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Hereditary mucoepithelial dysplasia (HMD), or simply mucoepithelial dysplasia, [1] [2] is a rare autosomal dominant multiepithelial disorder causing systemic maldevelopment of the epithelia and mucous membranes that line the surface of tissues and structures throughout the body, particularly affecting systems affiliated with mucosa, which includes the respiratory, digestive, urinary ...
The disease occurs on the bone-bound oral mucosa of the hard palate and alveolar ridges. Inflammatory papillary hyperplasia is usually asymptomatic. It presents as a cluster of individual papules or nodules that may be erythematous, somewhat translucent, or normal in surface coloration. Mucosa is erythematous and has a pebbly or papillary surface.
Vulvar Paget's disease affect women and presents as erythematous (red), eczematous lesions. [10] It is itchy and sometimes pain can be associated with the affected area. [10] The lesion is clearly separated from normal skin in most cases, and sometimes scattered areas of white scale can be present, giving a "strawberries and cream" appearance. [11]
Hereditary benign intraepithelial dyskeratosis is a rare [1] autosomal dominant disease of the conjunctiva and the oral mucosa caused by a duplication of chromosome 4q35. [2] In the mouth it appears similar to white sponge nevus , with painless, diffuse, folded and spongy white plaques. [ 2 ]
Junctional epidermolysis bullosa (JEB) is an inherited disease affecting laminin and collagen. This disease is characterized by blister formation within the lamina lucida of the basement membrane zone [13]: 599 and is inherited in an autosomal recessive manner. It also presents with blisters at the site of friction, especially on the hands and ...
The inflammation may include the perioral skin (the skin around the mouth), the vermilion border, or the labial mucosa. [1] The skin and the vermilion border are more commonly involved, as the mucosa is less affected by inflammatory and allergic reactions. [1] Cheilitis is a general term, and there are many recognized types and different causes.
Site – gingiva, buccal mucosa, alveolar mucosa, hard palate, floor of the mouth, larynx, oesophagus, penis, vagina, scrotum. Clinical presentation: It is a slow growing, diffuse, exophytic lesion usually covered by leukoplakic patches. Invasive lesions quickly invade bones.
Reticular erythematous mucinosis (REM) is a skin condition caused by fibroblasts producing abnormally large amounts of mucopolysaccharides. It is a disease that tends to affect women in the third and fourth decades of life.
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