Skin Disorders Common in Primary Biliary Cirrhosis

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http://www.medscape.com/viewarticle/528418_print

March 24, 2006 - Patients with primary biliary cirrhosis (PBC) have a wide

variety of cutaneous manifestations, Greek researchers report in the March

issue of the American Journal of Gastroenterology. This can be helpful in

the diagnosis of PBC, and in fact a dermatological condition may be the

presenting sign or symptom.

Lead investigator Dr. Meri Koulentaki told Reuters Health that the disease,

of probable autoimmune etiology, " affects the small intrahepatic bile ducts

of mainly middle-aged women, leading to liver cirrhosis. Early-stage PBC may

be oligo- or asymptomatic and therefore the diagnosis often is made at the

late disease stages. "

PBC is commonly associated with skin conditions, including pruritus and

melanosis, and to characterize such manifestations Dr. Koulentaki and

colleagues at University Hospital, Heraklion, Greece, compared 49 patients

with 45 matched controls.

In all, 330 instances of skin disorder were found in the patients with PBC

compared with 76 in the control subjects. In addition, 43 of the patients

had dermatological symptoms versus 14 of the controls.

The most common disorders seen in the patients were fungal infections

(31.5%). Also common were neoplastic lesions (18.4%), dermatitis-urticaria

(15.7%), and disturbances in pigmentation (12.4%).

Overall, 69.3% of patients had pruritus, the same proportion had xerosis,

and 57.1% had dermographism. Corresponding proportions in controls were,

22.2%, 2.2% and 4.4%. No controls had melanosis but this was seen in 46.9%

of patients.

Thus, continued Dr. Koulentaki, " dermatologic lesions -- most commonly

cutaneous fungal infections -- are very common even in the early stages of

biliary cirrhosis. Indeed, in more than one third of our patients, the

dermatologic lesion was the presenting sign or symptom leading to

diagnosis. "

Physicians should be aware of these manifestations, Dr. Koulentaki

concluded, " in order to achieve a prompt and early diagnosis, since early

treatment could alter the disease progression. "

Am J Gastroenterol. 2006;101:541-546

Clinical Context

PBC is a probable autoimmune disorder which targets small intrahepatic bile

ducts. Middle-aged women are the most common group to be affected by PBC.

The disease may be suggested by the presence of M2 anti-mitochondrial

antibodies (AMA), which are present in approximately 95% of cases. However,

AMA-positive and AMA-negative cases have a similar clinical and histological

disease profile, with 4 histological stages from inflammation to cirrhosis.

A number of dermatologic conditions have been associated with PBC. However,

systematic descriptions of these skin diagnoses are lacking. Therefore, the

authors of the current study performed a case-control analysis of

dermatologic symptoms, signs, and diagnoses in comparing a cohort of

patients with PBC with a group of control patients recruited from a

dermatology practice.

Study Highlights

* The research compared 49 consecutive individuals with PBC attending

one liver outpatient clinic with 45 age- and sex-matched control subjects

attending a dermatology clinic.

* All study subjects were examined by 2 dermatologists who were aware

of their diagnoses. The main study outcome was the incidence of dermatologic

symptoms, signs, and diagnoses among PBC patients vs controls.

* The median age of the cohort was 63 years. All participants with PBC

had histologically confirmed disease, and 45 were AMA-positive. Patients

with PBC were all receiving ursodeoxycholic acid, but none had received

D-penicillinamine.

* All subjects with PBC had at least 2 skin lesions. Subjects with PBC

had a total of 330 skin disorders compared with 76 in the control group.

* The most common skin disorders among subjects with PBC included

fungal infections (31.5% of patients), neoplastic lesions (18.4%),

dermatitis-urticaria (15.7%), and disturbances of pigmentation (12.4%).

* On all analyses, fungal skin infections were more common among

subjects with PBC vs controls, including interdigital mycoses (20 cases vs 1

case), plantar mycoses (19 vs 0 cases), and onychomycoses (24 cases vs 1

case). Melanosis was also more common among participants with PBC on all

analyses (23 vs 0 cases).

* On initial analysis, other skin conditions more commonly associated

with PBC included seborrheic keratosis, cherry hemangioma, varicose veins,

inguinal epidermophytoses, angular cheilitides, hand and foot eczema, atopic

dermatitis, xanthelasmas, and senile lentigines. However, after correction

for multiple comparisons, these conditions were not significantly more

common among patients with PBC compared with control subjects.

* Patients with PBC were more likely to have pruritis, xerosis cutis,

and dermographism compared with control subjects. 46.9% of subjects with PBC

had all 3 symptoms/signs. Erythema was not more common among participants

with PBC.

* 38.7% of subjects with PBC presented with either symptoms or signs

of dermatologic disease at least 9 weeks prior to the diagnosis of PBC.

* Among participants with PBC, there was no correlation between

specific dermatologic symptoms or diagnoses and subjects' age, duration of

disease, or the presence of AMA.

[Guest guest]

thank for posting this, very

interesting

>

> http://www.medscape.com/viewarticle/528418_print

>

>

>

> March 24, 2006 - Patients with primary biliary cirrhosis (PBC) have

a wide

> variety of cutaneous manifestations, Greek researchers report in

the March

> issue of the American Journal of Gastroenterology. This can be

helpful in

> the diagnosis of PBC, and in fact a dermatological condition may be

the

> presenting sign or symptom.

>

> Lead investigator Dr. Meri Koulentaki told Reuters Health that the

disease,

> of probable autoimmune etiology, " affects the small intrahepatic

bile ducts

> of mainly middle-aged women, leading to liver cirrhosis. Early-

stage PBC may

> be oligo- or asymptomatic and therefore the diagnosis often is made

at the

> late disease stages. "

>

> PBC is commonly associated with skin conditions, including pruritus

and

> melanosis, and to characterize such manifestations Dr. Koulentaki

and

> colleagues at University Hospital, Heraklion, Greece, compared 49

patients

> with 45 matched controls.

>

> In all, 330 instances of skin disorder were found in the patients

with PBC

> compared with 76 in the control subjects. In addition, 43 of the

patients

> had dermatological symptoms versus 14 of the controls.

>

> The most common disorders seen in the patients were fungal

infections

> (31.5%). Also common were neoplastic lesions (18.4%), dermatitis-

urticaria

> (15.7%), and disturbances in pigmentation (12.4%).

>

> Overall, 69.3% of patients had pruritus, the same proportion had

xerosis,

> and 57.1% had dermographism. Corresponding proportions in controls

were,

> 22.2%, 2.2% and 4.4%. No controls had melanosis but this was seen

in 46.9%

> of patients.

>

> Thus, continued Dr. Koulentaki, " dermatologic lesions -- most

commonly

> cutaneous fungal infections -- are very common even in the early

stages of

> biliary cirrhosis. Indeed, in more than one third of our patients,

the

> dermatologic lesion was the presenting sign or symptom leading to

> diagnosis. "

>

> Physicians should be aware of these manifestations, Dr. Koulentaki

> concluded, " in order to achieve a prompt and early diagnosis, since

early

> treatment could alter the disease progression. "

>

> Am J Gastroenterol. 2006;101:541-546

>

>

> Clinical Context

>

>

> PBC is a probable autoimmune disorder which targets small

intrahepatic bile

> ducts. Middle-aged women are the most common group to be affected

by PBC.

> The disease may be suggested by the presence of M2 anti-

mitochondrial

> antibodies (AMA), which are present in approximately 95% of cases.

However,

> AMA-positive and AMA-negative cases have a similar clinical and

histological

> disease profile, with 4 histological stages from inflammation to

cirrhosis.

>

> A number of dermatologic conditions have been associated with PBC.

However,

> systematic descriptions of these skin diagnoses are lacking.

Therefore, the

> authors of the current study performed a case-control analysis of

> dermatologic symptoms, signs, and diagnoses in comparing a cohort of

> patients with PBC with a group of control patients recruited from a

> dermatology practice.

>

>

> Study Highlights

>

>

> * The research compared 49 consecutive individuals with PBC

attending

> one liver outpatient clinic with 45 age- and sex-matched control

subjects

> attending a dermatology clinic.

> * All study subjects were examined by 2 dermatologists who were

aware

> of their diagnoses. The main study outcome was the incidence of

dermatologic

> symptoms, signs, and diagnoses among PBC patients vs controls.

> * The median age of the cohort was 63 years. All participants

with PBC

> had histologically confirmed disease, and 45 were AMA-positive.

Patients

> with PBC were all receiving ursodeoxycholic acid, but none had

received

> D-penicillinamine.

> * All subjects with PBC had at least 2 skin lesions. Subjects

with PBC

> had a total of 330 skin disorders compared with 76 in the control

group.

> * The most common skin disorders among subjects with PBC

included

> fungal infections (31.5% of patients), neoplastic lesions (18.4%),

> dermatitis-urticaria (15.7%), and disturbances of pigmentation

(12.4%).

> * On all analyses, fungal skin infections were more common among

> subjects with PBC vs controls, including interdigital mycoses (20

cases vs 1

> case), plantar mycoses (19 vs 0 cases), and onychomycoses (24 cases

vs 1

> case). Melanosis was also more common among participants with PBC

on all

> analyses (23 vs 0 cases).

> * On initial analysis, other skin conditions more commonly

associated

> with PBC included seborrheic keratosis, cherry hemangioma, varicose

veins,

> inguinal epidermophytoses, angular cheilitides, hand and foot

eczema, atopic

> dermatitis, xanthelasmas, and senile lentigines. However, after

correction

> for multiple comparisons, these conditions were not significantly

more

> common among patients with PBC compared with control subjects.

> * Patients with PBC were more likely to have pruritis, xerosis

cutis,

> and dermographism compared with control subjects. 46.9% of subjects

with PBC

> had all 3 symptoms/signs. Erythema was not more common among

participants

> with PBC.

> * 38.7% of subjects with PBC presented with either symptoms or

signs

> of dermatologic disease at least 9 weeks prior to the diagnosis of

PBC.

> * Among participants with PBC, there was no correlation between

> specific dermatologic symptoms or diagnoses and subjects' age,

duration of

> disease, or the presence of AMA.

>

>

>

>

>

>