eMedicine Specialties > Pediatrics: General Medicine > Rheumatology
Weber-Christian Disease: Treatment & Medication
Updated: May 8, 2009
- Overview
- Differential Diagnoses & Workup
- Treatment & Medication
- Follow-up
- Multimedia
Treatment
Medical Care
No uniformly effective therapy for Weber-Christian disease is known.
- Therapeutic responses have been reported with the use of fibrinolytic agents, hydroxychloroquine, azathioprine, thalidomide, cyclophosphamide, tetracycline, cyclosporine, mycophenolate, and clofazimine.
- Systemic steroids (eg, prednisone) may be effective in suppressing acute exacerbations.
- Nonsteroidal anti-inflammatory agents may reduce fever, arthralgias, and other signs of malaise.
- Involvement of specific organs may require specific supportive drugs.
Surgical Care
- No surgical treatment is indicated.
Consultations
- Consult a dermatologist to perform a skin biopsy and to consider the wide variety of causes of panniculitis.
Diet
- No specific dietary requirements are noted.
Activity
- Activity is ad lib, and trauma to the affected areas should be avoided.
Medication
No specific uniformly effective therapy for Weber-Christian disease is recognized. Therapeutic responses have been reported using fibrinolytic agents, hydroxychloroquine, azathioprine, thalidomide, cyclophosphamide, tetracycline, mycophenolate, and clofazimine. Systemic steroids (eg, prednisone) may be effective in suppressing acute exacerbations. Nonsteroidal anti-inflammatory agents (eg, aspirin, indomethacin) may reduce fever, arthralgias, and other signs of malaise. Involvement of specific organs may require specific supportive drugs.
Corticosteroids
These agents are used for suppression of acute inflammatory exacerbations. These agents have anti-inflammatory properties and cause profound and varied metabolic effects. They modify the body's immune response to diverse stimuli.
Prednisone (Deltasone, Meticorten, Orasone)
May decrease inflammation by reversing increased capillary permeability and suppressing PMN activity.
Adult
40-60 mg/d PO qd or divided bid/qid; taper over 2 wk as symptoms resolve
Pediatric
4-5 mg/m2/d PO; alternatively, 0.5-2 mg/kg PO divided bid/qid; taper over 2 wk as symptoms resolve
Coadministration with estrogens may decrease prednisone clearance; concurrent use with digoxin
may cause digitalis toxicity secondary to hypokalemia; phenobarbital, phenytoin, and rifampin may increase metabolism of glucocorticoids (consider increasing maintenance dose); monitor for hypokalemia with coadministration of diuretics
Documented hypersensitivity; viral infection; peptic ulcer disease; hepatic dysfunction; connective tissue infections; fungal or tubercular skin infections; GI ulceration or bleeding
Pregnancy
B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals
Precautions
Abrupt discontinuation of glucocorticoids may cause adrenal crisis; hyperglycemia, edema, osteonecrosis, myopathy, peptic ulcer disease, hypokalemia, osteoporosis, euphoria, psychosis, myasthenia gravis, growth suppression, and infections may occur with glucocorticoid use
Immunomodulators
These agents inhibit key factors that mediate immune reactions, which, in turn, decrease inflammatory responses. They may have potential long-term therapeutic response.
Azathioprine (Imuran)
Antagonizes purine metabolism and inhibits synthesis of DNA, RNA, and proteins. May decrease proliferation of immune cells, which results in lower autoimmune activity.
Adult
1 mg/kg/d PO for 6-8 wk; increase by 0.5 mg/kg q4wk until response occurs or dose reaches 2.5 mg/kg/d
Pediatric
Initial dose: 2-5 mg/kg/d PO/IV
Maintenance dose: 1-2 mg/kg/d PO/IV
Toxicity increases with allopurinol; concurrent use with ACE inhibitors may induce severe leukopenia; may increase levels of methotrexate metabolites and decrease effects of anticoagulants, neuromuscular blockers, and cyclosporine
Documented hypersensitivity; low levels of serum TPMT
Pregnancy
D - Fetal risk shown in humans; use only if benefits outweigh risk to fetus
Precautions
Increases risk of neoplasia; caution with liver disease and renal impairment; hematologic toxicities may occur; check TPMT level prior to therapy and monitor liver, renal, and hematologic function; pancreatitis rarely associated
Cyclosporine (Neoral, Sandimmune)
Cyclic polypeptide that suppresses some humoral immunity and, to a greater extent, cell-mediated immune reactions (eg, delayed hypersensitivity, allograft rejection, experimental allergic encephalomyelitis, graft versus host disease) for a variety of organs. For children and adults, base dosing on ideal body weight. Demonstrated to be helpful in variety of skin disorders.
Adult
2.5-5 mg/kg/d PO in divided doses
Pediatric
Administer as in adults
Carbamazepine, phenytoin, isoniazid, rifampin, and phenobarbital may decrease cyclosporine concentrations; azithromycin, itraconazole, nicardipine, ketoconazole, fluconazole, erythromycin, verapamil, grapefruit juice, diltiazem, aminoglycosides, acyclovir, amphotericin B, and clarithromycin may increase cyclosporine 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 UVB radiation in psoriasis (may increase risk of cancer)
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Evaluate renal and liver functions often by measuring BUN, serum creatinine, serum bilirubin, and liver enzymes; may increase risk of infection and lymphoma; reserve IV use only for those who cannot take PO
Cyclophosphamide (Neosar, Cytoxan)
Chemically related to nitrogen mustards. As an alkylating agent, mechanism of action of active metabolites may involve cross-linking of DNA, which may interfere with growth of normal and neoplastic cells. Demonstrated to be helpful in a variety of skin disorders.
Adult
2.5-3 mg/kg/d PO divided qid for nonmalignant disease
Pediatric
Administer as in adults
Allopurinol may increase risk of bleeding or infection and enhance myelosuppressive effects; may potentiate doxorubicin-induced cardiotoxicity; may reduce digoxin serum levels and antimicrobial effects of quinolones; chloramphenicol may increase half-life while decreasing metabolite concentrations; may increase effect of anticoagulants; coadministration with high doses of phenobarbital may increase rate of metabolism and leukopenic activity; thiazide diuretics may prolong cyclophosphamide-induced leukopenia and neuromuscular blockade by inhibiting cholinesterase activity
Documented hypersensitivity; severely depressed bone marrow function
Pregnancy
D - Fetal risk shown in humans; use only if benefits outweigh risk to fetus
Precautions
Regularly examine hematologic profile (particularly neutrophils and platelets) to monitor for hematopoietic suppression; regularly examine urine for RBCs, which may precede hemorrhagic cystitis
Mycophenolate (CellCept)
Inhibits inosine monophosphate dehydrogenase (IMPDH) and suppresses de novo purine synthesis by lymphocytes, thereby inhibiting their proliferation. Inhibits antibody production.
Adult
1 g PO bid
Pediatric
Not established; 15-23 mg/kg PO bid suggested
May elevate levels of acyclovir and ganciclovir; antacids and cholestyramine decrease absorption, reducing levels (do not administer together); probenecid may increase levels of mycophenolate; salicylates may increase toxicity of mycophenolate
Documented hypersensitivity
Pregnancy
D - Fetal risk shown in humans; use only if benefits outweigh risk to fetus
Precautions
Increases risk of infection; increases toxicity in patients with renal impairment; caution in active peptic ulcer disease
Hydroxychloroquine (Plaquenil)
Inhibits chemotaxis of eosinophils and locomotion of neutrophils and impairs complement-dependent antigen-antibody reactions.
Adult
310 mg (as base) PO qd or bid for several wk depending on response; 155-310 mg/d for prolonged maintenance therapy
Pediatric
3-5 mg/kg/d (as sulfate) PO qd or divided bid; not to exceed 7 mg/kg/d
Serum levels increase with cimetidine; magnesium trisilicate may decrease absorption
Documented hypersensitivity; psoriasis; retinal and visual field changes attributable to 4-aminoquinolones
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Caution in hepatic disease, G-6-PD deficiency, psoriasis, and porphyria; not recommended for long-term use in children; perform periodic (6 mo) ophthalmologic examinations; test periodically for muscle weakness
Thalidomide (Thalomid)
Immunomodulatory agent that may suppress excessive production of TNF-alpha and may down-regulate selected cell-surface adhesion molecules involved in leukocyte migration.
Adult
100-300 mg/d PO qd with water, preferably hs and at least 1 h pc
Start at low end of dose regimen for children <50 kg (110 lb)
Pediatric
Not established
May increase sedation effects of alcohol, barbiturates, chlorpromazine, and reserpine; because of teratogenic effects, women must use 2 additional methods of contraception or abstain from sexual intercourse
Documented hypersensitivity
Pregnancy
X - Contraindicated; benefit does not outweigh risk
Precautions
Perform pregnancy test within the 24-h period prior to initiating therapy, then weekly during first month, followed by monthly tests in women with regular menstrual cycles or q2wk with irregular menstrual cycles; bradycardia may occur; use protective measures (eg, sunscreens, protective clothing) against exposure to sunlight or UV light (eg, tanning beds); prescribing physician must enter STEPS program established by manufacturer
Antibiotics
Several antibiotics (eg, tetracycline, clofazimine) are used for their anti-inflammatory activity.
Tetracycline (Sumycin, Achromycin)
Treats gram-positive and gram-negative organisms, as well as mycoplasmal, chlamydial, and rickettsial infections. Inhibits bacterial protein synthesis by binding with 30S and possibly 50S ribosomal subunit(s).
Adult
250-500 mg PO q6h
Pediatric
<8 years: Not recommended
>8 years: 25-50 mg/kg/d PO divided qid; not to exceed 3 g/d
Bioavailability decreases with antacids containing aluminum, calcium, magnesium, iron, or bismuth subsalicylate; can decrease effects of PO contraceptives, causing breakthrough bleeding and increased risk of pregnancy; tetracyclines can increase hypoprothrombinemic effects of anticoagulants
Documented hypersensitivity; severe hepatic dysfunction
Pregnancy
D - Fetal risk shown in humans; use only if benefits outweigh risk to fetus
Precautions
Photosensitivity may occur with prolonged exposure to sunlight or tanning equipment; reduce dose in renal impairment; consider drug serum level determinations in prolonged therapy; tetracycline use during tooth development (<8 y) can cause permanent discoloration of teeth; Fanconilike syndrome may occur with outdated tetracyclines
Clofazimine (Lamprene)
Lipophilic rhimophenazine dye that inhibits template function of DNA by binding to it. Weakly bactericidal and has anti-inflammatory effects. Although mechanism of action unclear, seems to exert main effect on neutrophils and monocytes in various ways (eg, stimulating phagocytosis and release of lysosomal enzymes).
Adult
50-100 mg PO qd
Pediatric
Not established; has been used for various disease states at a dose of 1 mg/kg/d PO
Dapsone may inhibit anti-inflammatory activity
Documented hypersensitivity
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Severe abdominal symptoms may require exploratory laparotomies; caution in patients with GI problems (eg, abdominal pain, diarrhea); skin discoloration due to drug may result in depression or suicide; apply oil to skin for dryness and ichthyosis; decrease dose with severe hepatic impairment
Nonsteroidal anti-inflammatory drugs
These agents may reduce fever, arthralgia, and pain.
Aspirin (Anacin, Ascriptin, Bayer Aspirin, Bayer Buffered Aspirin)
Lowers elevated body temperature by vasodilating peripheral vessels, thereby enhancing dissipation of excess heat. Acts on heat-regulating center of hypothalamus to reduce fever. Treats mild to moderate pain. Inhibits prostaglandin synthesis, which prevents formation of platelet-aggregating thromboxane A2.
Short-acting anti-inflammatory agent with rapid absorption in proximal GI tract. Optimally effective only when stable serum levels of 150-250 mcg/L are achieved after 3-5 d of treatment. Serum aspirin levels can be checked after 5-10 d of treatment. Maximal anti-inflammatory action is generally achieved within 2-4 wk, with some further benefit occurring as long as 3 mo.
Adult
325-650 mg PO q4-6h; not to exceed 4 g/d
Pediatric
75-100 mg/kg/d PO divided qid; administer with food to minimize gastritis
325-650 mg PO q4-6h in children >40 kg; not to exceed 4 g/d
Effects may decrease with antacids and urinary alkalinizers; corticosteroids decrease salicylate serum levels; additive hypoprothrombinemic effects and increased bleeding time may occur with coadministration of anticoagulants; may antagonize uricosuric effects of probenecid and increase toxicity of phenytoin and valproic acid; doses >2 g/d may potentiate glucose-lowering effect of sulfonylurea drugs
Documented hypersensitivity; liver damage; hypoprothrombinemia; vitamin K deficiency; bleeding disorders; asthma; administration in children (<16 y) with influenzalike illness because of association of aspirin with Reye syndrome
Pregnancy
D - Fetal risk shown in humans; use only if benefits outweigh risk to fetus
Precautions
May cause transient decrease in renal function and aggravate chronic kidney disease; avoid use in patients with severe anemia, those with history of blood coagulation defects, or those who are taking anticoagulants; during therapy, regularly question parents and children about eating habits, abdominal pain or diarrhea, tinnitus or subtle hearing loss, behavioral changes, bruising, and epistaxis; family education about potential complications is essential
Indomethacin (Indochron E-R, Indocin)
Rapidly absorbed; metabolism occurs in liver by demethylation, deacetylation, and glucuronide conjugation. Inhibits prostaglandin synthesis.
Adult
25-50 mg PO bid/tid
75 mg SR PO bid; not to exceed 200 mg/d
Pediatric
1-2 mg/kg/d PO divided bid/qid; not to exceed 4 mg/kg/d or 150-200 mg/d
Coadministration with aspirin increases risk of inducing serious NSAID-related adverse effects; probenecid may increase concentrations and, possibly, toxicity of NSAIDs; may decrease effects of hydralazine, captopril, and beta-blockers; may decrease diuretic effects of furosemide and thiazides; may increase PT when taking anticoagulants (instruct patients to watch for signs of bleeding); may increase risk of methotrexate toxicity; phenytoin levels may be increased when administered concurrently
Documented hypersensitivity; GI bleeding; renal insufficiency
Pregnancy
B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals
D - Fetal risk shown in humans; use only if benefits outweigh risk to fetus
Precautions
Category D in third trimester of pregnancy; acute renal insufficiency, hyperkalemia, hyponatremia, interstitial nephritis, and renal papillary necrosis may occur; increases risk of acute renal failure in patients with preexisting renal disease or compromised renal perfusion; reversible leukopenia may occur (discontinue if persistent leukopenia, granulocytopenia, or thrombocytopenia is present); may cause severe headache in the first few days after initiation of therapy, which usually subsides with continued use; adverse effect sometimes avoided by starting at half dose for 3-4 d with subsequent increase
More on Weber-Christian Disease |
| Overview: Weber-Christian Disease |
| Differential Diagnoses & Workup: Weber-Christian Disease |
 Treatment & Medication: Weber-Christian Disease |
| Follow-up: Weber-Christian Disease |
| Multimedia: Weber-Christian Disease |
| References |
| Further Reading |
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References
Weber EP. A case or relapsing nonsuppurative nodular panniculitis. Brit J Derm. 1925;37:301.
Christian HA. Relapsing febrile nodular nonsuppurative panniculitis. Arch Intern Med. 1928;41:338.
Haubrich WS. Weber and Christian of Weber-Christian disease. Gastroenterology. Apr 2008;134(4):912. [Medline].
Valverde R, Rosales B, Ortiz-de Frutos FJ, Rodriguez-Peralto JL, Ortiz-Romero PL. Alpha-1-antitrypsin deficiency panniculitis. Dermatol Clin. Oct 2008;26(4):447-51, vi. [Medline].
Wu F, Zou CC. Childhood Weber-Christian disease: clinical investigation and virus detection. Acta Paediatr. Nov 2007;96(11):1665-9. [Medline].
Sharma AK, Sharma PR. Idiopathic lobular panniculitis (Weber Christian disease): a case report. Kathmandu Univ Med J (KUMJ). Apr-Jun 2006;4(2):243-5. [Medline].
Abuzahra F, Kovacs S, Beermann T, et al. Treatment of relapsing idiopathic nodular panniculitis with clofazimine. Br J Dermatol. Mar 2005;152(3):582-3. [Medline].
Eravelly J, Waters MF. Thalidomide in Weber-Christian disease. Lancet. Jan 29 1977;1(8005):251. [Medline].
Freedberg IM, Eisen AZ, Wolff K. Panniculitis. In: Fitzpatrick's Dermatology in General Medicine. Vol 1. New York, NY: Mc-Graw Hill; 1999:1275-8.
Hood AF, Kwan TH, Mihm ML, Jr. Panniculitis. In: Primer of Dermatopathology. Boston, MA: Little, Brown and Company; 1993:450.
Lazarus GS. Panniculitis and Disorders of the Subcutaneous Fat. [Full Text].
Lebwohl M. Panniculitis. In: Difficult Diagnoses in Dermatology. New York, NY: Churchill Livingstone; 1988:389-91.
Lemley DE, Ferrans VJ, Fox LM, et al. Cardiac manifestations of Weber-Christian disease: report and review of the literature. J Rheumatol. May 1991;18(5):756-60. [Medline].
Moschella SL, Hurley, HJ. Panniculitides. In: Dermatology. Vol 2. Philadelphia, PA: WB Saunders; 1985:1175-6.
Panush RS, Yonker RA, Dlesk A, et al. Weber-Christian disease. Analysis of 15 cases and review of the literature. Medicine (Baltimore). May 1985;64(3):181-91. [Medline].
Schuval SJ, Frances A, Valderrama E, et al. Panniculitis and fever in children. J Pediatr. Mar 1993;122(3):372-8. [Medline].
Usuki K, Kitamura K, Urabe A, Takaku F. Successful treatment of Weber-Christian disease by cyclosporin A. Am J Med. Aug 1988;85(2):276-8. [Medline].
White JW Jr, Winkelmann RK. Weber-Christian panniculitis: a review of 30 cases with this diagnosis. J Am Acad Dermatol. Jul 1998;39(1):56-62. [Medline].
Winkelmann RK, Dahl PR, Perniciaro C, Dahl PM. Asteroid bodies and other cytoplasmic inclusions in necrobiotic xanthogranuloma with paraproteinemia. J Am Acad Dermatol. Jun 1998;38(6 Pt 1):967-70. [Medline].
Further Reading
- Relevant clinical trials include the following:
- Related eMedicine topics include the following:
Keywords
Weber-Christian disease, idiopathic lobular panniculitis, relapsing febrile nodular nonsuppurative panniculitis, nodular nonsuppurative panniculitis, Pfeifer-Weber-Christian syndrome, lupus panniculitis, factitial panniculitis, pancreatic disease, histiocytic cytophagic panniculitis, skin inflammation, skin rash, skin lesions, hepatomegaly, splenomegaly, treatment, diagnosis
