PEMPHIGUS.Nowhere in medicine can the passage from basic science to clinical application be better seen than in the blistering disease of pemphigus pemphigus /pem·phi·gus/ (-gus) 1. a distinctive group of diseases marked by successive crops of bullae. 2. pemphigus vulgaris. . The discovery by Beuntner and Jordan [1] in 1964 of circulating antibodies against the cell surface of keratinocytes Keratinocytes Cells found in the epidermis. The keratinocytes at the outer surface of the epidermis are dead and form a tough protective layer. The cells underneath divide to replenish the supply. in the serum of patients with pemphigus vulgaris pemphigus vul·gar·is n. Pemphigus occurring in middle age, in which cutaneous flaccid acantholytic suprabasal bullae and oral mucosal erosions are first localized but become generalized after a few months, forming blisters that break easily and are pioneered our understanding that pemphigus vulgaris is a tissue-specific autoimmune disease autoimmune disease, any of a number of abnormal conditions caused when the body produces antibodies to its own substances. In rheumatoid arthritis, a group of antibody molecules called collectively RF, or rheumatoid factor, is complexed to the individual's own gamma of the skin and mucosa. This discovery led to the identification of antibodies in other autoimmune diseases of the skin. Throughout the fields of medicine, we are all aware of autoimmune diseases that target various organ-specific tissues. Effective clinical treatment has closely followed these basic science discoveries. In the review by Woldegiorgis and Swerlick (see page 694 of this issue), it is apparent that clinicians in the Southeast are not familiar with the clinical presentation of pemphigus vulgaris and pemphigus foliaceous foliaceous /fo·li·a·ceous/ (fo?le-a´shus) foliate. since an average delay in proper diagnosis was from 4 to 6 months. Pemphigus vulgaris is usually limited at its onset, often confined to the oral mucosa. Pemphigus foliaceous also is usually limited and localized at the onset. The lesions often are well demarcated and scattered in a seborrheic seb·or·rhe·ic adj. Of, relating to, or affected by seborrhea. seborrheic affected with or of the nature of seborrhoea. seborrheic dermatitis see seborrheic dermatitis. pattern on the face, scalp, and upper trunk. The primary lesions of small flaccid flaccid /flac·cid/ (flak´sid) (flas´id) 1. weak, lax, and soft. 2. atonic. flac·cid adj. Lacking firmness, resilience, or muscle tone. blisters are often inconspicuous. It is generally agreed that pemphigus vulgaris at its earliest onset should be aggressively treated because it will ultimately generalize. The prognosis with early therapy is generally much better. Likewise, pemphigus foliaceous will ultimately generalize, making early diagnosis and treatment of utmost importance. Because mortality and morbidity will ultimately be affected by early diagnosis in these two closely related diseases, recognition of the two forms of pemphigus is critical. Timely referral to a dermatologist for appropriate skin and/or oral biopsies with immunofluorescent staining should be the major teaching objective of this article. Treatment can best be provided by an experienced dermatologist or teaching center with expertise in immunosuppressive Immunosuppressive Any agent that suppresses the immune response of an individual. Mentioned in: Antirheumatic Drugs, Graft-vs.-Host Disease, Immunosuppressant Drugs immunosuppressive 1. pertaining to or inducing immunosuppression. 2. and glucocorticoid therapy for pemphigus. Larry D. Hudson 1021W. Oakland Ave. Suite 102 Johnson City, TN 37604 References (1.) Beuntner EH, Jordan RE: Demonstration of skin antibodies in sera of pemphigus vulgaris patients by indirect immunofluorescent staining. Proc Soc Exp Biol Med 1064; 117:505 |
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