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Copyright (c) 2020 Wolters Kluwer Health, Inc. All rights reserved.
Under the lens: Blue man syndrome
as an effect of amiodarone
By Lenel Caze, MS, CIC, EMT-P; Kirt Bowen, MPA, CIC, EMT-P, PA-C; Lorenzo Paladino, MD, EMT-P; and Tamara L. Bellomo, MSN, RN
Emergency Medical Services (EMS) person
nel notify a receiving facility that they're
enroute with a 68-year-old man experienc
ing shortness of breath who's had a produc
tive cough for 2 days. Upon arrival, the
patient is in moderate-to-severe respiratory
distress. He's on 100% oxygen via a non
rebreather mask and appears to be cyanotic.
EMS personnel state that the patient's
medical history is significant for atrial
fibrillation, chronic obstructive pulmo
nary disease (COPD), hypertension, and
hypothyroidism.
On admission to the ED, the nurse notes a
medication history of amiodarone, salmeterol,
metoprolol, and levothyroxine. The ECG mon
itor displays atrial fibrillation at a ventricular
rate of 126 beats/minute. Oxygen saturation
is 94% on room air after the patient is off
the non-rebreather mask for approximately
2 minutes. Auscultation reveals clear lungs
with no wheezing. The patient has a bluish
discoloration to his face and lower forearms
bilaterally. You suspect that the patient has
blue man syndrome based on his history of
taking amiodarone.
Pathophysiology
Blue man syndrome is a condition that
presents with ceruloderma-a bluish,
cyanotic-like skin hyperpigmentation
that's caused by long-term administra
tion of the antiarrhythmic medication
amiodarone (see The facts on amiodarone).
Although the symptomatic presentation
of blue man syndrome isn't life threat
ening, it may be due to the amiodarone
speeding up the natural, regulated cell
mechanism that removes unnecessary or
dysfunctional components (autophago
cytosis), which leads to an increased
production of lipofuscin that accumulates
in lysosomes. Men are more than twice
as likely as women to experience this
adverse reaction. Currently, there are no
studies to explain why.
The exact reason why amiodarone
causes the bluish discoloration of the skin
is unclear. Several different theories have
been hypothesized, including a drug-
induced metabolism disorder (lipidosis),
a photosensitivity reaction to UV light,
or skin hypersensitivity (leukocytoclastic
vasculitis). It's speculated that UV light
ARTCASTA / SHUTTERSTOCK
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Copyright (c) 2020 Wolters Kluwer Health, Inc. All rights reserved.
causes amiodarone and the end products
of its metabolism (metabolites) to attach
to the blood vessel walls and tissues. This
is associated with local vasodilatation and
increased dissemination of amiodarone
and its metabolites, resulting in chronic
tissue accumulation. Thus, only sun-
exposed regions of the body are affected.
How's it diagnosed?
For the healthcare team, formulating a
differential diagnosis is essential when
examining the patient who presents with
bluish discoloration of the skin because
there are various conditions that present
with similar findings, such as argyria and
methemoglobinemia (see Differentiating
blue man syndrome from other conditions).
Argyria is due to extended exposure
to silver salts either through ingestion
or contact. Toxic deposits can come from
occupational, medicinal, or chemical expo
sure. Argyria presents with skin that's
bluish-silverish-grayish in complexion.
This staining of the skin can also be seen in
the conjunctiva of the eyes and other muco
sal membrane areas. Histology will reveal
granules in the dermis, particularly evident
near the basal layer of sweat glands.
Methemoglobinemia is a result of the
presence of iron in ferric form instead of
the usual ferrous form, which, in turn,
results in a decreased availability of oxy
gen to the tissues. This decrease in oxygen
then causes a varying degree of cyanosis.
The condition can arise from a genetic
defect or, more commonly, a condition
acquired through exposure to certain
medications and chemicals, such as ben
zocaine and xylocaine; carcinogen ben
zene and nitrites used as meat additives;
or certain antibiotics, including dapsone
and chloroquine. The exposure can occur
through inhalation, ingestion, injection, or
skin and mucous membrane exposure.
Nursing considerations
EMS personnel must take due care in
noting the patient's presentation, the
The facts on amiodarone
Available in both injectable and oral form, amiodarone is a potent iodine-
containing antiarrhythmic medication that's widely used and highly effec
tive in the treatment of various atrial and ventricular arrhythmias. It's
absorbed from the gastrointestinal (GI) system and stored primarily in the
lungs, liver, spleen, and adipose tissue. The FDA has approved amioda
rone for the treatment of life-threatening ventricular arrhythmias; however,
it's commonly used to treat supraventricular tachyarrhythmias such as
atrial fibrillation, as well as for the prevention of ventricular tachyarrhyth
mias in high-risk patients. Proper dosage guidelines must be adhered to
when taking amiodarone and a general recommendation of lower doses
is preferred for the management of severe adverse reactions. Serum lab
values should be closely monitored for potential toxicity.
Besides blue man syndrome, other adverse reactions include:
-  central nervous system: malaise, fatigue, dizziness, tremors, ataxia,
paresthesias, and lack of coordination
-  cardiovascular: cardiac arrhythmias, congestive heart failure, cardiac
arrest, and hypotension
-  eye, ear, nose, and throat: corneal microdeposits (photophobia, dry eyes,
halos, blurred vision) and ophthalmic abnormalities, including permanent
blindness
-  endocrine: hypothyroidism or hyperthyroidism
-  GI: nausea, vomiting, anorexia, constipation, abnormal liver function
tests, and hepatotoxicity
-  respiratory: pneumonitis and infiltrates (shortness of breath, cough,
crackles, wheezes)
-  other: photosensitivity and angioedema.
Differentiating blue man syndrome from other conditions
-  Presentation: Always take the patient's clinical presentation into
account. Is the patient in distress? Tachypneic? Using accessory mus
cles? Appearing fatigued?
-  Vital signs: Assess oxygen saturation. Is the patient hypoxic?
Tachycardic?
-  History: Interview the patient. Is he or she taking amiodarone or recently
stopped taking it? Has the patient been aware of the skin pigmentation
(chronic, subacute) or is the condition acute?
course of the present illness, and his or
her past medical history, as well as carry
out a physical exam. This critical informa
tion must then be communicated to the
healthcare team. As an integral part of
the interdisciplinary team, nurses play a
vital role in treating patients with blue
man syndrome, including physical assess
ment, intervention, and education, as well
as addressing the patient's psychosocial
concerns.
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patho puzzler

Copyright (c) 2020 Wolters Kluwer Health, Inc. All rights reserved.
Nursing assessment. Take a complete
medical history, including the patient's
medication history and potential exposure
to chemicals. Assess for bluish discolor
ation of the skin, especially around areas
of the skin exposed to the sun, such as the
face and hands.
The physical exam should include an
evaluation of signs of respiratory distress
and impending respiratory failure such
as accessory muscle use. Auscultation of
lung sounds is also useful for ruling out
COPD, asthma, pneumothorax, pneumo
nia, or congestive heart failure as causes
of hypoxia.
Treatment. Discontinuation or lowering
of the amiodarone dosage is the mainstay
treatment. However, this is dependent on
whether the patient's current clinical sta
tus can tolerate a decrease in therapeutic
dosing or a substitute medication. Some
patients have no alternative but to toler
ate the adverse reaction until a change
in their condition allows for a substitute
therapy.
Intervention. Upon confirmed diagno
sis, anticipate amiodarone dosage reduc
tion or discontinuation. Carefully observe
for any cardiac dysrhythmias that may
occur as a result of the adjustment.
Education. Educate the patient about
the cause of blue man syndrome, treat
ment strategies, and when to notify the
healthcare provider. If the amiodarone
dosage is decreased or discontinued, teach
the patient that cardiac dysrhythmias may
occur. Educate the patient about the signs
and symptoms of potential dysrhythmias
and to notify the healthcare provider or
EMS immediately.
Psychosocial concerns. Although there
are no serious medical effects of the bluish
skin discoloration, the patient will likely
experience a body image disturbance.
Reassure the patient that although there's
no treatment for the skin discoloration,
avoiding sun exposure and/or lowering
the amiodarone dosage or discontinuing
amiodarone will most likely improve skin
color. To date, almost all patients who've
stopped amiodarone have had the condi
tion abate; however, the time to this end
point has varied.
Positive impact
Patients receiving amiodarone therapy
should be closely monitored on a regular
basis to assess for medication effective
ness, the development of adverse reac
tions, and potential drug interactions.
Blue man syndrome is a condition that's
directly related to amiodarone therapy.
It's essential for nurses to understand
this condition and know how to care for
patients who are experiencing it. A rapid
differential diagnosis, intervention, edu
cation, and addressing psychosocial con
cerns will have a positive impact on the
quality of patient care. 
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At Kingsborough Community College in Brooklyn, N.Y., Lenel Caze
is an , Lorenzo Paladino is the Medical Director,
and Tamara L. Bellomo is an Associate Professor. Kirt Bowen is
a Physician Assistant (Hospitalist) at St Luke's University Health
Network in Coaldale, Pa.
The authors have disclosed no financial relationships related to this
article.
DOI-10.1097/01.NME.0000653212.47123.2d
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