Pemphigus

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Pemphigus
Classification and external resources
Permphigus.jpg
ICD-10L10
ICD-9694.4
OMIM169600 Benign Chronic ~
169610 ~ Vulgaris, Familial
DiseasesDB9764 31179 33489
MedlinePlus000882
eMedicinederm/317
derm/318 ~ foliaceus
derm/314 Drug-induced ~
derm/543 ~ herpetiformis
derm/315 ~ IgA
derm/319 ~ Vulgaris
derm/535 ~Paraneoplastic
derm/150 Benign ~
  (Hailey-Hailey Disease)
Patient UKPemphigus
MeSHD010392
 
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This article is about a skin blistering disease. For another similar-looking blistering skin disease, see Pemphigoid.
Pemphigus
Classification and external resources
Permphigus.jpg
ICD-10L10
ICD-9694.4
OMIM169600 Benign Chronic ~
169610 ~ Vulgaris, Familial
DiseasesDB9764 31179 33489
MedlinePlus000882
eMedicinederm/317
derm/318 ~ foliaceus
derm/314 Drug-induced ~
derm/543 ~ herpetiformis
derm/315 ~ IgA
derm/319 ~ Vulgaris
derm/535 ~Paraneoplastic
derm/150 Benign ~
  (Hailey-Hailey Disease)
Patient UKPemphigus
MeSHD010392
Microscopic image of direct immunofluorescence using an anti-IgG antibody. The tissue is skin from a patient with Pemphigus vulgaris. Note the intercellular IgG deposits in the epidermis and the early intraepidermal vesicle caused by acantholysis.

Pemphigus (/ˈpɛmfɪɡəs/ or /pɛmˈfɡəs/) is a rare group of blistering autoimmune diseases that affect the skin and mucous membranes.[1]

In pemphigus, autoantibodies form against desmoglein. Desmoglein forms the "glue" that attaches adjacent epidermal cells via attachment points called desmosomes. When autoantibodies attack desmogleins, the cells become separated from each other and the epidermis becomes "unglued", a phenomenon called acantholysis. This causes blisters that slough off and turn into sores. In some cases, these blisters can cover a significant area of the skin.[2]

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, a historic paper that changed the understanding of pemphigus was published.[3][4] In 1971, an article investigating the autoimmune nature of this disease was published.[5][6]

Types[edit]

There are several types of pemphigus which vary in severity: pemphigus vulgaris, pemphigus foliaceus, Intraepidermal neutrophilic IgA dermatosis, and paraneoplastic pemphigus.

Note that Hailey-Hailey disease, also called familial benign pemphigus, is an inherited (genetic) skin disease, not an autoimmune disease. It is therefore not considered part of the Pemphigus group of diseases.[7]

Classification[edit]

Pemphigus is a group of autoimmune blistering diseases that may be classified into the following types:[8]

Diagnosis[edit]

Pemphigus is recognized by a dermatologist from the appearance and distribution of the skin lesions. It is also commonly diagnosed by specialists practicing otolaryngology- head and neck surgery, periodontists, oral and maxillofacial surgeons (highly specialized surgeons with an extensive background and qualifications in both medicine and dentistry), and eye doctors, as lesions can affect the eyes and mucous membrane of the oral cavity. Intraorally it resembles the more common diseases lichen planus and mucous membrane pemphigoid.[9] Definitive diagnosis requires examination of a skin or mucous membrane biopsy by a dermatopathologist or oral pathologist. The skin biopsy is taken from the edge of a blister, prepared for histopathology and examined with a microscope. The pathologist looks for an intraepidermal vesicle caused by the breaking apart of epidermal cells (acantholysis). Thus, the superficial (upper) portion of the epidermis sloughs off, leaving the bottom layer of cells on the "floor" of the blister. This bottom layer of cells is said to have a "tombstone appearance".

Definitive diagnosis also requires the demonstration of anti-desmoglein autoantibodies by direct immunofluorescence on the skin biopsy. These antibodies appear as IgG deposits along the desmosomes between epidermal cells, a pattern reminiscent of chicken wire. Anti-desmoglein antibodies can also be detected in a blood sample using the ELISA technique.

Half of pemphigus patients have oral lesions alone during the first year but develop skin lesions later.

Treatment[edit]

If not treated, pemphigus can be fatal from an overwhelming infection of the sores. The most common treatment is the administration of oral steroids, especially prednisone, often in high doses. The side effects of corticosteroids may require the use of so-called steroid-sparing or adjuvant drugs. The immunosuppressant CellCept (mycophenolic acid) is among those being used.[10]

Intravenous gamma globulin (IVIG) may be useful in severe cases, especially paraneoplastic pemphigus. Mild cases sometimes respond to the application of topical steroids. Recently, Rituximab, an anti-CD20 antibody, was found to improve otherwise untreatable severe cases of Pemphigus vulgaris.[11][12]

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

One of the most dangerous side effects of high dosage steroid treatments is intestinal perforations, which may lead to sepsis. Steroids and other medications being taken to treat Pemphigus may also mask the effects of the perforations. Patients on high dosages of oral steroids should closely monitor their GI health.

If paraneoplastic pemphigus is diagnosed with pulmonary disease, a powerful cocktail of immune suppressant 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.

If skin lesions do become infected, antibiotics may be prescribed. Tetracycline antibiotics have a mildly beneficial effect on the disease and are sometimes enough for Pemphigus Foliaceus. In addition, talcum powder is helpful to prevent oozing sores from adhering to bedsheets and clothes.

Pain is a common part of the disease. Only one literature review on peer-reviewed articles reporting pemphigous pain management has been published in the professional medical literature.[13]

Pemphigus in domestic animals[edit]

Pemphigus foliaceus skin eruption on the ventral abdomen of a dog

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

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

See also[edit]

Footnotes[edit]

  1. ^ Yeh SW, Ahmed B, Sami N, Ahmed AR (2003). "Blistering disorders: diagnosis and treatment". Dermatologic Therapy 16 (3): 214–23. doi:10.1046/j.1529-8019.2003.01631.x. PMID 14510878. 
  2. ^ International Pemphigus & Pemphigoid Foundation: What is Pemphigus?
  3. ^ Beutner, EH; Jordon, RE (November 1964). "Demonstration of skin antibodies in sera of pemphigus vulgaris patients by indirect immunofluorescent staining". Proc. Soc. Exp. Biol. Med. 117: 505–510. doi:10.3181/00379727-117-29622. PMID 14233481. 
  4. ^ "Dermatology Foundation: BEUTNER, JORDAN SHARE 2000 DERMATOLOGY FOUNDATION DISCOVERY AWARD". 
  5. ^ Jordon, Robert E.; Sams, Jr., W. Mitchell; Diaz, Gustavo; Beutner, Ernst H. (1971). "NEGATIVE COMPLEMENT IMMUNOFLUORESCENCE IN PEMPHIGUS". Journal of Investigative Dermatology 57 (6): 407–410. doi:10.1111/1523-1747.ep12293273. PMID 4108416. 
  6. ^ Serratos, BD; Rashid, RM (Jul 15, 2009). "Nail disease in pemphigus vulgaris.". Dermatology online journal 15 (7): 2. PMID 19903430. 
  7. ^ Hailey Hailey Disease Society
  8. ^ Stanley, John R. (2003). "Chapter 59: Pemphigus". In Freedberg et al. Fitzpatrick's Dermatology in General Medicine. (6th ed.). McGraw-Hill. p. 559. ISBN 0-07-138067-1. 
  9. ^ Sapp, J. Philip; Eversole, Lewis R.; Wysocki, George P. (1997). Contemporary Oral and Maxillofacial Pathology. Mosby. ISBN 978-0-8016-6918-7. also here
  10. ^ British Association of Dermtologists, Steroid sparing (or adjuvant) drugs
  11. ^ Ahmed AR, Spigelman Z, Cavacini LA, Posner MR (2006). "Treatment of pemphigus vulgaris with rituximab and intravenous immune globulin". N. Engl. J. Med. 355 (17): 1772–9. doi:10.1056/NEJMoa062930. PMID 17065638. 
  12. ^ Joly P, Mouquet H, Roujeau JC, et al. (2007). "A single cycle of rituximab for the treatment of severe pemphigus". N. Engl. J. Med. 357 (6): 545–52. doi:10.1056/NEJMoa067752. PMID 17687130. 
  13. ^ Rashid, RM; Candido, KD (Oct 2008). "Pemphigus pain: a review on management.". The Clinical journal of pain 24 (8): 734–5. doi:10.1097/AJP.0b013e31817af6fc. PMID 18806540. 

External links[edit]