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Lortat-Jaccob and Degos syndrome

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A chronic bullous disease of elderly persons, prevalent in women. Average age of onset is 65 years. It is characterized by recurrent bullae of mucosal and skin zones adjacent to body orifices. The bullae of the oral mucosa, most commonly affecting the gingivae and edentulous ridges, develop from vesicles about 3 to 6 mm in diameter. Bullae eventually rupture and coalesce, causing mild scarring. Conjunctivae affected in most patients. No severe discomfort. Histologically, the lesions are similar to those seen in erythema multiforme. Aetiology unknown.

Se also Duhring's disease, under Louis Adolphus Duhring, American dermatologist, 1845-1913.

Bibliography

  • E. Lortat-Jacob:
    Benign mucosal pemphigoid, dermatite bulleuse muco-synéchtante et atrophiante.
    British Journal of Dermatology, Oxford, 1958, 70: 361-367.

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