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The term pemphigus refers to a rare group of autoimmune intraepidermal diseases characterized by blistering, pustules, or vesicles on the skin and mucous membranes. The mode of action of the disease is still not clear, but a key component is acantholysis, the disruption of the normal mechanisms of intercellular adhesion, which leads to intraepidermal blister formation.
There are several types of presentations of this disease such as p. vulgaris, p. foliaceus, p. vegetans, etc. One catastrophic presentation of this disease is ocular cicatricial pemphigoid. The pemphigoid disease progresses creating a symblepharon (adhesive attachments between the conjunctiva covering the sclera and the mucosa covering the posterior aspect of the eyelids. Eventually the disease may extend over the cornea. The accompanying image depicts a case of complete keratinization of the ocular surface in a patient with ocular cicatricial pemphigoid.
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Complete keratinization of the ocular surface in patient with ocular cicatricial pemphigoid.
Click on the image for a larger depiction |
| The root term pemphig- derives from the Greek [πεμφίγος] meaning a pustule or blister; the suffix -oid is also Greek [ειδής] meaning “similar to” of “kind of”. Therefore the medical term pemphigoid means “similar to blisters”
There is discussion as to when was this word first used, but it looks as though it was first published in 1763 in the book “Pathologia Methodica Practica, seu de Cognoscendis Morbis” by the French physician and botanist Francois Boissier de la Croix de Sauvages (1706 – 1767)
Sources:
1. “Revue D’Histoire Des Sciences” Louis Dulieu, 1969
2. "Etymology of Pemphigus" Holubar, K. J Am Acad Dermat 1989:21, 155-156
3. "Pemphigus" Korman, N. J Am Acad Dermat 1988: 18/6 1219-38
4. “Ocular Cicatricial Pemphigoid” Khan R,. McDermott M., Hwang, F. Am Acad Ophthalm Eye Wiki https://eyewiki.aao.org/Ocular_cicatricial_pemphigoid
Image courtesy of EyeWiki
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