Pemphigoid non bullous

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 29.10.2020

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Synonym(s)

nodular pemphigoid (non-bullous); non bullous pemphigoid; vesicle-free bullous pemphigoid

Definition
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Localised or exanthematic autoimmune dermatosis of the skin (mucosal infection does not usually occur) of the elderly person. Blistering is hardly or only minimalistically pronounced in this form. The clinical symptoms are usually characterized by excessive (unexplained) itching.

Occurrence/Epidemiology
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The bullous pemphjgoid is the most common blistering autoimmune dermatosis. Incidence: 6-7/100.000 inhabitants/year. With increasing age, the incidence rises from about 10/100,000 inhabitants/year in 60-year-olds to over 40/100,000 inhabitants/year in 90-year-olds. There are indications that incidences have increased significantly in recent years. The bullous pemphigoid is more frequently found in autoimmune diseases, e.g. polymyositis, ulcerative colitis, chronic polyarthritis (rheumatoid arthritis).

Etiopathogenesis
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see below Pemphigoid bullous

Manifestation
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Occurs at an advanced age, usually after the beginning of the 6th decade. Rarely begins in childhood.

Clinical features
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In some patients the clinical picture develops atypically without the formation of the clinically groundbreaking blisters. This means that the clinical leading symptom "bulging (firm) bladder" and the clear clinical assignment to the blister-forming diseases is not applicable. Instead, a clinical picture with itchy, prurigo-like scratched skin changes is impressive. However, urticarial plaques are also usually found (this speaks against the diagnosis "prurigo").

Literature
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  1. Fisler RE et al (2003) Childhood bullous pemphigoid: a clinicopathologic study and review of the literature. At J Dermatopathol 25: 183-189
  2. Lever WF et al (1953) Pemphigus. Medicine 32: 2-123
  3. Powell AM et al (2002) Pemphigoid nodularis (non-bullous): a clinicopathological study of five cases. Br J Dermatol 147: 343-349
  4. Rose C et al (2007) Histopathology of anti-p200 pemphigoid. At J Dermatopaphol 29:119-124
  5. Sami N et al (2003) Influence of intravenous immunoglobulin therapy on autoantibody titres to BPAG1 and BPAG2 in patients with bullous pemphigoid. JEADV 17: 641-645

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

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Last updated on: 29.10.2020