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Perifolliculitis capitis abscedens et suffodiens, Perifolliculitis capitis abscedens et suffodiens of Hoffman, Hoffman disease, Dissecting folliculitis and Dissecting cellulitis of the scalp are all names for a single condition. Usually abbreviated as “PCAS,” this is a type of scalp inflammation that can result in alopecia, killing off hair follicles. This should not be confused with Werdnig-Hoffman disease, which is a form of infant muscular atrophy.
Who gets PCAS?
Anyone can get it, but the majority of cases are diagnosed in males of African descent between the ages of 18 and 40.
What causes this disease?
The exact cause is not known, but it seems to start with blockages of the pilosebaceous unit, the group of structures that hold hair follicles. The unit ruptures, exposing the surrounding tissue to infection. This is a chronic disease punctuated by outbreaks that can last months; the triggers for these flare-ups is unknown.
What complications are associated with PCAS?
PCAS causes pustules and nodules (acne conglobata) to form with interconnecting sinus tracts that drain pus and/or blood resulting in cellulitis. The external sores often turn into abscesses, while the entire area can experience redness, inflammation, tenderness and fever. Over time, repeated outbreaks can lead to scaring and permanent alopecia, resulting in patchy baldness.
The ruptures are a gateway to pathogens, so the main problem sufferers of PCAS have to deal with are bacterial and fungal infections. Before antibiotics were readily available, it was common for sufferers to die from Streptococcus that entered through the wounds.
Although rare, it is possible for untreated PCAS to lead to cutaneous squamous cell carcinoma (SCC,) the most common type of skin cancer. Outbreaks near the eyes can result in keratitis, causing inflammation of the cornea and, if untreated, loss of vision.
How is it diagnosed?
PCAS is a relatively rare disease, and is usually found through a process of elimination. Traditional treatments like cystic acne medications and antibiotics are ineffective when caused by PCAS, while further testing rules out infections as a direct cause of these sores. This is made more confusing by the infections that go along with the disease: Although the patient may experience relief from infection symptoms, the sores caused by PCAS remain.
How can PCAS be treated?
This is a chronic disease with no known cure: Treatment is focused on reducing the chance of complications, not outbreaks. Antibiotics and antibacterial soaps are commonly prescribed to prevent infection, while topical steroids may be used to reduce the tenderness of the lesions. Patients will usually work with both a dermatologist and an infectious disease (ID) specialist to control outbreaks.
In severe cases, surgical procedures may be used to drain off blood and pus from the sinus tracts. If the lesions aren’t responding to traditional treatment methods, surface radiation may be considered, but this is considered a last resort due to the side effects of nuclear medicine.
Image credit: 123rf.com
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