Ulcerative and oral lichen planus associated with sicca syndrome and primary biliary cirrhosis. 1994

G S Gart, and C Camisa
Department of Dermatology, Cleveland Clinic Foundation, Ohio.

The following case report supports the hypothesis that the coexistence of lichen planus, primary biliary cirrhosis, and sicca syndrome is not co-incidental. The fact that all three diseases are manifest in the same patient substantiates a theory of pathogenesis based on a cell-mediated immune response analogous to that in allogeneic bone marrow transplant recipients with chronic graft-versus-host disease.

UI MeSH Term Description Entries
D008010 Lichen Planus An inflammatory, pruritic disease of the skin and mucous membranes, which can be either generalized or localized. It is characterized by distinctive purplish, flat-topped papules having a predilection for the trunk and flexor surfaces. The lesions may be discrete or coalesce to form plaques. Histologically, there is a "saw-tooth" pattern of epidermal hyperplasia and vacuolar alteration of the basal layer of the epidermis along with an intense upper dermal inflammatory infiltrate composed predominantly of T-cells. Etiology is unknown. Cutaneous Lichen Planus,Lichen Planopilaris,Lichen Ruber Planus,Mucosal Lichen Planus,Lichen Rubra Planus,Lichen Planus, Cutaneous,Lichen Planus, Mucosal,Planopilaris, Lichen
D008105 Liver Cirrhosis, Biliary FIBROSIS of the hepatic parenchyma due to obstruction of BILE flow (CHOLESTASIS) in the intrahepatic or extrahepatic bile ducts (BILE DUCTS, INTRAHEPATIC; BILE DUCTS, EXTRAHEPATIC). Primary biliary cholangitis involves the destruction of small intra-hepatic bile ducts and decreased bile secretion. Secondary biliary cholangitis is produced by prolonged obstruction of large intrahepatic or extrahepatic bile ducts from a variety of causes. Biliary Cirrhosis,Biliary Cirrhosis, Primary,Biliary Cirrhosis, Secondary,Cholangitis, Chronic Nonsuppurative Destructive,Liver Cirrhosis, Obstructive,Primary Biliary Cholangitis,Biliary Cirrhosis, Primary, 1,Primary Biliary Cirrhosis,Secondary Biliary Cholangitis,Secondary Biliary Cirrhosis,Biliary Cholangitides, Primary,Biliary Cholangitis, Primary,Biliary Cholangitis, Secondary,Cholangitides, Primary Biliary,Cholangitis, Primary Biliary,Cholangitis, Secondary Biliary,Cirrhosis, Biliary,Cirrhosis, Secondary Biliary,Liver Cirrhoses, Biliary,Obstructive Liver Cirrhosis,Primary Biliary Cholangitides,Secondary Biliary Cholangitides
D005260 Female Females
D005533 Foot Dermatoses Skin diseases of the foot, general or unspecified. Foot Dermatosis,Dermatoses, Foot,Dermatosis, Foot
D006229 Hand Dermatoses Skin diseases involving the HANDS. Hand Dermatosis,Dermatoses, Hand,Dermatosis, Hand
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000368 Aged A person 65 years of age or older. For a person older than 79 years, AGED, 80 AND OVER is available. Elderly
D012859 Sjogren's Syndrome Chronic inflammatory and autoimmune disease in which the salivary and lacrimal glands undergo progressive destruction by lymphocytes and plasma cells resulting in decreased production of saliva and tears. The primary form, often called sicca syndrome, involves both KERATOCONJUNCTIVITIS SICCA and XEROSTOMIA. The secondary form includes, in addition, the presence of a connective tissue disease, usually rheumatoid arthritis. Sicca Syndrome,Sjogren Syndrome,Sjogrens Syndrome,Syndrome, Sicca,Syndrome, Sjogren's
D012883 Skin Ulcer An ULCER of the skin and underlying tissues. Skin Ulcers,Ulcer, Skin,Ulcers, Skin
D017676 Lichen Planus, Oral Oral lesions accompanying cutaneous lichen planus or often occurring alone. The buccal mucosa, lips, gingivae, floor of the mouth, and palate are usually affected (in a descending order of frequency). Typically, oral lesions consist of radiating white or gray, velvety, threadlike lines, arranged in a reticular pattern, at the intersection of which there may be minute, white, elevated dots or streaks (Wickham's striae). (Jablonski, Illustrated Dictionary of Dentistry) Oral Lichen Planus

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