Pediatric emergency medicine trisk 233

lymphadenopathy and splenomegaly. Finally HIV infection causes adenopathy in the
cervical, axillary, and inguinal regions, particularly during the acute phase of infection.
Nodes generally decrease in size after the first 1 to 2 weeks as the immune response to
infection subsides, however a modest degree of adenopathy can persist. Children with
unexplained recurrent or opportunistic infections, fevers, failure to thrive,
hepatosplenomegaly, and generalized adenopathy should be evaluated for HIV infection.


TABLE 47.2
CAUSES OF GENERALIZED LYMPHADENOPATHY


Systemic infection
Bacterial
Bacteremia
Group A streptococcus infection (pharyngitis, scarlet fever)
Brucellosis
Leptospirosis
Tularemia
Viral
Epstein–Barr virus
Cytomegalovirus
Human immunodeficiency virus
Adenovirus
Varicella
Rubella
Rubeola (measles)
Hepatitis B virus
Fungal
Histoplasmosis
Coccidioidomycosis

Blastomycosis
Parasitic
Toxoplasmosis
Malaria
Spirochete
Syphilis
Lyme disease
Autoimmune disease
Juvenile idiopathic arthritis
Systemic lupus erythematosus
Dermatomyositis
Serum sickness
Drug reaction with eosinophilia and systemic symptoms (DRESS)
Autoimmune hemolytic anemia
Chronic granulomatous disease (sarcoid)
Malignancy
Leukemia (acute lymphoblastic or acute myelogenous leukemia)
Non-Hodgkin lymphoma
Hodgkin lymphoma
Neuroblastoma
Histiocytosis
Hemophagocytic lymphohistiocytosis (HLH)


Langerhans cell histiocytosis (LCH)
Sinus histiocytosis with massive lymphadenopathy (Rosai–Dorfman disease)
Storage disease
Gaucher disease
Niemann–Pick disease
Miscellaneous

Autoimmune lymphoproliferative syndrome (ALPS)
Castleman disease
Hyperthyroidism
Noninfectious systemic disease that also presents with generalized adenopathy includes
inflammatory or autoimmune diseases, serum sickness, malignancy, histiocytosis, and
genetic storage diseases. More than half of patients with systemic lupus erythematosus or
systemic onset juvenile idiopathic arthritis manifest generalized adenopathy during the
acute phase of illness. Nodes are typically soft, nontender, and located in the cervical,
axillary, and inguinal regions.
Serum sickness is an immune complex–mediated hypersensitivity reaction to a number
of drugs, with clinical manifestations including fever, rash, myalgias, and arthralgias.
Symptoms begin 1 to 2 weeks after exposure to an inciting agent, and resolve
spontaneously within weeks of discontinuation. Skin manifestations are variable, though
typically urticarial and macular involving the lower trunk, groin, or axillary regions and
spreading to the back, upper extremities, and hands. Lymphadenopathy may be noted with
the rash, but may be seen without the rash and may be accompanied by splenomegaly.
Table 47.3 lists drugs that have been implicated in serum sickness reactions.
Drug reaction with eosinophilia and systemic symptoms (DRESS) is a rare, potentially
life-threatening drug-induced hypersensitivity reaction postulated to be caused by a drugspecific immune response and reactivation of herpes virus. Clinical manifestations
include fever, diffuse morbilliform skin eruption, facial edema, hematologic abnormalities
(eosinophilia, atypical lymphocytosis), lymphadenopathy, and visceral involvement (liver,
kidney, lung, and/or bone marrow). The reaction begins 2 to 6 weeks after the initiation of
the offending medication. The aromatic antiepileptic agents and the sulfonamides are the
most frequent causes of this disorder ( Table 47.3 ). Stopping the offending agent and
avoiding similar cross-reacting drugs as well as supportive care are mainstay measures of
treatment.