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FIGURE 47.5 Child with nontuberculous mycobacterium (NTM) lymphadenitis.

In addition to infectious causes of cervical lymphadenopathy, there are numerous
noninfectious etiologies that cause cervical node enlargement as a manifestation of
systemic disease. Such noninfectious etiologies that may be encountered in pediatric
patients include Kawasaki disease, malignancy, histiocytosis, lymphoproliferative
disorders, and periodic fever syndromes.
An important cause of acute cervical adenopathy in young children is Kawasaki
disease, or mucocutaneous lymph node syndrome (see Chapter 86 Cardiac Emergencies ).
Kawasaki disease usually affects children younger than 4 years of age and is rare after 8
years of age. Typical presentation of the disease is characterized by fever of at least 5
days’ duration, along with bilateral nonexudative conjunctivitis, changes to oral mucosa,
peripheral extremity changes, a polymorphous rash, and cervical lymphadenopathy. The
cervical lymphadenopathy in Kawasaki disease, seen in approximately 50% to 70% of
patients, occurs during the early phase of the illness and may be unilateral or bilateral.
The nodes are firm, mildly tender, and at least 1.5 cm in diameter. It is important to
diagnose Kawasaki disease early because prompt treatment with intravenous immune
globulin (IVIG) and aspirin can prevent coronary artery aneurysms, the most serious
complication of this disease.
Various malignancies, including lymphoma and neuroblastoma may present with
chronic cervical adenopathy. Lymph nodes in Hodgkin lymphoma are painless, with a
rubbery and firm consistency, and located in the cervical, supraclavicular, or axillary
regions. Palpation of such nodes in children, particularly when a history of nonspecific
symptoms such as fatigue, anorexia, and weight loss are elicited, should lead to prompt
evaluation with chest radiograph to evaluate for mediastinal masses. Neuroblastoma, most


common in infants and children less than 2 years of age, is a neuroendocrine tumor
arising in the adrenal glands, but can originate in the high thoracic and cervical
sympathetic ganglia or metastasize to cervical and supraclavicular lymph nodes. Again,
prompt imaging with chest radiograph, as well as abdominal imaging with computed

tomography (CT) or magnetic resonance imaging (MRI) should be initiated in cases of
suspected neuroblastoma.
Rare noninfectious causes of chronic cervical lymphadenopathy deserve consideration
in the appropriate clinical context. Sarcoidosis is a multisystem granulomatous disorder
that affects young adults, particularly African Americans, causing systemic symptoms
such as weight loss, cough, fatigue, and joint pain. Patients often have chronic bilateral
cervical adenopathy, and chest radiograph in such patients may reveal hilar adenopathy as
well.
Autoimmune lymphoproliferative syndrome (ALPS), also known as Canale–Smith
syndrome, is a genetic disorder of lymphocyte apoptosis leading to lymphadenopathy,
splenomegaly, and pancytopenia. Children with ALPS present in the first year of life with
massive cervical adenopathy and splenomegaly.
Histiocytic disorders, another uncommon group of diseases that cause prominent
cervical adenopathy, occur when there is an overproduction of dendritic cells or
macrophages which cause organ damage and tumor formation. Langerhans cell
histiocytosis (LCH) affects children less than 3 years of age, causing lytic bone lesions
and unilateral or bilateral soft and matted cervical lymphadenopathy. Sinus histiocytosis
with massive lymphadenopathy (Rosai–Dorfman disease) presents in children as
massively enlarged, nontender cervical lymphadenopathy, along with involvement of the
nasal cavity, lytic bone lesions, pulmonary nodules, or rash.
Finally, the syndrome of periodic fever, aphthous stomatitis, pharyngitis, and cervical
adenitis (PFAPA) is an autoinflammatory disease presenting in young children that cycles
every 2 to 9 weeks. Children with PFAPA experience tender cervical lymphadenopathy
with flares. There is no specific diagnostic test, and though corticosteroids and
nonsteroidal anti-inflammatory drugs alleviate symptoms, the condition resolves
spontaneously by 10 years of age.

Other Lymphadenopathy of the Head and Neck
Beyond cervical lymphadenopathy, acute localized adenopathy of the head and facial
regions has a more narrow differential diagnosis, and the location of lymphadenopathy

often suggests a cause, based on the location of focal infections and drainage patterns of
nodes. Submandibular and submental nodes, draining the lips, buccal mucosa, and floor
of the oropharynx, enlarge with infection within the oral cavity. This includes dental
caries and abscesses, as well as gingivostomatitis. Herpetic gingivostomatitis generally
affects the anterior tongue, buccal mucosa, or lips, while Coxsackievirus
gingivostomatitis affects the posterior oropharynx, sparing the gingiva and lips, and
causes characteristic lesions on palmar and plantar surfaces. Lymphadenopathy in the


submandibular or submental regions on physical examination should prompt a careful
oral and dental examination.
Preauricular nodes, located anterior to the ear, drain the conjunctiva and lateral eyelids.
These nodes enlarge with eye or conjunctival infections, of which viral infections are a
prominent cause. The combination of conjunctivitis and ipsilateral preauricular
adenopathy is called oculoglandular syndrome, or Parinaud syndrome. Infections that can
present as oculoglandular syndrome include adenovirus or chlamydial conjunctivitis in
neonates. Rarely, catscratch disease and tularemia manifest as an oculoglandular
syndrome.
Posterior auricular nodes, located behind the ear, and occipital nodes, found at the base
of the scalp, commonly enlarge in response to scalp infections or chronic inflammation.
Pediculosis (lice), tinea capitis, bacterial scalp infections, and inflammation from
seborrheic dermatitis are all common causes of such node enlargement in children.

Axillary and Epitrochlear
Axillary adenopathy is commonly present with any infection or inflammation of the upper
extremities. Most commonly, injuries to the hand, such as occur after falling or with
puncture wounds or bites, may present with concomitant axillary adenopathy as a reactive
response to disruption in skin integrity. Axillary adenopathy is also a common part of B.
henselae infection (catscratch disease), as outlined previously as a cause of cervical
adenopathy.

Epitrochlear adenopathy is significantly less common than axillary adenopathy in
children, and any epitrochlear node greater than 0.5 cm is considered enlarged.
Epitrochlear nodes may become inflamed after infections of the third, fourth, or fifth
finger; medial portion of the hand; or ulnar portion of the forearm. Most commonly, these
infections are caused by pyogenic bacteria (e.g., Streptococcus pyogenes or S. aureus,
including MRSA), but depending on the inciting event, other pathogens may be
responsible (e.g., Streptobacillus moniliformis and Spirillum minus in rat-bite fever or F.
tularensis in tularemia). Rare causes of both axillary and epitrochlear adenopathy include
rheumatologic disease of the hand or wrist and secondary syphilis.

Inguinal
Inguinal adenopathy most often results from lower extremity skin or soft tissue infection.
However, inguinal lymph nodes also drain tissues in the perianal region and unexplained
adenopathy in this area should prompt examination for perirectal abscesses, fissures, or
other inflammation. In addition, sexually transmitted diseases such as chlamydia or
gonorrhea may cause inguinal adenopathy. Acute genital infection with herpes simplex
virus-2 (HSV-2) often presents with tender inguinal adenopathy, occasionally as the only
sign. Chancroid, lymphogranuloma venereum, and syphilis are rare causes of inguinal
node swelling and tenderness. The presence of genital lesions, which may be either
painful (as in herpes simplex virus or chancroid) or painless (as in syphilis), offers clues
to these diagnoses. Therefore, careful history taking and physical examination are
necessary to exclude these possibilities.


Iliac
Enlarged iliac nodes are palpable deeply over the inguinal ligament and become inflamed
with lower extremity infection, urinary tract infection, abdominal trauma, and
appendicitis. Of note, iliac adenitis, which can present with fever, limp, and inability to
fully extend the leg, may mimic the signs and symptoms of septic hip arthritis. Unlike in
hip disease, however, hip motion is not limited on examination. Iliac adenitis may also be

confused with appendicitis, but the pain initially occurs in the thigh and hip rather than in
the periumbilical region or right lower quadrant.

GENERALIZED LYMPHADENOPATHY
Generalized lymphadenopathy, defined as enlargement of lymph nodes in two or more
noncontiguous regions, can be a manifestation of both infectious and noninfectious
systemic illnesses ( Table 47.2 ). Akin to localized lymphadenopathy, generalized
lymphadenopathy in children is most often caused by bacterial or viral infections. As an
example, the high incidence of vomiting and abdominal pain in streptococcal pharyngitis
has been attributed to abdominal lymph node swelling and inflammation, suggesting a
more systemic pattern of adenopathy in streptococcal disease. More rare bacterial causes
of generalized lymphadenopathy include the zoonotic infections brucellosis, leptospirosis,
and tularemia. Brucellosis is acquired by exposure to cattle, sheep, goats, or
unpasteurized milk or cheese and causes systemic symptoms including fever, night
sweats, weight loss, arthralgias, and epididymoorchitis, as well as nonspecific
examination findings including generalized lymphadenopathy and hepatosplenomegaly.
Leptospirosis is most common in tropical climates, acquired via exposure to contaminated
soil or water (particularly during swimming). Clinical manifestations are nonspecific,
including fever, rigors, myalgias, headache, conjunctivitis, rash, hepatosplenomegaly, and
lymphadenopathy. Brucellosis, leptospirosis, and tularemia (discussed previously) should
be considered in the differential diagnosis of a child presenting with systemic symptoms,
particularly fever, and physical examination findings of generalized lymphadenopathy if
the appropriate exposure history is elicited.
Common viral causes of generalized adenopathy include EBV or CMV mononucleosis,
and rubella and measles infections in parts of the world where these diseases are endemic.
Though EBV mononucleosis classically causes fever, pharyngitis, and anterior and
posterior cervical adenopathy; axillary and inguinal lymphadenopathy may also be
presenting signs. In children with symptoms of infectious mononucleosis but a negative
monospot and/or negative antibody titers to EBV antigens, CMV may be the cause.
Rubella produces a prodrome of low-grade fever and lymphadenopathy (posterior

cervical, postauricular, or generalized) followed by the development and rapid spread of a
pink, maculopapular rash from face to the trunk and extremities. Measles (rubeola)
produces a prodrome of fever, malaise, and anorexia followed by cough, conjunctivitis,
coryza, and characteristic Koplik spots on the buccal mucosa. The exanthem of measles
also has a cranial to caudal progression of a blanching, maculopapular rash, and lesions
can become confluent. Children with severe measles may exhibit generalized