Porphyria

Porphyria refers to a group of rare disorders that result from the buildup of natural chemicals in the body called porphyrins, which are essential for the function of haemoglobin. When these chemicals accumulate, they can cause a range of symptoms affecting the skin, nervous system, or both.

There are two main types of porphyria:

  • Acute Porphyria
    Primarily affects the nervous system, causing symptoms like abdominal pain, muscle weakness, and neurological issues.
  • Cutaneous Porphyria
    Affects the skin, leading to sensitivity to sunlight, blistering, scarring, and changes in skin pigmentation.

Symptoms of Cutaneous Porphyria

  • Blistering on sun-exposed areas (hands, face, arms).
  • Redness, swelling, and itching after sun exposure.
  • Fragile skin that is prone to scarring.
  • Hyperpigmentation or hypopigmentation.

Triggers

Porphyria symptoms can be triggered or worsened by:

  • Sun exposure.
  • Certain medications.
  • Alcohol consumption.
  • Stress or infections.
  • Hormonal changes.

Treatment Options for Porphyria

At East Malvern Dermatology, we provide comprehensive care for managing cutaneous porphyria:

  • Sun Protection:
    Strict sun avoidance and the use of broad-spectrum sunscreens to prevent flare-ups.
  • Phototherapy (UVB):
    In some cases, controlled phototherapy may help increase skin tolerance to sunlight.
  • Topical Treatments:
    Steroid creams to reduce inflammation and discomfort during flare-ups.
  • Lifestyle Modifications:
    Guidance on avoiding known triggers such as specific medications, alcohol, and dietary factors.
  • Medical Referral:
    Collaboration with other specialists if systemic treatment is required for acute porphyria.

Managing porphyria requires expert care and personalised treatment.

Book a consultation today with our dermatologists to discuss effective management strategies tailored to your needs.

Book Your Appointment Today.

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Information sourced from DermNetNZ

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