Anti-p200 pemphigoid L12.8

Author: Prof. Dr. med. Peter Altmeyer

All authors of this article

Last updated on: 29.10.2020

Dieser Artikel auf Deutsch

Synonym(s)

anti-laminin gamma-1 pemphigoid; anti-laminin gamma pemphigoid; Pemphigoid anti-p200-Pemphigoid

History
This section has been translated automatically.

Zillikens, 1996; Chen, 1996

Definition
This section has been translated automatically.

Very rare (about 100 cases have been described so far), chronic, bullous autoimmune skin disease with subepithelial cleavage and neutrophilic (eosinophilic) infiltration of the dermo-epidermal junctional zone.

Occurrence/Epidemiology
This section has been translated automatically.

Very rare. So far, less than 100 case descriptions have been published worldwide.

Etiopathogenesis
This section has been translated automatically.

Autoimmune disease; target antigen is a 200 kDa protein (p200) located under the lamina lucida. The NC1 domain of the laminin-gamma1 chain has been identified as the major target antigen. It is probably an adhesion molecule of the cutaneous basement membrane. The protein connects laminin with type IV collagen. It is formed by dermal fibroblasts and keratinocytes.

Some patients also show reactivities against BP230, BP180, laminin 332 or type VII collagen. This phenomenon is calledepitope spreading.

Manifestation
This section has been translated automatically.

Predominantly 5-6 years of age. Men are more frequently affected than women.

Clinical features
This section has been translated automatically.

Heavily itchy, large, deep red patches and plaques and large bulging blisters on the trunk and extremities. Rare vesicular disease pattern. Mucous membrane involvement (oral and genital mucous membrane) in 20% of patients. Healing without scarring.

An association with psoriasis is present in 1/3 of patients.

To what extent esophageal carcinomas are associated with the clinical picture needs further investigation.

Laboratory
This section has been translated automatically.

In the laboratory, an IgG antibody against 200kDa laminin gamma1 is detected by immunoblotting. The antibodies against pemphigoid-AK BP180 and BP 230; collagen VII, laminin 332 are not found.

Histology
This section has been translated automatically.

Subepidermal cleavage with diffuse neutrophil, more rarely eosinophil inflammatory infiltrate at the dermo-epidermal junction zone. More rarely are microabscesses in the papilla tips.

Direct Immunofluorescence
This section has been translated automatically.

In lesional and perilesional skin, linear deposition of IgG and C3 along the dermo-epidermal junction zone.

In addition, saline split skin examinations with the patient's serum, which show IgG and C3 depositions at the dermal base of the artificial split, are performed.

Therapy
This section has been translated automatically.

A standard therapy of anti-p200 pemphigoid cannot be proposed yet. Rapid response to immunosuppressive therapy, e.g. azathioprine 1.0-1.5 mg/kg bw/day p.o. combined with systemic glucocorticoids such as prednisone, initially 1.0-1.5 mg/kg bw/day, later reduction to 20-10 mg/day, has been described.

Alternatives to Azathioprine are:

Progression/forecast
This section has been translated automatically.

Variable course; complete healing after discontinuation of immunosuppressive therapy is possible, as is a recurrent course.

Note(s)
This section has been translated automatically.

Laminin gamma 1 is a glycoprotein of the extracellular matrix, which lies below the lamina lucida and is involved in the construction of the basement membrane. Since the IgG antibodies of the patients in the immunoblot with keratinocyte extract show a reaction with a 200kDa protein, the autoantigen of this disease was first designated as p200 before it was identified as laminin gamma 1.

Literature
This section has been translated automatically.

  1. Chen KR et al (1996) Coexistence of psoriasis and an unusual IgG-mediated subepidermal bullous dermatosis: identification of a novel 200-kDa lower lamina lucida target antigen. Br J Dermatol 134: 340-346
  2. Commin MH et al (2016) Clinical and immunological features and outcome of anti-p200 pemphigoid. Br J Dermatol 175:776-781.
  3. Goetze S et al.,(2016) Anti-p200/laminin γ1 pemphigoid associated with metastatic esophageal cancer. J Eur Acad Dermatol Venereol doi: 10.1111/jdv.13983.
  4. Rose C et al (2007) Histopathology o anti-p200 pemphigoid. On J Dermatopathol 29: 119-124
  5. Shimanovich I et al (2003) The autoantigen of anti-p200 pemphigoid is an acidic noncollagenous N-linked glycoprotein of the cutaneous basement membrane. J Invest Dermatol 121: 1402-1408
  6. Yasuda H et al (2004) Two cases of subepidermal blistering disease with anti-p200 or 180-kD bullous pemphigoid antigen associated with psoriasis. Dermatology 209: 149-155
  7. Zillikens D et al (1996) A novel subepidermal blistering disease with autoantibodies to a 200-kDa antigen of the basement membrane zone. J Invest Dermatol 106: 1333-1338

Incoming links (1)

Pemphigoid antibodies;

Disclaimer

Please ask your physician for a reliable diagnosis. This website is only meant as a reference.

Authors

Last updated on: 29.10.2020