Dermatological manifestations in primary biliary cirrhosis patients: a case control study.
OBJECTIVE
Primary biliary cirrhosis (PBC), a disease of probable autoimmune etiology that affects the small intrahepatic bile ducts of mainly middle-aged women is commonly associated with pruritus, xanthomatous lesions, and melanosis. We conducted a prospective study to systematically describe the skin disorders of a group of PBC patients.


METHODS
A prospective evaluation and analysis of dermatological manifestations including oral and genital lesions was carried out, in 49 PBC patients (45 females and 4 males). Median age 63 yr (range 35-87 yr). They were compared with 45 age and sex matched controls, selected among persons attending the dermatologic outpatient clinic.


RESULTS
A total of 330 skin disorders were found in the 49 PBC patients versus 76 in the 45 controls; 31.5% of all lesions were skin fungal infections. Of all lesions analyzed with the Bonferroni rule of multiple comparisons significantly more common in PBC patients were plantar mycoses, onychomycoses, and interdigital mycoses. Pruritus was found in 69.3% of patients versus 22.2% of controls, xerosis in 69.3%versus 2.2%, dermographism in 57.1%versus 4.4%, and melanosis in 46.9%versus 0%. In 38.7% of the PBC patients the dermatologic lesion was the presenting symptom.


CONCLUSIONS
PBC patients present with a wide variety of cutaneous manifestations varying in severity. Multiple skin fungal infections have been found even in the early stages. Since in more than one third of our PBC patients the dermatologic lesion was the presenting sign or symptom leading to diagnosis we believe that physicians should be aware so that a prompt and early diagnosis may be achieved.
Koulentaki
Meri
M
Department of Gastroenterology, University Hospital Heraklion, PO Box 1352, 71110 Heraklion, Crete, Greece.
Ioannidou
Despina
D
Stefanidou
Maria
M
Maraki
Sofia
S
Drigiannakis
I
I
Dimoulios
Philippas
P
Melono
Jean Marie Enele
JM
Tosca
Androniki
A
Kouroumalis
Elias A
EA
D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D008105:Liver Cirrhosis, Biliary / Q000150:complications / Q000175:diagnosis*; D008297:Male; D008875:Middle Aged; D011446:Prospective Studies; D012871:Skin Diseases / Q000175:diagnosis / Q000209:etiology*; D013223:Statistics as Topic