Treatment Options and Outlook
Treatment targets relief of symptoms during out-
breaks and mitigation of future outbreaks to the
extent possible. Treatment varies with the form
and severity of pemphigus, though typically
includes oral or injected CORTICOSTEROID MEDICA-
TIONSalong with oral ANALGESIC MEDICATIONS for
PAINrelief and ANTIBIOTIC MEDICATIONSto treat infec-
tion when necessary. Severe outbreaks may
requireIMMUNOSUPPRESSIVE MEDICATIONSto subdue
theIMMUNE RESPONSE, or PLASMAPHERESIS, a therapy
that cleanses the blood’s serum of antibodies.
Cytotoxic drugs such as those used in CHEMOTHER-
APYalso improve symptoms in some people with
severe outbreaks.
The most serious complication of pemphigus
itself is loss of the skin’s ability to protect the body
from bacterial invasion, resulting in widespread
skin or systemic infection. However, pemphigus is
a chronic condition that requires ongoing medica-
tion therapy. For many people who have pemphi-
gus vulgaris, the more common and more severe
form, the most significant complications arise from
the long-term use of the medications necessary to
control outbreaks. These medications all have seri-
ous side effects and adverse consequences for
other body structures and functions. Pemphigus
that appears as an adverse DRUGreaction typically
ends when the person stops taking the medica-
tion. Paraneoplastic pemphigus improves with
treatment for the underlying cancer, though may
cause life-threatening pulmonary complications
when it affects the airways.
Risk Factors and Preventive Measures
Because doctors do not know the mechanisms
that set pemphigus in motion, there are no identi-
fied risk factors. The condition tends to first mani-
fest in people who are age 40 or older, though can
occur at any age. Researchers suspect GENEmuta-
tions underlie pemphigus as they do other AUTOIM-
MUNE DISORDERS, though have not yet been able to
identify them. Early diagnosis and aggressive
treatment are key to mitigating symptoms and
outbreaks, improving QUALITY OF LIFEas well as
helping preserve other structures and functions.
People who have milder forms of pemphigus may
go extended periods without symptoms.
See also ANTIBODY; BLISTER; BULLA; BULLOUS PEM-
PHIGOID; KERATINOCYTE; LESION; MUTATION.petechiae Smooth, reddened, pinpoint lesions
that result from microscopic bleeding under the
surface of the SKIN. Petechiae most commonly
appear on the lower legs though can appear any-
where on the body. The presence of petechiae is a
symptom that signals an underlying health condi-
tion that causes a low platelet count such as
THROMBOCYTOPENIA, LEUKEMIA, MONONUCLEOSIS, or
SYSTEMIC LUPUS ERYTHEMATOSUS(SLE). Platelets are
the body’s first-line response in the COAGULATION
process, clumping together (aggregating) to slow
bleeding. Antiplatelet therapy such as ASPIRIN THER-
APY, often prescribed for people at risk forHEART
ATTACKor STROKE, intentionally blocks the actions
of platelets and may also result in petechiae. The
appearance of petechiae, which often is sudden,
requires prompt evaluation from a doctor.
See also ECCHYMOSIS; LESION; MONONUCLEOSIS,
INFECTIOUS; PLATELET; PURPURA.photosensitivity A heightened reaction to sun-
light or other ultraviolet light that results in a RASH
or SUNBURNat much lower or shorter exposure
than would ordinarily cause sunburn. Photosensi-
tivity may develop as a reaction to a medication,
such as the antibiotic medication tetracycline or
the herbal antidepressant remedy ST. JOHN’S WORT
(hypericum), or as a symptom of an underlying
health condition such as SYSTEMIC LUPUS ERYTHE-
MATOSUS(SLE) or ALBINISM. Photosensitivity may
manifest as a red, splotchy rash on areas of SKIN
exposed to the sun or as a full-fledged sunburn.
Rarely, an individual may have an allergic reac-
tion to ultraviolet light that causes the fairly
immediate eruption of URTICARIA(hives) with sun
exposure.
The dermatologist can diagnose photosensitiv-
ity based on its presentation and a history of
recent sun exposure. Treatment may include topi-
cal or oral ANTIHISTAMINE MEDICATIONSif the rash
itches (although topical antihistamines can them-
selves increase sun sensitivity). Topical CORTICOSTE-
ROID MEDICATIONSoften help reduce INFLAMMATION
from widespread sunburn. However, the most
effective treatment for photosensitivity is preven-photosensitivity 183