Poikiloderma

Poikiloderma describes a condition in which the skin becomes discolored and undergoes structural changes. Clinicians view poikiloderma as a cluster of signs rather than a distinct single disease. The condition is relatively common and long-standing, but it is not life-threatening.

Poikiloderma may be inherited, meaning you are born with it, or it can be acquired later in life. It’s linked with several uncommon inherited syndromes as well as acquired disorders such as lupus.

The most frequently encountered acquired form is poikiloderma of Civatte, sometimes referred to as sun-related aging.

Pictures of poikiloderma?

What are the symptoms of poikiloderma?

Poikiloderma produces a net-like (reticular) pattern on the skin with the following changes:

• reddish-brown pigmentation
• telangiectasia — small visible blood vessels that resemble broken capillaries
• skin thinning (atrophy)

Poikiloderma of Civatte is identifiable by its typical distribution. In this variant, skin alterations appear on the neck, chest, and cheeks and have these characteristics:

• they are bilateral and symmetrical, appearing similarly on both sides of the face and neck
• they affect the lateral cheeks and neck and the V-shaped area of the chest formed by the neck sides and the lower part of the breastbone
• they almost never involve the patch of skin under the chin that remains shaded from sunlight

Mild burning or itching can occur in the affected areas, though most people with poikiloderma experience little to no discomfort. The skin changes tend to progress slowly over time.

What causes poikiloderma to develop?

Because poikiloderma represents a set of findings rather than a single disease, it may arise from or be associated with many conditions, including:

• genetic syndromes
• infections such as Lyme disease
• connective tissue disorders like lupus and dermatomyositis
• metabolic conditions such as amyloidosis
• immune-related problems, for example after bone marrow transplant rejection
• medications such as corticosteroids or radiation therapy for cancer
• some rare malignancies
• environmental exposures including ultraviolet radiation from the sun

The precise cause of poikiloderma of Civatte is unclear, but sun exposure is almost certainly a key factor. Other potential contributors include:

• heredity
• hormonal shifts, particularly in women with reduced estrogen levels due to menopause or oophorectomy
• sensitivities to topical chemicals, such as those in perfumes or cosmetics

Dermatologists believe long-term ultraviolet exposure plays a major role in poikiloderma of Civatte because ultraviolet light is well known to damage skin and that damage accumulates. The longer the cumulative sun exposure, the greater the likelihood of skin injury. Clues that sun exposure is central to this form of poikiloderma include:

• a higher risk in people with fair complexions
• protection from sunlight (for example, the area beneath the chin) is typically spared while the surrounding sun-exposed skin is affected
• shielding the skin from sun exposure can slow progression and sometimes produce some improvement

Your risk of developing poikiloderma increases if it runs in your family or if you have one of the associated acquired disorders.

Factors that raise the likelihood of poikiloderma of Civatte include being:

• middle-aged
• female, especially around menopause or after removal of the ovaries
• fair-skinned
• living in a sunny climate
• someone who has had significant lifetime sun exposure
• from a family with a history of the condition
• sensitive to chemical irritants, particularly those in fragrances and makeup

How is poikiloderma diagnosed?

See your clinician if you notice worrisome changes in your skin. A doctor can inspect your skin and exclude more serious disorders.

Poikiloderma of Civatte is often diagnosed clinically by history and physical exam. If poikiloderma is suspected to be secondary to another inherited or acquired illness, your provider may order blood work, imaging studies, or other tests based on your accompanying symptoms.

How is poikiloderma treated?

Poikiloderma cannot be completely cured, but treatments may lessen the skin changes and slow further progression.

Addressing any underlying cause is essential and should be the first step. After that, dermatologic approaches may be used to reduce discoloration and make the affected skin less noticeable.

Pulsed dye lasers and intense pulsed light (IPL) therapies are costly but are among the primary methods used to improve visible blood vessels and discoloration. These procedures do not fully restore the skin, and symptoms may worsen temporarily before improving.

According to the Australasian College of Dermatologists, topical agents that lighten or depigment the skin can improve brownish discoloration, and follow-up laser treatment can address redness. Phototherapy can help both brown and red changes.

Since treatment options for reversing poikiloderma are limited, preventing additional damage by protecting skin from ultraviolet light is the cornerstone of managing poikiloderma of Civatte. Recommended measures include:

• using broad-spectrum sunscreen with SPF 30 or higher (some clinicians suggest SPF 50 or more) and reapplying regularly whenever you’re exposed to sunlight in both summer and winter
• avoiding peak sun hours, typically about two hours before to two hours after midday
• wearing sun-protective clothing
• wearing wide-brimmed hats that shade the face, neck, and chest
• using scarves or high-neck garments to cover vulnerable areas

What is the outlook for poikiloderma?

Although poikiloderma can be distressing cosmetically or mildly irritating, it is not life-threatening. There is no cure, but therapies can reduce the appearance of skin discoloration and protective measures can prevent further harm from sun exposure.