Pemphigus

(Pemphigus Syndromes)

Definition

Pemphigus is a group of disorders that affects the skin. The attacks cause blisters and burn-like wounds on skin and mucous membranes (like mouth). There are 3 forms of the disease:
  • Pemphigus vulgaris—most common type of pemphigus
  • Pemphigus foliaceus
  • Paraneoplastic pemphigus—most serious type, usually occurs with cancer

Causes

Pemphigus is an autoimmune disorder. The immune system attacks healthy skin and mucus membranes. The attack causes the sores on the skin.It is not clear what causes the immune system to attack normal body tissue. It is likely due to a combination of genetic and environmental factors. For some, medication may be the cause.

Risk Factors

Factors that may increase your chance of pemphigus include:
  • Family members with pemphigus
  • A history of having autoimmune diseases, such as myasthenia gravis , systemic lupus erythematosus , or thymoma
  • Jewish or Mediterranean descent
  • Regular use of certain medications:
    • Chelating agents, such as penicillamine
    • ACE inhibitors
    • Nonsteroidal anti-inflammatory drugs (NSAIDs), such as aspirin
    • Antibiotics, such as penicillin
    • Antiseizures, such as phenobarbital

Symptoms

Pemphigus may occur over a small or large section of the skin. Itching and pain are common symptoms. Other symptoms will vary according to the type of pemphigus:Pemphigus vulgaris may cause:
  • Lesion that may extend deep into the skin
  • Blisters that usually start in the mouth or on the scalp
  • Lesions that progress to the face, neck, upper body, armpits, and groin
  • Blisters which may appear in the esophagus, rectum, nose, throat, larynx (voice box), eyes, vulva, or rectum
  • Red skin
  • Painful, open sores
  • Blisters that may expand into surrounding tissue when pressure is added to them
  • An outer layer of skin to be easily rubbed off
  • Soft and easily broken blisters that release fluid
  • Large areas of open skin, increasing the risk of fluid imbalance and infection
  • Blisters that usually heal without scarring, but may change skin color to brown
Pemphigus foliaceus may cause:
  • Superficial lesions
  • Blisters that itch or produce a burning sensation
  • Sores that are usually not found in the mouth or on other mucus membranes
  • Blisters that first show up on the face, scalp, chest, or upper back
  • Open blisters, causing shallow sores
  • Red skin
  • Scales and crusts
  • Worsening symptoms in sunlight
Paraneoplastic pemphigus may cause:
  • Sores on mucous membranes, in the mouth, eye, and esophagus
  • Blisters that appear on palms of hands and soles of feet
  • Itchy or painful lesions

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