Syn.: Erythrodermic pemphigoid.
Pat .: “Bullous pemphigoid.
Loc .: Skin changes are generalized.
Clinical: This type of pemphigoid is more common in 7-8 women. decade of life. It is clinically manifested by inflammation of the skin of the whole body with the presence of exfoliation; if blisters appear, it is usually after erythroderma for several weeks. Nikolsky’s symptom may be positive. Some patients have elevated serum IgE levels and peripheral eosinophilia. This form of pemphigoid causes diagnostic difficulties, especially at the beginning of the disease.
Hist .: Presence of subepidermal microvesicles, an inflammatory infiltrate along the BMZ composed of neutrophils and eosinophils, as well as lymphoid cells. Swelling of the dermis.
DI: In DIF (direct immunofluorescence = examination of the patient’s tissue) – the presence of in vivo bound IgG and the complement component C3 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 IgG directed against BMZ; WIB (western immunoblot) – reaction of circulating antibodies with 180 kD (BP180) and / or 230 kD (BP230) BMZ antigens.
DR: Psoriatic erythrodemia, toxic epidermal necrolysis, drug-induced erythroderma.
Healing: Prednisone, Azathioprine, Erythromycin, Minocycline.
Year: Good, skin inflammation subsides fairly quickly with general corticosteroid use.
Lit.: [1] Tappeiner G., Konrad K., Holubar K.: Erythrodermic bullous pemphigoid. Report of a case. J Am Acad Dermatol 1982, 6; 489-92. [2] Scrivener Y., Heid E., Grosshans E., Cribier B.: Erythrodermic bullous pemphigoid. J Am Acad Dermatol 1999, 41; 658-9. [3] Scrivener Y., Cribier B., Le Coz C. i wsp.: Erythroderma with immunoglobulin deposits along the basal membrane. Pemphigoid erythroderma? Ann Dermatol Venereol 1998, 125; 13-7. [4] Korman N.J., Woods S.G.: Erythrodermic bullous pemphigoid is a clinical variant of bullous pemphigoid. Br J Dermatol 1995, 133; 967-71. [5] Saitoh A., Osada A., Ohtake N. i wsp.: Erythrodermic bullous pemphigoid. J Am Acad Dermatol 1993, 28; 124-5.
Source: A. Kaszuba, Z. Adamski: “Lexicon of dermatology”; XNUMXst edition, Czelej Publishing House