Pediatric emergency medicine trisk 0932 0932

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.