Individuals with Krabbe disease with severe motor deficits tend to be more susceptible to overfeeding, as they require less calorie consumption and thus consume fewer calories than what caretakers may expect. There is also evidence that routine vaccines may accelerate disease progression; many individuals with Krabbe disease tend to not follow traditional vaccination procedures. Prognosis
In infantile Krabbe disease, death usually occurs in early childhood. A 2011 study found one-, two-, and three-year survival rates of 60%, 26%, and 14%, respectively, with a few surviving longer. Patients with late-onset Krabbe disease tend to have a slower progression of the disease and live significantly longer. Epidemiology
This disease does not only impact humans, but other animals such as monkeys, mice, and dogs have been observed to develop Krabbe disease as well. While certain gene deletions are more frequent than others, novel mutations resulting in Krabbe disease have been discovered worldwide. Most commonly, the underlying cause of the disease is a deletion of a GALC gene, which causes a deficiency in the GALC enzyme. This is the circumstance in 80% of patients who have European and Mexican origins. The mortality rate of early infantile Krabbe disease is 90% before the age of two. Later onset of symptoms is associated with longer life expectancy, with older children generally surviving two to seven years after the initial diagnosis.Krabbe disease occurs in about one in 100,000 births. Because the disease is genetic, incidence rates vary widely from population to population.
Outcome and treatment
There is limited evidenced-based data concerning the treatment of AF. Treatment should be sought immediately in order to avoid hospitalization. If not treated, hospitalization for an extended period of time (usually two weeks) is likely. During hospitalization, the patient is tested for signs of system degradation, especially of the skeletal structure and the digestive tract. By this time open sores will develop on the upper torso. Some will be the size of dimes, others will be large enough to stick a couple fingers into. They will crust up, causing cohesion to any fabric the sores touch, which is extremely painful to remove. It is recommended to sleep on ones sides until the cystic condition subsides, in order to avoid any uncomfortable situations. Debridement and steroid therapy is preferred over antibiotics. Recurrent AF is extremely rare. Bone lesions typically resolve with treatment, but residual radiographic changes, such as sclerosis and hyperostosis, may remain. Scarring and fibrosis may result from this acute inflammatory process. The disease activates at the height of puberty, usually at around 13 years of age. Acne fulminans predominantly affects young males aged 13 to 22 years with a history of acne. Studies have found successful treatment with Antimicrobial Agents and Oral Prednisolone. History
In 1958, at a meeting of the Detroit Dermatological Society, Burns and Colville presented a 16-year-old white boy with acute febrile disease and acne conglobata. Many similar cases have been reported since then.