Epidermolytic ichthyosis (EI), also known as bullous epidermis ichthyosis (BEI), epidermolytic hyperkeratosis (EHK), bullous congenital ichthyosiform erythroderma (BCIE), bullous ichthyosiform erythroderma: 482  or bullous congenital ichthyosiform erythroderma Brocq, is a rare and severe form of ichthyosis this skin disease affects around 1 in 300,000 people. The condition is caused by a genetic mutation, so it cannot be completely cured without some form of gene therapy. While some research has been done into possible gene therapy treatments, the work hasnt yet been successfully developed to the stage where it can be routinely given to patients. The condition involves the clumping of keratin filaments. : 562

Presentation
Epidermolytic hyperkeratosis is a skin disorder that is present at birth. Affected babies may have very red skin (erythroderma) and severe blisters. Because newborns with this disorder are missing the protection provided by normal skin, they are at risk of becoming dehydrated and developing infections in the skin or throughout the body (sepsis). As affected individuals get older, blistering is less frequent, erythroderma becomes less evident, and the skin becomes thick (hyperkeratotic), especially over joints, on areas of skin that come into contact with each other, or on the scalp or neck. This thickened skin is usually darker than normal. Bacteria can grow in the thick skin, often causing a distinct odor. Epidermolytic hyperkeratosis can be categorized into two types :-

People with PS-type epidermolytic hyperkeratosis have thick skin on the palms of their hands and soles of their feet (palmoplantar or palm/sole hyperkeratosis) in addition to other areas of the body.
Rifabutin (Rfb) is an antibiotic used to treat tuberculosis and prevent and treat Mycobacterium avium complex. It is typically only used in those who cannot tolerate rifampin such as people with HIV/AIDS on antiretrovirals. For active tuberculosis it is used with other antimycobacterial medications. For latent tuberculosis it may be used by itself when the exposure was with drug-resistant TB.Common side effects include abdominal pain, nausea, rash, headache, and low blood neutrophil levels. Other side effects include muscles pains and uveitis. , especially when hitting Bartonella and Babesia colonies in the capillaries of the ciliary body in the eye anterior chamber. While no harms have been found during pregnancy it has not been well studied in this population. Rifabutin is in the rifamycin family of medications. It works by blocking RNA production in bacteria.Rifabutin was approved for medical use in the United States in 1992. It is on the World Health Organizations List of Essential Medicines. Medical uses
Rifabutin is now recommended as first-line treatment for tuberculosis (TB), but rifampicin was used more widely because of its cheaper cost. However, due to the expiration of patents, prices are now similar. Rifabutin is also used in the treatment of Mycobacterium avium complex disease, a bacterial infection most commonly encountered in people with late-stage AIDS. Its has fewer drug interactions than rifampicin, therefore people with HIV/AIDS on HAART are typically prescribed rifabutin instead of rifampicin for the treatment of TB.