What is DRESS Syndrome?

DRESS (drug reaction with eosinophilia and systemic symptoms) is a poorly understood syndrome that has an unknown prevalence in the pediatric population because it is often missed by doctors. 

Symptoms include fever, an itchy rash covering over half the body, enlarged lymph nodes, and inflammation of the liver. A blood test reveals increased white blood cells, "atypical lymphocytes" (abnormally large and irregular lymphocytes because they are infected with the virus), and elevated eosinophils. 

What causes it? There have been links to both herpesvirus (including oral and genital herpes, epstein barr virus and roseola virus) and a couple months after starting certain anti-seizure medications. Bad cases will involve >90% of the skin and affect multiple organs such as the kidneys, and there may be involvement of the mucosal lining of the mouth and lips which can mimic life threatening drug and infectious reactions known as Stevens Johnson Syndrome and Toxic Epidermal Necrolysis.

It is very difficult to avoid getting this syndrome, as it is unknown why some patients get it and others do not. It can take months to fully recover from DRESS syndrome. The treatment, if caused by a medication, is to stop that medication. Most patients get IV or topical steroids to calm the immune system. 

Most often, pediatric rashes are harmless. In rare cases such as this, however, some detective work is required to make a swift and proper diagnosis.

A Swollen Pediatric Joint

A 6 year old girl with a swollen right knee in the absence of trauma, fever, rash or lymphadenopathy. Symptoms are worse in the early morning, and improve throughout the day. She also complains of eye pain. What is the diagnosis?

Juvenile Idiopathic Arthritis (oligoarticular type)


Arthritis in children is not uncommon. When encountered by pediatricians, it is characterized into one of three subtypes: systemic, oligoarticular, and polyarticular.

• The oligoarticular type is the most common. Usually occuring around 5 years old, it is seen in the large joints (but not the hips) and is known for it's casual but unique association with uveitis (irritation of the eye) and a positive ANA (an antibody in the blood). If left untreated, this uveitis can lead to glaucoma when they are older.

• The polyarticular type is the second most common, and usually affects the large and small joints on both sides symmetrically. Older children with bloodwork that is rheumatoid factor positive are likely to progress to arthritis as adults.

• The systemic type is often easiest to recognize, as an adolescent will often present with fever, a salmon-colored rash, and lymphadenopathy. Many different joints can be affected, and treatment requires disease-modifying drugs (DMARDs). Untreated systemic JIA can progress to a dangerous condition known as macrophage activating syndrome. 

The disease is manageable when caught early, and is typically managed by a specialist known as a rheumatologist. An early step is be sure the swelling isn't the result of an athletic injury or fall. The pediatrician will be sure to rule out more serious causes such as a septic (infected) joint.

An Update on Cat Scratch Fever

Cat Scratch Disease, also known as Cat Scratch Fever, is found in more locations than just a Ted Nugent album. This disease, first isolated in 1992, is caused by a bacteria called Bartonella henselae and affects roughly 6.6 in every 100,000 children in the United States. It's mostly benign (harmless), and in some cases goes unnoticed. Every now and then, though, a mother may see this in her child and become concerned:

The bacteria is transmitted to cats, typically kittens, by fleas. When a child is scratched by the cat, or in some cases pets the cat and then rubs his or her eye, the bacteria enters their bloodstream.

In 2-3 weeks, the child will develop lymphadenopathy, or the enlargement of the lymph nodes. The most common location of the lymphadenopathy is in the neck (33% of cases), followed by the axilla (underarm, 27%) and inguinal (groin, 18%). The child will also develop the characteristic fever and may complain of a sore throat.

In children with weak immune systems (immunocompromised), the disease may become disseminated (spread throughout the body) and cause infection of the bone (osteomyelitis), brain (encephalitis, resulting in seizures) and eye (oculoglandular conjunctivitis). In children with heart valve problems, this disease may cause endocarditis (infection of heart valve). For this reason, the CDC recommends that these children and adults avoid playing with cats or kittens, especially those with fleas.

Since the disease goes away on its own, a doctor will only need to prescribe pain medication for the painful lymph nodes. There is limited evidence that antibiotics are helpful in treating Cat Scratch Fever, so put on an old record and let Ted do all of the work.

• Cases in adults are not uncommon. 80% of patients with cat-scratch disease are < 21 years old

• Cases of dogs, monkeys, porcupine quills and thorns have been reported

• The disease is not contagious. There are no reports of person-to-person transmission

• Laboratory diagnosis is difficult, since the organism can be difficult to see. Currently, polymerase chain reaction (PCR) or Warthin-Starry Stain is used

• Differential diagnosis of chronic lymphadenopathy includes L. venereum, mycobacteria, tularemia, brucellosis. mononucleosis, syphilis, toxoplasmosis, systemic fungal infections, sarcoidosis, lymphoma, connective tissue disease and kawasaki disease

Reference

Cat Scratch Disease. In DynaMed [database online]. EBSCO Information Services. 

Updated 2012 Oct 25. Accessed May 10, 2014.