What are the other Names for this Condition? (Also known as/Synonyms)

• AOSD-Associated Flagellate Erythema
• Flagellate Erythema due to Adult-Onset Still’s Disease

What is Adult-Onset Still’s Disease Associated Flagellate Erythema? (Definition/Background Information)

• Adult-Onset Still’s Disease Associated Flagellate Erythema is an uncommon skin condition that occurs against a background of adult-onset Still’s disease, a rare auto-inflammatory condition that may affect several systems of the body
• Adult-onset Still’s disease (AOSD) is marked by certain characteristic symptoms that include high fevers, joint pain, and salmon-colored rashes on the skin. The symptoms are known to ‘come and go’, meaning that some individuals may remain symptom-free for a period of time
• Flagellate erythema is an uncommon skin rash that has a specific linear streak pattern. The pattern resembles flagellation - as though the individual has been beaten by a whip (or whiplashed). There are other causes of flagellate erythema besides adult-onset Still’s disease. One cause is prior treatment with the chemotherapy agent bleomycin
• Adult-Onset Still’s Disease Associated Flagellate Erythema is usually seen in adults. It is believed that the itchy skin rash develops secondary to AOSD, as an abnormal immune response by the body. The skin rashes in AOSD-Associated Flagellate Erythema may occur on the back, chest, or arms and legs
• Adequate treatment of the underlying adult-onset Still’s disease may help improve the skin rashes. In some individuals, symptomatic treatment, using steroid medications or laser therapy for flagellate erythema, may be necessary
• The prognosis of Adult-Onset Still’s Disease Associated Flagellate Erythema is good in a majority of cases, with early treatment. However, the overall prognosis also depends on the underlying cause and its severity

Who gets Adult-Onset Still’s Disease Associated Flagellate Erythema? (Age and Sex Distribution)

• Adult-Onset Still’s Disease Associated Flagellate Erythema may occur at any age; adults are affected the most. Many cases are observed in older children and teens too
• Both males and females may be affected
• Individuals of all racial and ethnic background can be affected

What are the Risk Factors for Adult-Onset Still’s Disease Associated Flagellate Erythema? (Predisposing Factors)

• Individuals with adult-onset Still’s disease are at an increased risk for developing Adult-Onset Still’s Disease Associated Flagellate Erythema

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases one’s chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Adult-Onset Still’s Disease Associated Flagellate Erythema? (Etiology)

• Research scientists believe that Adult-Onset Still’s Disease Associated Flagellate Erythema is caused by an immunological response of the body to adult-onset Still’s disease (AOSD). The characteristic skin rashes form as a reaction to the underlying condition
• The exact cause of adult-onset Still’s disease is unknown, but it is believed to be the result of an underlying viral or bacterial infection, such as infectious mononucleosis or Lyme disease
• It is proposed that flagellate erythema is not a disorder in itself; rather,  the manifestation of a systemic condition that occurs secondary to the associated AOSD

What are the Signs and Symptoms of Adult-Onset Still’s Disease Associated Flagellate Erythema?

The signs and symptoms of Adult-Onset Still’s Disease Associated Flagellate Erythema include:

• The itchy (pruritic) rash occurs as red whiplash-like streak on any part of the body skin
• Scratching and rubbing the area may make the condition worse, resulting in bleeding
• The rashes occur usually on the back and flank region (trunk or chest) and on the extremities
• Additionally, the symptoms of adult-onset Still’s disease may characteristically include high fever, joint pain, and salmon-colored skin rashes. Apart from this, it may also lead to muscle pain, sore throat, enlarged lymph nodes, and involvement of the major organs

How is Adult-Onset Still’s Disease Associated Flagellate Erythema Diagnosed?

A diagnosis of Adult-Onset Still’s Disease Associated Flagellate Erythema may involve the following:

• A thorough medical history assessment and complete physical examination
• Assessment of the signs and symptoms
• Dermoscopy: It is a diagnostic tool where a dermatologist examines the skin using a special magnified lens
• Wood’s lamp examination: In this procedure, the healthcare provider examines the skin using ultraviolet light. It is performed to examine the change in skin pigmentation
• Blood tests that include complete blood count, C-reactive protein levels, erythrocyte sedimentation rate (ESR), antinuclear antibodies (ANA) test, and liver function test
• Imaging studies, such as X-rays of the affected joints, CT scans to detect enlarged organs, and abdominal ultrasound scans
• Echocardiogram and CT scan of chest may be undertaken, to detect any heart/lung abnormalities
• Skin or muscle biopsy: A skin/muscle biopsy is performed and sent to a laboratory for a pathological examination. The pathologist examines the biopsy under a microscope. After putting together clinical findings, special studies on tissues (if needed) and with microscope findings, the pathologist arrives at a definitive diagnosis

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Adult-Onset Still’s Disease Associated Flagellate Erythema?

The complications of Adult-Onset Still’s Disease Associated Flagellate Erythema may include:

• Change in skin pigmentation
• Cosmetic issues that result in emotional stress
• Bleeding and ulceration of the skin may result in secondary viral or bacterial infections
• Complications due to adult-onset Still’s disease that may include chronic joint pain and joint damage, and involvement of the liver, heart, and lungs
• Recurrence of the skin rashes

How is Adult-Onset Still’s Disease Associated Flagellate Erythema Treated?

The treatment options of Adult-Onset Still’s Disease Associated Flagellate Erythema include treating the symptoms of the underlying adult-onset Still’s disease (AOSD), since it is an incurable condition. The treatment of AOSD is undertaken through:

• Administration of medication, such as non-steroidal anti-inflammatory drugs (NSAIDs), based on the healthcare provider’s assessment
• Steroid therapy for severe cases
• Symptomatic and supportive therapy
• Canakinumab is an injectable medicine which blocks the abnormal inflammatory biochemicals which drive AOSD. It is FDA approved for this indication

Typically, symptomatic treatment is provided for flagellate erythema, if necessary, which may include:

• Topical steroid creams and oral steroids for itchy skin
• Cooling the area of the rash can be helpful
• Skin hyperpigmentation can be treated using laser therapy, if it causes significant cosmetic concerns

How can Adult-Onset Still’s Disease Associated Flagellate Erythema be Prevented?

Currently, there are no known methods to prevent the occurrence of Adult-Onset Still’s Disease Associated Flagellate Erythema. However, one may lower the risk for the skin condition by considering the following factors:

• Treating adult-onset Still’s disease early and adequately
• Inform your physician if you are allergic to certain medications

What is the Prognosis of Adult-Onset Still’s Disease Associated Flagellate Erythema? (Outcomes/Resolutions)

• The prognosis for Adult-Onset Still’s Disease Associated Flagellate Erythema is usually good, with prompt treatment of adult-onset Still’s disease
• However, the overall prognosis is dependent on the underlying disorder, the severity of the symptoms presented, and damage to internal organs, if any
• Involvement of the heart, lungs, brain, or kidney generally portray poorer outcomes

Additional and Relevant Useful Information for Adult-Onset Still’s Disease Associated Flagellate Erythema:

• Cleaning the skin too hard with strong chemicals or soaps may aggravate the skin condition. Care must be taken avoid strong soaps and chemicals that could potentially worsen the condition
• The presence of dirt on the body is not a causative factor for the condition. However, it helps to be clean and hygienic, which may help the condition from getting worse