eMedicine Specialties > Dermatology > Lymphoma and Related Processes
Kimura Disease: Treatment & Medication
Updated: May 25, 2007
- Overview
- Differential Diagnoses & Workup
- Treatment & Medication
- Follow-up
Treatment
Medical Care
- Observation is acceptable if the lesions are neither symptomatic nor disfiguring.
- Intralesional or oral steroids can shrink the nodules but seldom result in cure. A medium-potency steroid (eg, triamcinolone acetonide), used in solution form for intralesional injection, is usually well tolerated.
- Cyclosporine has been reported to induce remission in patients with KD. A dose of 5 mg/kg/d was effective, but, in most cases, the lesions recurred upon cessation of therapy.4
- Oral pentoxifylline has been reported to be effective in one patient with KD; however, the lesions relapsed after discontinuation of therapy.5
- All trans -retinoic acid in combination of prednisone has resulted in remission of KD in one patient, and he remained disease free 12 months after discontinuation of all therapy.6
- Radiotherapy has been used to treat recurrent or persistent lesions. A report by Hareyama et al7 reported on the use of radiotherapy at dosages of 26-30 Gy; local control was achieved in 74% of lesions. Considering the benign nature of KD, radiation should be considered only for recurrent, disfiguring lesions.
Surgical Care
- Conservative surgical excision is considered the treatment of choice; however, lesions often recur after excision.
Medication
The goals of pharmacotherapy are to reduce morbidity and to prevent complications.
Immunosuppressants
Suppress response of immune system to diverse stimuli.
Cyclosporine (Sandimmune, Neoral)
Demonstrated to be helpful in a variety of skin disorders. Cyclic polypeptide that suppresses some humoral immunity and, to a greater extent, cell-mediated immune reactions, such as delayed hypersensitivity, allograft rejection, experimental allergic encephalomyelitis, and graft versus host disease for a variety of organs. For children and adults, base dosing on ideal body weight.
Adult
3-5 mg/kg/d PO
Pediatric
Not established
Carbamazepine, phenytoin, isoniazid, rifampin, and phenobarbital may decrease concentrations; azithromycin, itraconazole, nicardipine, ketoconazole, fluconazole, erythromycin, verapamil, grapefruit juice, diltiazem, aminoglycosides, acyclovir, amphotericin B, and clarithromycin may increase toxicity; acute renal failure, rhabdomyolysis, myositis, and myalgias increase when taken concurrently with lovastatin
Documented hypersensitivity; uncontrolled hypertension or malignancies; concomitant administration with PUVA or UV-B radiation in psoriasis (may increase cancer risk)
Pregnancy
C - Safety for use during pregnancy has not been established.
Precautions
Evaluate renal and liver functions often by measuring BUN, serum creatinine, serum bilirubin, and liver enzyme levels and blood pressure; may increase risk of infection and lymphoma; reserve IV use only for those who cannot take PO; adverse effects include nephrotoxicity, hypertension, hepatotoxicity, gingival enlargement, hyperkalemia, hypomagnesemia, pancreatitis, and paresthesia; factors that may increase risk for neurotoxicity from cyclosporine include hypomagnesemia, hypocholesterolemia, fever, infection, hypertension, intravenous administration, and rapidly increasing cyclosporine blood levels
Triamcinolone (Amcort, Aristocort)
For inflammatory dermatosis responsive to steroids. Decreases inflammation by suppressing migration of polymorphonuclear leukocytes and reversing capillary permeability. Intralesional injections may be used for localized skin disorders.
Adult
0.3 mL intralesionally of a 5-10 mg/mL concentration for total dose of 1.5-3 mg
Pediatric
<12 years: Not established
>12 years: Administer as in adults
Coadministration with barbiturates, phenytoin, and rifampin decreases effects of triamcinolone
Documented hypersensitivity; fungal, viral, and bacterial skin infections
Pregnancy
C - Safety for use during pregnancy has not been established.
Precautions
Atrophy and perilesional and linear hypopigmentation may occur with intralesional administration; repeated injections of high concentrations of triamcinolone may result in multiple complications, eg, severe infections, hyperglycemia, edema, osteonecrosis, myopathy, peptic ulcer disease, hypokalemia, osteoporosis, euphoria, psychosis, myasthenia gravis, growth suppression; abrupt discontinuation of high doses of glucocorticoids may cause adrenal crisis
Prednisone (Orasone, Deltasone, Meticorten, Sterapred)
May decrease inflammation by reversing increased capillary permeability and suppressing PMN activity.
Adult
Begin at 60 mg/d PO, taper by 10 mg/wk for a 6-wk trial
Pediatric
Not established
Coadministration with estrogens may decrease clearance; when used with digoxin, digitalis toxicity secondary to hypokalemia may increase; phenobarbital, phenytoin, and rifampin may increase the metabolism of glucocorticoids (consider increasing maintenance dose); monitor for hypokalemia with coadministration of diuretics; coadministration with ritonavir may significantly increase serum concentrations of prednisone; concomitant therapy with montelukast may result in severe peripheral edema; clarithromycin may increase risk of psychotic symptoms
Documented hypersensitivity; viral infection, peptic ulcer disease, hepatic dysfunction, connective-tissue infections, and fungal or tubercular skin infections; GI bleeding or ulceration
Pregnancy
B - Usually safe but benefits must outweigh the risks.
Precautions
May unmask hypertension or diabetes or exacerbate peptic ulcer disease and tuberculosis; long-term sequelae associated with long-term steroid use include osteoporosis, cataracts, and pituitary-hypothalamic axis suppression; with high doses, patients may develop a steroid psychosis and are at increased risk of infections, particularly when oral steroids are used in conjunction with other immunosuppressants; frequently monitor patient's blood sugar level, blood pressure, and weight; monitor for Cushing syndrome
Hemorheologic agents
These agents are used to treat vascular disease.
Pentoxifylline (Pentoxil, Trental)
May alter rheology of red blood cells, which, in turn, reduces blood viscosity
Adult
400 mg PO bid with meals (based on a single case report by Hongcharu et al)
Pediatric
Not established
Coadministration with cimetidine or theophylline, increases effect/toxic potential; increases effect of antihypertensives; may increase hypoprothrombinemic effect of dicumarol; may increase blood glucose – lowering effect of insulin and susceptibility to hypoglycemia
Documented hypersensitivity to drug or methylxanthines; cerebral and/or retinal hemorrhage
Pregnancy
C - Safety for use during pregnancy has not been established.
Precautions
Caution in renal impairment, coagulation defects, recent surgery, and increased risk of bleeding; serious adverse effects include angina, aplastic anemia, cardiac dysrhythmia, confusion, depression, edema, hepatitis, hypotension, increased liver function test, jaundice, leukopenia, and seizure, thrombocytopenia
Retinoids
These agents regulate cell growth and differentiation.
Tretinoin (Vesanoid)
May inhibit granulocyte differentiation.
Adult
45 mg/m2/d PO divided bid (based on a single case report by Boulanger et al)
Pediatric
Not established
CYP450 substrate (caution with coadministration of inhibitors or inducers of CYP450); ketoconazole significantly increases AUC; coadministration with tetracyclines may increase risk for pseudotumor cerebri and intracranial hypertension; coadministration with vitamin A may increase risk of hypervitaminosis A; fatal thrombotic complications have been reported when coadministered with antifibrinolytic agents (eg, tranexamic acid, aminocaproic acid, aprotinin); concomitant administration with methotrexate may increase risk of liver toxicity; concurrent use of tretinoin and voriconazole may result in hypercalcemia
Documented hypersensitivity (including sensitivity to retinoids, paraben); leukocytosis
Pregnancy
D - Unsafe in pregnancy
Precautions
Should only be administered by experienced oncologists; severe leukocytosis with pulmonary infiltrates and respiratory failure expected; patients commonly experience headache, fever, weakness, and fatigue; serious adverse effects include pseudotumor cerebri, headache, nausea/vomiting, fever, skin dryness, bone pain, weight gain
More on Kimura Disease |
| Overview: Kimura Disease |
| Differential Diagnoses & Workup: Kimura Disease |
 Treatment & Medication: Kimura Disease |
| Follow-up: Kimura Disease |
| References |
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References
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Further Reading
Keywords
eosinophilic granuloma of soft tissue, eosinophilic hyperplastic lymphogranuloma, eosinophilic lymphofolliculosis, eosinophilic lymphofollicular granuloma, eosinophilic lymphoid granuloma
