You are in: eMedicine Specialties > Dermatology > LYMPHOMA AND RELATED PROCESSES Kimura DiseaseArticle Last Updated: May 25, 2007AUTHOR AND EDITOR INFORMATIONSection 1 of 10
  Author: Sena J Lee, MD, PhD, Staff Physician, Department of Dermatology, Hospital of University of Pennsylvania Sena Lee is a member of the following medical societies: American Academy of Dermatology Coauthor(s): Alaina J James, MD, PhD, Staff Physician, Department of Dermatology, University of Pennsylvania Medical Center; William D James, MD, Paul R Gross Professor of Dermatology, University of Pennsylvania School of Medicine; Vice-Chair, Program Director, Department of Dermatology, University of Pennsylvania Health System; Laura M Tamburin, MD, Affiliated Dermatology, PC; Patricia Mercado, MD, Chief, Dermatology Division at Birmingham Veterans Administration Medica, Associate Professor of Dermatology, Department of Dermatology, University of Alabama at Birmingham Editors: Takeji Nishikawa, MD, Emeritus Professor, Department of Dermatology, Keio University School of Medicine; Director, Samoncho Dermatology Clinic; Managing Director, The Waksman Foundation of Japan Inc; Michael J Wells, MD, Associate Professor, Department of Dermatology, Texas Tech University Health Sciences Center; Daniel S Loo, MD, Associate Professor, Residency Program Director, Department of Dermatology, Boston University School of Medicine; Joel M Gelfand, MD, MSCE, Medical Director, Clinical Studies Unit, Assistant Professor, Department of Dermatology, Associate Scholar, Center for Clinical Epidemiology and Biostatistics, University of Pennsylvania; Dirk M Elston, MD, Director, Department of Dermatology, Geisinger Medical Center Author and Editor Disclosure Synonyms and related keywords: eosinophilic granuloma of soft tissue, eosinophilic hyperplastic lymphogranuloma, eosinophilic lymphofolliculosis, eosinophilic lymphofollicular granuloma, eosinophilic lymphoid granuloma INTRODUCTIONSection 2 of 10
  BackgroundKimura disease (KD) is a chronic inflammatory disorder of unknown etiology, most commonly manifesting as painless unilateral cervical lymphadenopathy or subcutaneous masses in the head or neck region. The first report of KD was from China in 1937, in which Kimm and Szeto1 described 7 cases of a condition they termed "eosinophilic hyperplastic lymphogranuloma." The disorder received its current name in 1948, when Kimura et al2 noted the vascular component and referred to it as an "unusual granulation combined with hyperplastic changes in lymphoid tissue." Controversy exists in the literature regarding whether KD and angiolymphoid hyperplasia with eosinophilia (ALHE) are the same entity. Some authors believe that KD represents a chronic deeper, form of ALHE; however, most recent papers distinguish the 2 on the basis of clinical and histopathologic characteristics. For more information, see Angiolymphoid Hyperplasia with Eosinophilia. ALHE appears to represent an arteriovenous malformation with secondary inflammation. Kimura disease may represent a primary inflammatory process with secondary vascular proliferation. PathophysiologyThe pathophysiology of KD remains unknown, although an allergic reaction, trauma, and an autoimmune process have all been implicated as the possible cause. The disease is manifested by an abnormal proliferation of lymphoid follicles and vascular endothelium. Peripheral eosinophilia and the presence of eosinophils in the inflammatory infiltrate suggest that KD may be a hypersensitivity reaction. Some evidence has indicated that TH2 lymphocytes may also play a role, but further investigation is needed. KD is generally limited to the skin, lymph nodes, and salivary glands, but patients with KD and nephrotic syndrome have been reported. The basis of this possible association is unclear. FrequencyUnited StatesKD has rarely been reported in the United States. InternationalThe prevalence of KD is not known, but most cases are reported from the Far East. Mortality/MorbidityKD is a benign disorder with no potential for malignant transformation, but spontaneous involution is rare. The main concern is the capacity for lesions to grow and cause disfigurement. RaceMost cases of KD have been reported in Asians, and the prevalence among persons of other races is thought to be low. A retrospective review of 21 histopathologic specimens diagnosed as KD at the US Armed Forces Institute of Pathology found the following racial distribution: 7 whites, 6 blacks, 6 Asians, 1 Hispanic, and 1 Arab. This illustrates that if clinically suspected, KD should be included on the differential diagnosis for persons of any racial group. SexMales are affected by KD more commonly than females, with a 6:1 ratio in one series. AgeKD is usually seen in young adults. A series by Kung et al3 reported a median age of 28 years. CLINICALSection 3 of 10
HistoryLesions of KD typically are slowly enlarging painless masses with occasional pruritus of the overlying skin. PhysicalPatients may present with a solitary enlarged painless lymph node or generalized lymphadenopathy. Salivary gland involvement is also frequently observed. Other findings include single or multiple subcutaneous nodules, which are usually located on the head or neck, especially in the periauricular, parotid, or submandibular regions. Less frequently, the eyelids, orbit, and lacrimal glands may be involved. The average diameter of lesions is 3 cm. Although KD mainly affects the head and neck, involvement of the extremities and inguinal lymph nodes has been reported. CausesThe cause of KD is unknown; however, an allergic reaction or an alteration of immune regulation is suspected. Proposed theories include persistent antigenic stimulation following arthropod bites and parasitic or candidal infection. To date, none of these theories has been substantiated. DIFFERENTIALSSection 4 of 10
Angiolymphoid Hyperplasia with Eosinophilia Cylindroma Dermatofibrosarcoma Protuberans Kaposi Sarcoma Pyogenic Granuloma (Lobular Capillary Hemangioma)
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| Drug Name | Cyclosporine (Sandimmune, Neoral) |
|---|---|
| Description | 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 Dose | 3-5 mg/kg/d PO |
| Pediatric Dose | Not established |
| Contraindications | Documented hypersensitivity; uncontrolled hypertension or malignancies; concomitant administration with PUVA or UV-B radiation in psoriasis (may increase cancer risk) |
| Interactions | 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 |
| 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 |
| Drug Name | Triamcinolone (Amcort, Aristocort) |
|---|---|
| Description | 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 Dose | 0.3 mL intralesionally of a 5-10 mg/mL concentration for total dose of 1.5-3 mg |
| Pediatric Dose | <12 years: Not established >12 years: Administer as in adults |
| Contraindications | Documented hypersensitivity; fungal, viral, and bacterial skin infections |
| Interactions | Coadministration with barbiturates, phenytoin, and rifampin decreases effects of triamcinolone |
| 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 |
| Drug Name | Prednisone (Orasone, Deltasone, Meticorten, Sterapred) |
|---|---|
| Description | May decrease inflammation by reversing increased capillary permeability and suppressing PMN activity. |
| Adult Dose | Begin at 60 mg/d PO, taper by 10 mg/wk for a 6-wk trial |
| Pediatric Dose | Not established |
| Contraindications | Documented hypersensitivity; viral infection, peptic ulcer disease, hepatic dysfunction, connective-tissue infections, and fungal or tubercular skin infections; GI bleeding or ulceration |
| Interactions | 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 |
| 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 |
These agents are used to treat vascular disease.
| Drug Name | Pentoxifylline (Pentoxil, Trental) |
|---|---|
| Description | May alter rheology of red blood cells, which, in turn, reduces blood viscosity |
| Adult Dose | 400 mg PO bid with meals (based on a single case report by Hongcharu et al) |
| Pediatric Dose | Not established |
| Contraindications | Documented hypersensitivity to drug or methylxanthines; cerebral and/or retinal hemorrhage |
| Interactions | 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 |
| 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 |
These agents regulate cell growth and differentiation.
| Drug Name | Tretinoin (Vesanoid) |
|---|---|
| Description | May inhibit granulocyte differentiation. |
| Adult Dose | 45 mg/m2/d PO divided bid (based on a single case report by Boulanger et al) |
| Pediatric Dose | Not established |
| Contraindications | Documented hypersensitivity (including sensitivity to retinoids, paraben); leukocytosis |
| Interactions | 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 |
| 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 |
Article Last Updated: May 25, 2007
