Download A 13-Year-Old Boy with Pharyngitis, Oral Ulcers, and Dehydration

Case Challenge:
Dermatology
A 13-Year-Old Boy with Pharyngitis,
Oral Ulcers, and Dehydration
Leanne W. Trapp, MD; Steven J. Schrantz, MD; Monica A. Joseph-Griffin, MD;
Joseph R. Hageman, MD; and Shoshana E. Waskow, MD
A
13-year-old boy with no significant past medical history
was admitted to our institution
with pharyngitis, oral ulcers, and dehydration. He was well until 8 days prior
to admission when he developed a fever
to 103o F and pharyngitis. He was seen
by his primary care physician, who orLeanne W. Trapp, MD, is a Resident in Pediat-
rics, Comer Children’s Hospital, Pritzker School
dered a rapid strep test and throat culture, both of which were negative, so
symptomatic care was recommended.
Four days prior to admission the patient developed ulcers on the inside of
his mouth and lips. He was seen again
by his primary care physician and started on antiviral medication and ibuprofen
with hydrocodone, and a herpes simplex virus–polymerase chain reaction
test was ordered. Mouth pain became
so severe that the patient was unable to
remain adequately hydrated. He was admitted to the hospital for pain control,
intravenous fluids, and further work-up.
In the 2 days prior to presentation to
the hospital, he developed painful lesions on the glans of his penis. Recent
medication use included NyQuil (Vicks),
Theraflu (Novartis), Famvir (Novartis)
and Vicoprofen (Abbott). The patient
denied any history of sexual activity or
of Medicine, University of Chicago. Steven J.
Schrantz, MD, Department of Medicine, Division
of Infectious Diseases NorthShore University
HealthSystem; and Clinical Assistant Professor,
University of Chicago Pritzker School of Medicine.
Monica A. Joseph-Griffin, MD, is Clinical Assistant
Professor, Department of Pediatrics, NorthShore
University HealthSystem; and Clinical Assistant
All images courtesy of Leanne W. Trapp, MD.
Professor, Pritzker School of Medicine, University
of Chicago. Joseph R. Hageman, MD, is Emeritus
Attending Physician, Department of Pediatrics,
NorthShore University HealthSystem; and Senior
Clinician Educator, Pritzker School of Medicine,
University of Chicago. Shoshana E. Waskow, MD,
is an independent practitioner, Pediatric Associates of the NorthShore.
Figure 1. Cracked, scabbed lips with ulcerations.
Address correspondence to: Leanne W. Trapp,
MD, Comer Children’s Hospital, 5721 S. Maryland
Avenue, Chicago, IL 60637; fax: 773-834-0748;
email: [email protected].
Disclosure: The authors have no relevant financial relationships to disclose.
doi: 10.3928/00904481-20130326-06
148 | Healio.com/Pediatrics
For diagnosis, see page 149
Editor’s note: Each month, this department features a discussion of an unusual diagnosis in genetics, radiology, or dermatology. A description and images are presented,
followed by the diagnosis and an explanation of how the diagnosis was determined. As
always, your comments are welcome via email at [email protected].
PEDIATRIC ANNALS 42:4 | APRIL 2013
Case Challenge
oral sex. Review of systems was positive
for a mild, nonproductive cough 1 week
prior to admission, but negative for rhinorrhea, vomiting, diarrhea, shortness of
breath, chest pain, arthralgia/joint pain,
change in vision, headaches, dysuria, or
frequency; fevers had resolved 4 days
prior to admission.
PHYSICAL EXAMINATION
On admission, the patient appeared
tired and mildly dehydrated. He was
afebrile with a pulse of 107 beats per
minute, blood pressure of 118/52 mm
Hg, and respiratory rate of 20 breaths
per minute. His conjunctivae were
mildly injected bilaterally. Lips were
scabbed with dried blood present (see
Figure 1, page 148). Buccal mucosa
and gums were erythematous with multiple erosive lesions and white plaques
(see Figure 2). Tonsils were enlarged 3+
bilaterally, and erythematous with exudates present. The cardiac examination
revealed normal rate and rhythm with
no murmur. The lungs were clear to
auscultation bilaterally with no wheezing or crackles present. Head of penis
was erythematous with vesicles present and scab formation at the urethral
meatus (see Figure 3). No other rashes
were noted on his skin.
HOSPITAL COURSE
The patient was given an intravenous (IV) normal saline bolus in the
emergency department and 1 dose of
dexamethasone IV (0.6 mg/kg). He was
admitted to the general pediatrics floor
and started on maintenance IV fluids
and IV pain medication. The herpes
simplex virus–polymerase chain reaction (HSV-PCR) test of an oral lesion
done at his pediatrician’s office was
negative for HSV 1 and 2, and the antiviral medication was discontinued. He
was continued on IV solumedrol for 2
days but it was discontinued after no
improvement in symptoms was noted.
Initial laboratory work-up included
PEDIATRIC ANNALS 42:4 | APRIL 2013
Figure 2. Cracked, scabbed lips with ulcerations
and erythematous tongue with ulcerations.
serum electrolytes, blood urea nitrogen,
creatinine, liver function tests, complete
blood count with differential, mono-spot
test, urinalysis, and urine culture, all of
which were normal. C-reactive protein
was elevated at 64.2 mg/L and erythrocyte sedimentation rate was 35 mm/
hour.
On day 2 of hospitalization, the patient
developed lesions in his nares bilaterally
and crusting of lower eyelids bilaterally
with continued conjunctival injection.
He was also noted to have scattered vesicular lesions on his scrotum but no rash
anywhere else. Further work-up included
Epstein-Barr virus and cytomegalovirus
titers, HLA-B27, and HIV antibody, all
of which were negative. Cold agglutinin
test was performed on hospital day 2 and
was negative.
He was started on a 5-day course of
azithromycin for possible Mycoplasma
pneumoniae infection while awaiting
the results of Mycoplasma antibody
tests. Ophthalmology was consulted due
to lesions in the eye and work-up was
negative for corneal or lens involvement.
Mucositis slowly began to improve over
course of hospitalization. On hospital
day 4, Mycoplasma immunoglobulin M
(IgM) was reported to be positive and the
patient was diagnosed with Mycoplasma
pneumoniae-associated mucositis.
Figure 3. Erythematous head of penis with
crusting.
Diagnosis:
Mycoplasma pneumoniaeAssociated Mucositis
DISCUSSION
Mycoplasma pneumoniae is a freeliving organism that lacks a cell wall.
It most commonly causes communityacquired atypical pneumonia in children. The most common symptoms are
nonproductive cough, myalgias, sore
throat, headache, and dyspnea. Though
less common, Mycoplasma pneumoniae infection has been associated with
several extrapulmonary manifestations
including neurologic complications
such as aseptic meningitis or encephalitis, cardiac complications such as
pericarditis or myocarditis, renal complications such as glomerulonephritis
or acute renal failure, and hematologic
complications such as hemolysis.1
Mycoplasma pneumoniae is also a
rare but well-known cause of Stevens–
Johnson syndrome (SJS). SJS occurs
in 1% to 5% of cases of Mycoplasma
pneumoniae infection.1 According to
the classification by Bastuji-Garin and
colleagues,2 SJS is characterized by extensive erosion of the skin with poorly
defined circular lesions and often with
Healio.com/Pediatrics | 149
Case Challenge
at least one mucosal surface involved.2
Less than 10% of the body surface area
is involved in epidermal detachment.
Other classifications suggest that at
least two mucous membrane surfaces
should be involved.3
There have been several case reports
in which Mycoplasma infection is localized to the mucous membranes with
very little to no skin involvement.4-6 Patients will often have ocular, oral, and
genital involvement as in our patient.
This was previously known as atypical SJS but it has been suggested that
the term Mycoplasma pneumoniaeassociated mucositis would be a more
appropriate definition.1 This is because
patients with only mucous membrane
involvement recover faster than those
patients with SJS, and they do not
have the higher risk of mortality seen
in SJS.1 Skin involvement is also a key
criterion in the diagnosis of SJS.
The etiology of Mycoplasma pneumoniae-associated mucositis is unknown but it has been suggested that
an antibody may be directed against
a specific mucous membrane antigen,
causing the clinical sequelae that are
seen.4 The diagnosis is often made by
detection of Mycoplasma pneumoniae
IgM and patients are often treated with
macrolide antibiotics. In all the case
reports reviewed, the mucositis was severe enough to require hospitalization.
The use of corticosteroids is controversial, as it is in the treatment of SJS
due to the concern of predisposing the
patient to further infection. Most of the
patients recovered with the use of antibiotics alone, without the use of corti-
150 | Healio.com/Pediatrics
costeroids and without any major lasting sequelae. In the few cases that were
refractory to antibiotic treatment and
supportive care, intravenous immunoglobulin provided rapid improvement in
the mucositis.7-9
CONCLUSIONS
The case presented represents a rare
but clinically significant manifestation
of Mycoplasma pneumoniae infection.
In children, Mycoplasma pneumoniae
is one of the most common causes of
SJS, but it is important to consider this
organism in mucous membrane-limited disease as well. It is also important
to distinguish between Mycoplasma
pneumoniae-associated mucositis and
SJS, as the long-term sequelae and
mortality rate differ greatly. Patients
who present with mucous membranelimited disease, however, should be
monitored closely for the progression
to skin involvement and SJS. Our patient was discharged home on hospital
day 5 with resolving mucositis. He was
able to tolerate oral fluids and was to
complete a 5-day course of azithromycin.
This patient’s recovery proceeded
slowly. After discharge, he continued
to require significant pain medication
for both oral and penile pain and local care for the penile lesions. Given
his slow improvement, he received a
second 5-day course of azithromycin
(initiated 10 days after the first course).
He returned to school approximately 3
weeks after his initial presentation to
the pediatrician’s office (about 12 days
after hospital discharge). At that point,
he had minimal remnants of the oral
and penile ulcerations and required no
pain medications. He experienced a total weight loss of 20 pounds (15% of
pre-illness body weight) that he slowly
regained over the month after his return
to full activity.
REFERENCES
1. Schalock PC, Dinulos JG. Mycoplasma pneumoniae-induced cutaneous disease. Int J Dermatol. 2009;48(7):673-680.
2. Bastuji-Garin S, Rzany B, Stern RS, Shear
NH, Naldi L, Roujeau JC. Clinical classification of cases of toxic epidermal necrolysis,
Stevens-Johnson syndrome, and erythema
multiforme. Arch Dermatol. 1993;129(1):9296.
3. Huff JC, Weston WL, Tonnesen MG. Erythema multiforme: a critical review of characteristics, diagnostic criteria and causes. J Am
Acad Dermatol. 1983;8(6):763-775.
4. Schalock PA, Dinulos JGD, Pace N, Schwarzenberger K, Wenger JK. Erythema multiforme due to Mycoplasma pneumoniae
infection in two children. Pediatr Dermatol.
2006;23(60:546-555.
5. Figueira-Coelho J, Lourenco S, Pires AC,
Mendcona P, Malhado JA. Mycoplasma
pneumoniae- associated mucositis with minimal skin manifestations. Am J Clin Dermatol.
2008;9(6):399-403.
6. Ravin KA, Rappaport LD, Zuckerbraun NS,
Wadowsky RM, Wald ER, Michaels MM.
Mycoplasma pneumoniae and atypical Stevens-Johnson Syndrome: a case series. Pediatrics. 2007;119(4):e1002-1005.
7. Bressan S, Mion T, Andreola B, Bisogna G,
Da Dalt L. Mycoplasma pneumonia-associated mucositis treated with immunoglobulins.
Acta Paediatrica. 2011;100(11):e238-240.
8. Zipitis CS, Thalange N. Intravenous immunoglobulins for the management of StevensJohnson syndrome with minimal skin manifestations. Eur J Pediatr. 2007;166(6):585-588.
9. Birch J, Chamlin S, Duerst R, Jacobsohn D.
Mycoplasma pneumonia and atypical Stevens-Johnson Syndrome in a hematopoietic
stem cell transplant recipient. Pediatr Blood
Cancer. 2008;50(6):1278-1279.
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