Pat .: It is not clear since the 200 kD antigen has not been fully characterized.
Localization: Skin lesions can be localized on the trunk, upper and lower limbs, rather than mucous membranes being spared.
Clinical: Skin lesions have the character of blisters on an inflamed substrate, although they may resemble erythema multiforme, erythema gyratum repens, and urticaria. In some cases, coexistence of psoriasis is observed.
Hist .: Presence of sub-epidermal microvesicles, presence of an inflammatory infiltrate along the BMZ composed of neutrophils and eosinophils.
DI: In DIF (direct immunofluorescence = examination of the patient’s tissue) the presence of in vivo bound IgG and the C3 complement component with a linear pattern along the basement membrane (dermal-epidermal border) – BMZ; in IIF (indirect immunofluorescence = examination of the patient’s serum), the presence of circulating linear IgGs directed against BMZ; WIB (western immunoblot) – reaction of circulating antibodies with 200 kD BMZ antigens.
DR: Erythema gyratum repens, erythema multiforme, pokrzywka.
But: Dapsone is the treatment of choice, systemic corticosteroids are not sufficient to suppress the disease. In some cases, the use of methotrexate with general corticosteroids, intravenous immunoglobulins, mycophenolate mofetil is effective.
Year: The disease usually clears up within a few months.
Lit.: [1] Zillikens D., Ishiko A., Jonkman M.F. i wsp.: Autoantibodies in anti-p200 pemphigoid stain skin lacking laminin 5 and type VII collagen. Br J Dermatol 2000, 143; 1043-9. [2] Hofmann S.C., Voith U., Sasaki T. i wsp.: Th e autoantigen in anti-p200 pemphigoid is synthesized by keratinocytes and fi broblasts and is distinct from nidogen-2. J Invest Dermatol 2008, 128; 87-95. [3] Rose C., Weyers W., Denisjuk N. i wsp.: Histopathology of anti-p200 pemphigoid. Am J Dermatopathol 2007, 29; 119-24. [4] Wozniak K., Kowalewski C., Hashimoto T. i wsp.: Penicillininduced anti-p200 pemphigoid: an unusual morphology. Acta Derm Venereol 2006, 86; 443-6. [5] Yamane N., Sawamura D., Nishie W. i wsp.: Anti-p200 pemphigoid in a 17-year-old girl successfully treated with systemie corticosteroid and dapsone. Br J Dermatol 2007, 156; 1075-8.
Source: A. Kaszuba, Z. Adamski: “Lexicon of dermatology”; XNUMXst edition, Czelej Publishing House