, they were now living a shared apartment, and his mother had two steady part-time jobs.

Discussion
Syphilis is caused by the spirochete Treponema pallidum. It is a very old disease that despite understanding the organism and readily available treatment, still causes disease. Syphilis is transmitted sexually. According to the Centers for Disease Control, “[i]n 2018, a total of 35,063 cases of [Primary and Secondary] syphilis were reported in the United States, yielding a rate of 10.8 cases per 100,000 population …. This rate represents a 14.9% increase compared with 2017 (9.4 cases per 100,000 population), and a 71.4% increase compared with 2014 (6.3 cases per 100,000 population).” The increase is associated with an increase in men, particularly men who have sex with men. Syphilis is also considered a risk factor for Human Immunodeficiency Virus (HIV).

Syphilis can also be transmitted to the fetus because of the maternal spirochetemia and transplacental transmission resulting in congenital syphilis if not treated. Transmission increases with the advancing pregnancy but can occur at any time. Fetuses and infants can be affected from birth or years later. “After decreasing from 10.5 to 8.4 reported congenital syphilis cases per 100,000 live births during 2008-2012, the rate of reported congenital syphilis has subsequently increased each year since 2012. In 2018, there were a total of 1,306 reported cases of congenital syphilis, including 78 syphilitic stillbirths and 16 infant deaths, and a national rate of 33.1 cases per 100,000 live births. This rate represents a 39.7% increase relative to 2017 (23.7 cases per 100,000 live births) and a 185.3% increase relative to 2014 (11.6 cases per 100,000 live births).”

Evaluation includes a detailed history and physical examination. Early congenital syphilis is also associated with anemia, thrombocytopenia, abnormal liver enzymes, and cerebrospinal fluid with pleocytosis and elevated protein. The diagnosis is made by detection of spirochetes or antibodies in body tissues or fluids. Two commonly used serological tests are rapid plasma reagin (RPR) and Venereal Disease Research Laboratory tests (VDRL). Prevention is key so all pregnant women are tested at their first prenatal appointment in the first trimester and again later in pregnancy (28-32 week gestation) if in high risk areas. Women who are positive for syphilis and their sexual partners should be screened for HIV as well and appropriately managed and treated including their newborns. Treatment for syphilis is penicillin, but the timing and length of treatment varies by clinical status including age and symptoms. Normal infants of women adequately treated before pregnancy do not need evaluation or treatment usually. Normal infants of women adequately treated during pregnancy and at least 4 weeks before delivery are not evaluated but are treated with one dose of penicillin. Infants of women with shorter durations of treatment or with abnormal physical examinations require more evaluation and longer durations of treatment.

Adolescents with sexually transmitted infections should be screened for syphilis as multi-organism infections are common. Syphilis has 3 stages. Primary syphilis has characteristic painless chancres at the site of primary inoculation. Secondary syphilis has symptoms similar to early congenital syphilis but especially a polymorphous rash on palms and soles, lymphadenopathy and fever. If untreated syphilis enters a latent stage and is not contagious during this time. Tertiary syphilis occurs 10-30 years after primary infection and classically has granulomatous skin growths and cardiac and neurological problems. Neurosyphilis can occur at any stage of symptomatic infection.

Learning Point
Clinical signs and symptoms of congenital syphilis include:

  • Fetal affects
    • Abortion, spontaneous
    • Still birth
    • Preterm delivery
    • Hydrops (non-immune)
    • Perinatal death
  • Early congenital syphilis or infants and children affected before 2 years of age
    • Adenopathy – especially epitrochlear nodes that are palpable
    • Dermatological findings
      • Rash – usually maculopapular and oval, it is more prominent on the palms and soles and becomes more copper- colored and has desquamation.
      • Pemphigus syphiliticus has bullous lesions that may cause cracking and peeling and wrinkling.
      • Oral mucus patches
      • Condylomata lata
    • Hepatomegaly – can occur with or without jaundice
    • Splenomegaly
    • Neurological findings – asymptomatic, cranial nerve palsies, seizures
    • Ophthalmological findings – cataract, chorioretinitis
    • Orthopaedic findings – osterochronditis, periostitis
    • Pneumonia alba
    • Rhinitis (‘snuffles”)
    • Other
      • Fever
      • Hypopituitarism and diabetes insipidus
      • Malabsorption in the gastrointestinal tract
      • Myocarditis
      • Nephrotic syndrome
      • Pancreatitis
  • Late congenital syphilis or children affected after 2 years of age
    • Dental findings – Hutchinson’s teeth*, mulberry molars
    • Rhagades – cracks in the skin particularly edges of the mouth
    • Neurological findings – cranial nerve palsies, deafness*, optic nerve atrophy, general paresis, hydrocephalus, mental retardation, seizures
    • Ophthalmological findings – healed chorioretinitis, interstitial keratitis*
    • Orthopaedic findings – facial changes including frontal bossing, saddle nose deformity, high arch palate, protuberant mandible, short maxilla, saber shins and abnormal joints

*Hutchinson’s triad of late congenital syphilis is Hutchinson’s teeth, deafness and interstital keratitis.

Questions for Further Discussion
1. Name the TORCH infections?
2. What are the most common sexually transmitted infections in your location? How common is syphilis?
3. What services are available in your community for teenage mothers?

Related Cases

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

Evidence-based medicine information on this topic can be found at SearchingPediatrics.com and the Cochrane Database of Systematic Reviews.

Information prescriptions for patients can be found at MedlinePlus for these topics: Syphilis and Infections and Pregnancy.

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

To view images related to this topic check Google Images.

To view videos related to this topic check YouTube Videos.

Cooper JM, Sanchez PJ. Congenital syphilis. Semin Perinatol. 2018;42(3):176-184. doi:10.1053/j.semperi.2018.02.005

Heston S, Arnold S. Syphilis in Children. Infect Dis Clin North Am. 2018;32(1):129-144. doi:10.1016/j.idc.2017.11.007

Syphilis – 2018 Sexually Transmitted Diseases Surveillance. Published October 8, 2019. Accessed June 16, 2020. https://www.cdc.gov/std/stats18/syphilis.htm

Arrieta AC, Singh J. Congenital Syphilis. New England Journal of Medicine. Published online November 27, 2019. doi:10.1056/NEJMicm1904420

Author
Donna M. D’Alessandro, MD
Professor of Pediatrics, University of Iowa