Management of severe

psoriasis

Psoriatic erythroderma



Psoriasis is one of the leading causes of
erythroderma



1-2% fo psoriasis patients experience
erythroderma at some time in their life



patients with intense inflammatory lesions
are more unstable and likely to show
dissemination

but a far greater problem is erythroderma

secondary to unsuccesful therapy



In the past the major cause of
erythroderma was the reduction or
discontinuation of systemic
corticosteroids



This is the reason why such therapy is no
longer considered acceptable.



Also too aggressive topical therapy or UV
radiation in early eruptive stage of the
disease may trigger erythroderma.

Clinical findings



The patient is diffusely red with marked
desquamation and often exudation.



Usually clues as to the preexisting psoriasis
are somewhere to be found, sometimes history
helps.



Very rare patient presents almost de novo with
erythroderma and turns out to have psoriasis.



Pruritis is usually present



The patient is often systemically ill with
lymphadenopathy, fever, chills ; fluid
and proteines loss.



A severe complication is an acute
respiratory distress syndrome

Differential diagnosis



Drug eruptions,



Exacerbation of underlying skin disease
- lymphoma – especially mycosis
fungoides.

Pustular Psoriasis



Neutrophils in the epidermis are a
classic feature of psoriasis.



In this disease, pus does not equate
with infection.

Generalized Pustular Psoriasis

von Zumbush Type



This form of pustular psoriasis can be viewed
as the maximum variant of acute, explosive
psoriasis.



The patient is acutely ill with fever, chills and
often an elevated neutrophil count.



Multiple erythematous patches and plaques
dotted pustules cover wide areas of the body.



The pustules may coalesce.



Typically the palms and soles are
involved



In addition, the oral mucosa , genital
mucosa and even upper airways may
show pustules.



As the lesions dry out they become
scaly



Most often there is a trriger for von
Zumbusch pustular psoriasis



In the past it was often the
rapidreduction or termination of
systemic corticosteroid therapy



Other medications such as b-blokers or
antimalarial agents may also be
responsible, as well as pregnancy or oral
contraceptive use



The prognosis is serious- death may
occur



The patients lack effective antibacterial
activity and are at risk of lifethreatening
infections, especially pneumonia.



In addition the massive protein loss
leads to an enterpathy and associated
metabolic derangements.

Psoriatic Arthritis



Seronegative arthritis, often acral, which appears

in associatin with psorisis and has a relatively

typical radiologic appearance.



Psoriatic arthritis has a prevalence of 0.02 – 0.1 %



About 5-7 % of psoriasis patients have arthritis



Children are rarely affected



Over 70% of patients with psoriatic arthritis have

preexisting psoriasis, while in about 10% the two

problems appear in the same time period

Etiology and pathogenesis



The connection between the skin and
the the joints in psoriasis remains
somewhat a mystery.



Certain HLA markers are more common
and may have diagnostic and prognostic
sagnificance.



They include A26 B17 B27 Cw6 DR3
DR4 DR7

Clinical findings



At least 70% of patients with psoriatic
arthritis have nail changes



Affected finger is swelling.

Systemic therapy cytostatic

agents



Methotrexate - the folic acid antagonist



Helpful against psoriatic arthritis



Is usually administrated orally and well

absorbed, although it can be given

intramusculary or intravenously



It is excreted unchanged through the

kidneys, but also metabolized in the liver

to polyglutamated forms that are also

potent



Methotrexate is rarely administrated on
a continouous basis.



Instead it is given as pulse therapy



Most popular is the intermittent therapy
– MTX is given once weekly in three
divided doses 12 h apart – this approach
seems to maximize the antipsoriatic
effect and reduce the side effects

Adverse reactions



Hepatic fibrosis or even cirrhosis



Thrombocytopenia, leukopeniaanemia



Diarrhea,bleeding, ulcers



The RBC and patelet count should be checked

every 2 weeks, later every 4 weeks



Hepatic and renal function must be monitored



A baseline chest X-ray is needed, and

repaeted evaluation every 18-24 months

because of the risk of pulmonary fibrosis

Contraindications for methotrexate use in

psoriasis



Childhood



Renal and hepatic disease



Pregnancy or nursing



Desire for children



HIV/AIDS or other immunodeficiency



Chronic infections



Gastric or duodenal ulcer



Anemia, thrombocytopenia, leukopenia

cyclosporine



Cyclosporine A- has proven to be very
effective for treating severe psoriasis.



It has a rapid onset of action and is the
treatement of choice for severe or
explosive forms of both ordinary and
pustular psoriasis

While it is also helpful in psoriatic

artritis , the onset of action is slower

Cyclosporine is available for intravenous

and oral therapy.

the recommended initial dose is 2.5

mg/kg daily, dose can be gradually
increases to 5.0 mg/kg daily

The main side effects are renal

toxicity, hypertension and hepatic
damage.

Contradications for cyclosporine use in

psoriasis



Pregnancy



Decreased renal function



Sagnificant hypertension



Preexisting malignancy



HIV/AIDS or other chronic infections



Drug or alcohol abuse, current UV
radiation or PUVA therapy

Retinoids



The aromatic retinoids

acitretin

and

etretine

are the major option in treating severe
psoriasis: * generalized pustular

psoriasis

* psoriatic erythroderma

Retinoids



Acitretin is the active form of etretinate



both products have a significant effect
on psorisis:



- blocking epidermal proliferation,



- improving differentiation



- modulating the inflammatory or
immune response

Retinoids



As a monotherapy they are not as effective
as methotrexate, but they are often
combined with other topical modalities or
UV radiation.



After 8-12 weeks of therapy about 70% of
patients have a stisfactory response



Acitretin is prescribed in a single daily dose
of 30-50 mg irrespective of the body weight

Retinoids - contraindications



Patient of child bearing years – because of
teratogenicity



A contraception for 2 years after the end of
treatment are strongly recommended.



Side effects: dryness of the lips, diffuse hair loss



50% of patients develop elevated cholesterol
and TG levels, while 25% abnormalities of liver
function tests