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ISOTRETINOINACCUTANEACNEPREGNANCYCYSTICFETALHEALTHMEDICINE
ISOTRETINOIN (ACCUTANE) TREATMENT OF ACNE
Isotretinoin (Accutane) treatment of acne
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It has long been known that vitamin A has a regulatory effect
on growth and differentiation of epithelial tissue. Since the
1940's, high doses of vitamin A have been employed in the
treatment of severe acne and various disorders of keratinization.
Vitamin A's use, however, was limited by its side effects - liver
toxicity and pseudotumor cerebri in particular. In an attempt to
find a drug with a better therapeutic index than vitamin A,
several synthetic retinoids have been developed (1).
One of these, isotretinoin (13-cis-retinoic acid), was first
synthesized in 1955. It was largely ignored until the results of
the first major clinical trial demonstrating its efficacy in the
treatment of severe recalcitrant cystic acne were published in
1972 (2). in 1982, isotretinoin was approved in the United States
for that single indication only. Its trade name is Accutane. A
major feature of this drug is that it produces a prolonged
remission in patients with severe cystic acne, many of whom had
not responded to other therapies. The most striking discovery,
however, is that the remission is often sustained for months or
years after the four- to five-month treatment course has been
completed.
Transport and metabolism of vitamin A (retinol) and isotretinoin
Significant differences between vitamin A and isotretinoin
with regard to their transport and metabolism account for their
difference in toxicity (3). The major source of vitamin A
(retinol) is the conversion of dietary plant carotenoids in the
intestinal mucosa. Retinol is stored in the liver, which contains
over 90% of body stores. Mobilization from the liver is
accomplished when retinol is bound to a specific transport
protein, retinol binding protein, that delivers it to tissues.
Plasma levels of vitamin A tend to remain constant despite wide
variations in diet. Extremely high dietary intake (eg. polar bear
liver, vitamin A tablets) produces hypervitaminosis A.
Hypervitaminosis A results in greatly increased hepatic stores
and subsequent toxicity.
In contrast, isotretinoin binds to serum albumin. Isotretinoin
is readily absorbed orally and put into circulation via the
portal system. It is not significantly stored in any organ
system, and plasma levels vary with the amount ingested.
Mode of action of isotretinoin in the treatment of acne
Acne is a disease of the pilosebaceous unit. The pathogenesis
of acne is believed to include several factors. Among them are
excessive sebum production, abnormal keratinization of follicular
epithelium, proliferation of 'Propionibacterium acnes',
inflammation, and hormonal regulation of sebaceous glands.
Isotreinoin affects several of these mechanisms, but its exact
mode of action is not known. Isotretinoin causes pronounced but
temporary inhibition of sebum production and atrophy of sebaceous
glands (4). In general, after two weeks of standard therapy
(1mg/kg/day), sebum production is decreased by more than 50% and
by the end of a four- to five- month treatment period, it is
reduced by greater than 90%. While there are some patients who
continue to have decreased sebum production months to years after
cessation of therapy, the sebum production of the vast majority
returns to the baseline level within a few months. Since sebum
production returns to normal in most patients, this is obviously
not the sole mechanism for the prolonged remission of acne seen
in these individuals. Many of these patients continue to improve
even after therapy is discontinued (5).
Some investigators believe that isotretinoin inhibits
follicular keratinization in patients with acne. They postulate
that this prevents the formation of comedomes (whiteheads and
blackheads), the precursors of inflammatory acne lesions.
Isotretinoin does decrease the quantity of 'Propionbacterium
acnes' in treated patients. This organism is normal flora within
the follicles. Increased numbers of these bacteria in patients
with acne produce various chemotactic factors and enzymes that
may increase inflammation. The decreased number of these bacteria
in isotretinoin-treated patients is thought to be a result of
decreased sebum production, producing a poor environment for the
proliferation of this organism. Therefore, the decreased
bacterial counts are thought to be secondary, and not the primary
mechanism of action of Accutane.
Clinical side effects
Mucocutaneous side effects are the most common seen in
isotretinoin treatment (6). More than 90% of people treated
experience cheilitis, usually within the first two weeks of
treatment. This is most easily treated with bland emolliation. A
generalized xerosis is very common, but frequently acne patients
view this as a beneficial rather than adverse effect. Should
xerosis become problematic, the use of a noncomedogenic emollient
lotion can be employed. Frequently patients develop nose bleeds
and non-infectious conjunctivitis. Symptomatic treatment of these
problems is all that is indicated. Less than 30% of patients
notice temporary thinning of the hair. Less than 10% of these
have clinically apparent hair loss, and hair density returns to
normal after treatment is discontinued.
Approximately 20% of patients on isotretinoin have musculo-
skeletal pains. These tend to be minor, and are relieved by non-
steroidal anti-inflammatory agents. More worrisome are the
skeletal abnormalities seen in patients on long term isotretinoin
treatment for various keratinizing disorders (7). These patients
however , were usually treated with higher doses of 2mg/kg/day
for more than two years. The ossification disorder seen in these
patients resembles diffuse idiopathic skeletal hypostosis.
Children being treated for these disoreders had x-ray findings
suggesting premature closure of the epiphyses of the knees.
More recently, in a small prospective study of patients on
doses of 2 mg/kg/day of isotretinoin for the treatment of
keratinizing disorders, radiologically documented skeletal
changes were noted after only 6-12 months of therapy. These
changes consisted of slightly increased bone formation in areas
of ligament attachment. It is not yet known whether this is
reversible.
A small number of patients may have a temporary flare of acne
at the onset of treatment. Some clinincians feel that this can be
minimized by the administration of low dose prednisone or oral
antibiotics.
Pseudotumor cerebri ( benign intracranial hypertension) has
occurred in patients treated with isotretinoin. Patients with
headache, nausea, vomitting or visual disturbances should be
screened for papilledema. The drug should be stopped immediately
if papilledema is present.
Patients with visual disturbances should also be checked for
corneal opacities. Corneal opacities occur more frequently in
those patients receiving higher doses of isotretinoin for
keratinizing disorders but has also been reported in patients
treated for cystic acne. The opacities resolve six to seven weeks
after discontinuation of the drug.
There have been rare reports of inflammatory bowel disease in
patients treated with isotretinoin. Occasional patients report
fatigue or lassitude.
Laboratory abnormalities
One fourth of patients treated with isotretinoin develop
elevated serum triglycerides during their four- to five- month
course of treatment for acne. Approximately one eighth have a
decrease in the HDL level and one sixteenth have elevated
chloresterol. These changes usually resolve after therapy is
discontinued. It is important to monitor blood lipids at the
onset of therapy and at intervals of two to four weeks until it
is seen that there are no significant changes. Minor elevations
in triglycerides are best treated by dietary maneuvres. It is
not known what role the lipid abnormalities may play in the
production of coronary heart disease, but the risk is currently
thought to be low since abnormalities return to normal after
therapy is stopped. It is perhaps best that patients with high
triglyceride elevations be treated with the lowest effective
dosage of isotretinoin for a shorter period of time. Triglyceride
elevations of greater than 500 mg/dL necessitate discontinuation
of the drug, as these patients are at risk for developing acute
pancreatitis. A few patients develop mild leukopenia, anemia, or
thrombocytosis. Mild pyuria and liver function test abnormalities
occasionally occur. These changes appear to be reversible after
discontinuation of the drug and are usually not clinically
significant.
Isotretinoin and pregnancy
The most serious side effect of isotretinoin is its
teratogenicity. The drug should not be used by women who are
pregnant or those who plan to become pregnant during treatment.
Contraception is recommended for women taking the drug, beginning
one month before treatment, and continuing until one month after
discontinuation of treatment. Many physicians obtain a pregnancy
test within two weeks prior to starting therapy.
Reported fetal abnormalities include hydrocephalus,
microcephalus, abnormalities of the external ear (micropinna,
small or absent external auditory canals), Micropthalmia and
cardiovascular abnormalities. These abnormalities have occured
only in children born to mothers exposed to the drug during
pregnancy.
The package insert recommends that patients who become
pregnant discuss with their physician the desirability of
continuing the pregnancy. Since the half-life of isotretinoin is
less than one to three days, it is believed that there is no risk
of teratogenicity during subsequent pregnancies occurring at
least one month after treatment has been stopped.
While no reproduction studies have been performed on humans
taking isotretinoin, studies in rats have not revealed impaired
fertility. No significant changes have been seen in the number or
mobility of spermatazoa of human males receiving isotretinoin. As
it is not known whether isotretinoin is secreted into milk, it is
inadvisable to treat nursing mothers with the drug.
Dosage and administration
Isotretinoin is presently approved by the FDA only for the
treatment of severe recalcitrant nodulocystic acne. While dosages
of 0.1, 0.5, and 1.0 mg/kg/day orally seem equally effective in
inducing a remission of acne, there is a significantly higher
relapse rate in patients rate in patients treated at lower doses.
As the goal of isotretinoin therapy is not only the clearing of
active disease but the indefinite maintenance of clearing, it is
recommended that patients be treated with 1 mg/kg/day for 16-20
weeks. Extensive truncal lesions, which do not respond as quickly
or completely as facial lesions, may require dosages of 1.5-2.0
mg/kg/day. Isotretinoin is supplied in 10,20, and 20 mg capsules.
Most patients are treated with 40 mg twice daily. Patients should
be advised not to ingest any vitamin A supplements during their
treatment course.
Other indications
Isotretinoin has also been shown to be effective for a variety
of other skin disorders, but it is not yet approved by the FDA
for these indications. These include several variants of acne:
hidradenitis suppurativa, gram negative folliculitis, and
rosacea.
Retinoids also have an antineoplastic effect. Several patients
with the basal cell nervus syndrome, an autosomal dominant
disorder in which patients develop multiple basal cell
carcinomas, have been treated. Isotretinoin resulted in reduction
in tumor sizes, but usually did not completely destroy the
tumors. Retinoids are also being studied for possible future
roles in cancer prevention. Isotretinoin and other retinoids (
especially etretinate, an aromatic retinoid not yet available in
the US) have been used to treat a wide variety of keratinizing
skin disorders, such as psoriasis, keratosis follicularis,
ichthyosis, pityriasis rubra pilaris, and others (8). Discussion
of this is beyond the scope of this paper. Although the drug is
very effective for several of these disorders, the major problem
is that long-term treatment is required for these chronic
diseases, and long-term safety of retinoids has not been
established.
Conclusion
Isotretinoin (Accutane) is an exceedingly useful drug,
producing dramatic prolonged remissions after four to five weeks
in most patients with severe nodulocystic acne. Because of its
side effects, teratogenicity, and cost (about $600 for a four- to
five- month course of treatment), the drug should be reserved for
the less than 1% of patients with severe acne.