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Incontinentia pigmenti

Incontinentia Pigmenti (IP) is a genetic disorder that affects the skin, hair, teeth, and nails. It is also known as Bloch Sulzberger syndrome, Bloch Siemens syndrome, melanoblastosis cutis and naevus pigmentosus systematicus. more...

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The skin lesions evolve through characteristic stages:

  1. blistering (from birth to about four months of age),
  2. a wart-like rash (for several months),
  3. swirling macular hyperpigmentation (from about six months of age into adulthood), followed by
  4. linear hypopigmentation.

Alopecia, hypodontia, abnormal tooth shape, and dystrophic nails are observed. Some patients have retinal vascular abnormalities predisposing to retinal detachment in early childhood. Cognitive delays/mental retardation are occasionally seen.

The diagnosis of IP is established by clinical findings and occasionally by corroborative skin biopsy. Molecular genetic testing of the IKBKG gene (chromosomal locus Xq28) reveals disease-causing mutations in about 80% of probands. Such testing is available clinically. In addition, females with IP have skewed X-chromosome inactivation; testing for this can be used to support the diagnosis.

IP is inherited in an X-linked dominant manner. IP is lethal in most, but not all, males. A female with IP may have inherited the IKBKG mutation from either parent or have a new gene mutation. Parents may either be clinically affected or have germline mosaicism. Affected women have a 50% risk of transmitting the mutant IKBKG allele at conception; however, most affected male conceptuses miscarry. Thus, the expected ratio for liveborn children is 33% unaffected females, 33% affected females, and 33% unaffected males. Genetic counseling and prenatal testing is available.

This disorder was first reported by Bruno Bloch, a German dermatologist in 1926 and Marion Sulzberger, an American dermatologist in 1928.


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From Gale Encyclopedia of Alternative Medicine, 4/6/01 by Paula Ford-Martin


Blisters are small, raised lesions where fluid has collected under the skin. They may be caused by an allergic reaction, burns, frostbite, or by excessive friction or trauma to the skin. Blisters may also be a symptom of a systemic illness, or of a specific skin disorder.


The thin-skinned sac of a blister contains fluid, and in most cases should not be ruptured, as rupturing can introduce infection and slow the healing process. Blisters that contain blood instead of fluid are aptly named blood blisters, and are caused by a rupture of blood vessels beneath the surface of the skin, usually due to trauma.

Causes & symptoms

Blisters can be caused by a number of conditions and environmental agents, including:

  • Friction. Rubbing or pinching can cause skin irritation and blistering. Friction blisters frequently occur on the hands and feet.
  • Disease. Blisters are symptomatic of skin disorders such as impetigo, incontinentia pigmenti syndrome (IPS), and pemphigus vulgaris. Blisters may also be caused by diseases such as herpes and chickenpox.
  • Contact dermatitis. Skin contact with an allergen (e.g., latex, cosmetics, cleaning solutions) can trigger redness, irritation, rash, and blistering of the skin. Blisters also typically appear after skin contact with poison ivy, oak, or sumac.
  • Burns. Blisters appear in cases of severe sunburn and thermal burns.
  • Frostbite. Severely frostbitten skin frequently blisters.
  • Trauma. Blood blisters are caused by trauma to the skin.


Diagnosis and treatment of most minor blisters can typically be made at home by examination of the affected area. Blisters thought to be caused by a systemic illness or disease may require professional diagnosis by a physician, dermatologist, or other healthcare professional. A medical history, physical examination, and further medical testing may be part of the diagnostic procedure.


Unless they are hindering movement or are extremely painful due to their size and/or location, blisters should not be ruptured, or "popped," as doing so can introduce bacteria into the wound. If a blister does burst, the extra skin should be left intact. Blisters that are excessively large or painful should only be punctured using antiseptic procedures, preferably by or under the direction of a qualified healthcare professional.

Treatment of blisters depends on their cause. Blisters that are symptomatic of a disease or disorder require treatment of the illness itself. Blisters caused by friction or trauma can be treated by cleansing with mild soap, applying an antiseptic, and covering the area with a sterile bandage. An herbalist, aromatherapist, or holistic healthcare professional may recommend a compress of an antiseptic or anti-microbial herb such as marigold (Calendula officinalis), thyme (Thymus vulgaris), lavender (Lavandula angustifolia), or tea tree oil (Melaleuca alternifolia).

The blister should be kept clean and the bandage changed frequently. Blood blisters should be bandaged firmly to apply pressure to the area and prevent further blood vessel ruptures.

Allopathic treatment

Conventional medicine typically follows the same procedures for treating skin blisters. A prescription or over-the-counter antiseptic ointment may be recommended to clean the blistered area.

Expected results

With proper treatment, most minor blisters will heal without complication in a matter of days. More serious blisters caused by severe burns and certain diseases may produce permanent scarring or discoloration of the skin.


Friction blisters can be prevented by wearing adequate protection on the area prone to blistering (e.g., gloves for individuals who work with their hands constantly, and thick socks and properly fitting shoes for runners and long-distance walkers).

Fair-skinned individuals who are prone to sunburn should take extra precautions to avoid skin blistering, such as using a high SPF sunscreen (at least 30 SPF) and wearing a large brimmed hat and long-sleeved, loose clothing in the sun.

Key Terms

A bacterial infection of the skin characterized by skin blistering.
Incontinentia pigmenti syndrome (IPS)
An inherited skin disorder characterized by blistered lesions in infancy, which heal but leave uneven pigmentation of the skin.
Pemphigus vulgaris
An autoimmune skin disorder that causes blistering of the skin and mucous membrane.

Further Reading

For Your Information


  • Lawless, Julia. The Complete Illustrated Guide to Aromatherapy. Boston, MA: Element Books, 1997.

Gale Encyclopedia of Alternative Medicine. Gale Group, 2001.

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