2. Bloch-Sulzberger disease, Bloch Siemens syndrome, melanoblastosis cutis. Above are some other common names used for Incontinentia Pigmenti. Incontinentia Pigmenti is a genetic disorder that affects the skin, nails, teeth, and hair. First, the skin begins to blister(from birth to about 1 month of age). Then you get a wart-like rash(for several months). This disease can also affect the shape of your teeth.
3. Causes Incontinentia Pigmenti usually occurs in females, and is inherited from the mother. This genetic disease is carried on the X chromosome. Girls have 2 X chromosomes and the abnormal gene is on one of them. The other chromosome is normal and balanced.
4. Incidence rate and Prevalence rate 700-1,000 cases of Incontinentia Pigmenti have been reported. Approximately 1 in 388,571 or 0.00% or 700 people in the USA have been reported to have Incontinentia Pigmenti. Incontinentia Pigmenti is listed as a rare disease by the Office of Rare Diseases of the National Institutes of Health. Being a rare disease, that means that 1 per 2,000 people will get this disease.
5. Symptoms Skin redness Spiral lines of small fluid-filled blisters Warty skin growths Skin discolorations Reduced Pigmentation Hair loss Discoloration of nails
6. Preventions/Complications The skin blisters on children with incontinentia pigmenti are far less important than the much more serious complication. The destruction of the retina is by far the more serious of the two. Early diagnosis or therapy can slow down the progress of the destruction of the retina.
7. Ectodermal Dysplasia The Ectodermal Dysplasia Society is a charity dedicated to improving the health and wellbeing of people whose lives are affected by Incontinentia Pigmenti. The Society works together to increase awareness and understanding to both the patient and the family.
