Pemphigus

Pemphigus
Pemphigus, from 1886 medical book
Specialty	Dermatology

Pemphigus (/ˈpɛmfɪɡəs/ or /pɛmˈfaɪɡəs/) is a rare group of blistering autoimmune diseases that affect the skin and mucous membranes.^[1] The name is derived from the Greek root pemphix, meaning "blister".^[2]

In pemphigus,

Originally, the cause of this disease was unknown, and "pemphigus" was used to refer to any blistering disease of the skin and mucosa. In 1964, researchers found that the blood of patients with pemphigus contained antibodies to the layers of skin that separate to form the blisters.[4]^[5] In 1971, an article investigating the autoimmune nature of this disease was published.^[6][7]

Types

The several types of pemphigus (pemphigus vulgaris, pemphigus foliaceus, intraepidermal neutrophilic IgA dermatosis, and paraneoplastic pemphigus) vary in severity. Skin lesions caused by pemphigus can lead to fatal infections, so treatment is extremely important.

• desmoglein 3. Sores often originate in the mouth, making eating difficult and uncomfortable. Although PV may occur at any age, it is most common among people between 40 and 60. It is more frequent among Ashkenazi Jews. Rarely, it is associated with myasthenia gravis. Nail disease may be the only finding and has prognostic value in management.^{[citation needed][8]}
• eczema^[8] PF is endemic in Brazil ^[9] and Tunisia.^[10]
• eosinophils, and acantholysis.^[8]
• The least common and most severe type of pemphigus is
  Castleman's disease. It may precede the diagnosis of the tumor. Painful sores appear on the mouth, lips, and the esophagus. In this variety of pemphigus, the disease process often involves the lungs, causing bronchiolitis obliterans
  (constrictive bronchiolitis). Though much less frequent, it is still found the most in the Ashkenazi Jewish population. Complete removal of and/or cure of the tumor may improve the skin disease, but lung damage is generally irreversible.
• Endemic pemphigus foliaceus includes fogo selvagem, the new variant of endemic pemphigus foliaceus in El Bagre, Colombia, and the Tunisian endemic pemphigus in North Africa.[11]

Pemphigus is a group of autoimmune blistering diseases that may be classified into these types:[14]

• Pemphigus vulgaris
• Pemphigus vegetans
• Pemphigus vegetans of Hallopeau
• Pemphigus vegetans of Neumann
• Pemphigus foliaceus, of which there several forms:
• Pemphigus erythematosus or Senear–Usher Syndrome
• Endemic pemphigus foliaceus with its three variants, Fogo Selvagem, the new variant endemic pemphigus Foliaeus and Tunisian endemic pemphigus foliaceus
• Paraneoplastic pemphigus
• IgA pemphigus, of which there several forms:
• Subcorneal pustular dermatosis
• Intraepidermal neutrophilic IgA dermatosis
• Drug induced pemphigus

Treatment

If not treated, pemphigus can be fatal, usually from overwhelming opportunistic

• Topical steroids, such as clobetasol
• Intralesional injection of steroids, such as dexamethasone
• Immunosuppressant drugs, such as CellCept (mycophenolic acid): In recent years, adjuvant drugs, especially biologics, have shown great promise.^[16]
• Serum- or plasma-pooled products, such as intravenous gamma globulin (IVIG) may be useful in severe cases, especially paraneoplastic pemphigus.
• Biologics such as Rituximab, an anti-CD20 antibody, which was found to improve otherwise severe cases of recalcitrant pemphigus vulgaris.^[17][18] Currently rituximab is considered a first-line treatment for some pemphigus patients according to Delphi consensus recommendations ^[19]

All of these drugs may cause severe side effects, so patients should be closely monitored by doctors. Once the outbreaks are under control, dosage is often reduced, to lessen side effects.

A meta-analysis of the literature found insufficient evidence to determine the optimal treatment regimen for pemphigus vulgaris and pemphigus foliaceus, but it found that adding cyclophosphamid and azathioprine to a glucocorticoid regimen reduced the amount of glucocorticoid needed for treatment, and topical epidermal growth factor significantly reduced lesion healing time.^[20]

If skin lesions do become infected,

If paraneoplastic pemphigus is diagnosed with pulmonary disease, a powerful cocktail of immunosuppressant drugs is sometimes used in an attempt to halt the rapid progression of bronchiolitis obliterans, including methylprednisolone, ciclosporin, azathioprine, and thalidomide. Plasmapheresis may also be useful.

Animals affected

Pemphigus foliaceus has been recognized in pet dogs, cats, and horses, and is the most common autoimmune skin disease diagnosed in veterinary medicine. PF in animals produces clusters of small vesicles that quickly evolve into pustules. Pustules may rupture, forming erosions or become crusted. Left untreated, PF in animals is life-threatening, leading to not only loss of condition, but also secondary infection.

PV is a very rare disorder described in pet dogs and cats. Paraneoplastic pemphigus has been identified in pet dogs.

See also

• List of conditions caused by problems with junctional proteins
• List of cutaneous conditions
• List of immunofluorescence findings for autoimmune bullous conditions
• List of target antigens in pemphigus
• Pemphigoid
• Pemphigus herpetiformis

References