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Bullous pemphigoid

bullous pemphigoid, bullous pemphigoid pictures
Bullous pemphigoid is an acute or chronic autoimmune skin disease, involving the formation of blisters, more appropriately known as bullae, at the space between the epidermis and dermis skin layers It is classified as a type II hypersensitivity reaction, with the formation of anti-hemidesmosome antibodies

Contents

  • 1 Signs and symptoms
  • 2 Causes
  • 3 Pathophysiology
  • 4 Diagnosis
  • 5 Treatment
  • 6 Prognosis
  • 7 Epidemiology
  • 8 Research
  • 9 See also
  • 10 References
  • 11 Further reading
  • 12 External links

Signs and symptomsedit

Clinically, the earliest lesions may appear urticarial like hives Tense bullae eventually erupt, most commonly at the inner thighs and upper arms, but the trunk and extremities are frequently both involved Any part of the skin surface can be involved Oral lesions are present in a minority of cases1 The disease may be acute, but typically will wax and wane Several other skin diseases may have similar symptoms However, milia are more common with epidermolysis bullosa acquisita, because of the deeper antigenic targets A more ring-like configuration, with a central depression or centrally collapsed bullae may indicate linear IgA disease Nikolsky's sign is negative unlike pemphigus vulgaris where it is positive

Causesedit

In most cases of bullous pemphigoid, no clear precipitating factors are identified1 Potential precipitating events that have been reported include exposure to ultraviolet light and radiation therapy12 Onset of bullous pemphigoid has also been associated with certain drugs, including furosemide, and other nonsteroidal anti-inflammatory agents, captopril, penicillamine, and antibiotics2

Pathophysiologyedit

The bullae are formed by an immune reaction, initiated by the formation of IgG3 autoantibodies targeting Dystonin, also called Bullous Pemphigoid Antigen 1,4 and/or type XVII collagen, also called Bullous Pemphigoid Antigen 2,5 which is a component of hemidesmosomes A different form of dystonin is associated with neuropathy4 Following antibody targeting, a cascade of immunomodulators results in a variable surge of immune cells, including neutrophils, lymphocytes and eosinophils coming to the affected area Unclear events subsequently result in a separation along the dermoepidermal junction and eventually stretch bullae

Diagnosisedit

Diagnosis is based on two biopsies of the skin, one submitted for routine H&E staining and one for immunofluorescence studies

Treatmentedit

Treatments include class I topical steroids clobetasol, halobetasol, etc which in some studies have proven to be equally effective as systemic, or pill, therapy and somewhat safer1 However, in difficult-to-manage or widespread cases, systemic prednisone and powerful steroid-free immunosuppressant medications, such as methotrexate, azathioprine or mycophenolate mofetil, may be appropriate16 Antibiotics such as tetracycline or erythromycin may also control the disease, particularly in patients who cannot use corticosteroids6 The anti-CD20 monoclonal antibody rituximab has been found to be effective in treating some otherwise refractory cases of bullous pemphigoidcitation needed

IgA-mediated pemphigoid can often be difficult to treat even with usually effective medications such as rituximab7

Prognosisedit

Bullous pemphigoid may be self-resolving in a period ranging from several months to many years even without treatment1 Poor general health related to old age is associated with a poorer prognosis1

Epidemiologyedit

Very rarely seen in children, bullous pemphigoid most commonly occurs in people 70 years of age and older1 Estimated frequency is seven to 14 cases per million per year, but has been reported to be as high as 472 cases per million per year in Scottish men older than 851 At least one study indicates the incidence might be increasing in the United Kingdom8 Some sources report it affects men twice as frequently as women,6 while others report no difference between the sexes1

Many mammals can be afflicted, including dogs, cats, pigs, and horses, as well as humans It is very rare in dogs; on average, three cases are diagnosed around the world each yearcitation needed

Researchedit

Animal models of bullous pemphigoid have been developed using transgenic techniques to produce mice lacking the genes for the two known autoantigens, dystonin and collagen XVII45

See alsoedit

  • Cicatricial pemphigoid
  • Gestational pemphigoid
  • List of target antigens in pemphigoid
  • List of immunofluorescence findings for autoimmune bullous conditions

Referencesedit

  1. ^ a b c d e f g h i j Wolff K, Goldsmith L, Gilchrest B, Stephen Katz, Amy Paller, David Leffell 17 October 2007 "Chapter 54 Bullous Pemphigoid" In Wolff K, Goldsmith LA, Katz SI, Gilchrest BA, Paller AS, Leffell DJ Fitzpatrick's Dermatology In General Medicine, Two Volumes 7th ed Mcgraw-hill ISBN 978-0-07-146690-5 Retrieved July 21, 2012 
  2. ^ a b Lawrence S Chan 2011 "Bullous Pemphigoid" eMedicine Reference 
  3. ^ "Dorlands Medical Dictionary:bullous pemphigoid" Retrieved 2010-06-24 
  4. ^ a b c "OMIM Entry - 113810 - DYSTONIN; DST" permanent dead link
  5. ^ a b "OMIM Entry - 113811 - COLLAGEN, TYPE XVII, ALPHA-1; COL17A1" permanent dead link
  6. ^ a b c ""Bullous Pemphigoid" Quick Answers to Medical Diagnosis and Therapy" Retrieved 2012-07-21 
  7. ^ He Y, Shimoda M, Ono Y, Villalobos IB, Mitra A, Konia T, Grando SA, Zone JJ, Maverakis E 2015 "Persistence of Autoreactive IgA-Secreting B Cells Despite Multiple Immunosuppressive Medications Including Rituximab" JAMA Dermatol 151 6: 646–50 PMID 25901938 doi:101001/jamadermatol201559 
  8. ^ Langan SM, Smeeth L, Hubbard R, Fleming KM, Smith CJ, West J 2008 "Bullous pemphigoid and pemphigus vulgaris--incidence and mortality in the UK: population based cohort study" BMJ 337: a180 PMC 2483869  PMID 18614511 doi:101136/bmja180 

Further readingedit

  • Wojnarowska, F; Kirtschig, G; Highet, AS; Venning, VA; Khumalo, NP; British Association of, Dermatologists Aug 2002 "Guidelines for the management of bullous pemphigoid" The British journal of dermatology 147 2: 214–21 PMID 12174090 doi:101046/j1365-2133200204835x 

External linksedit

  • "Bullous Pemphigoid" PDF badorguk Archived from the original PDF on 6 October 2010 
  • Bullous pemphigoid on American Osteopathic College of Dermatology
  • DermNet immune/pemphigoid
  • Bullous pemphigoid on Penn State College of Medicine
  • Skin antibodies pemphigoid- look under the heading "Other diseases"

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