You are in: eMedicine Specialties > Pediatrics: General Medicine > Rheumatology Weber-Christian DiseaseArticle Last Updated: Jun 14, 2006AUTHOR AND EDITOR INFORMATIONSection 1 of 10
  Author: Moise L Levy, MD, Professor, Departments of Pediatrics and Dermatology, Baylor College of Medicine; Chief, Department of Dermatology, Texas Children's Hospital Moise L Levy is a member of the following medical societies: American Academy of Dermatology, American Academy of Pediatrics, American Society for Laser Medicine and Surgery, Harris County Medical Society, Society for Investigative Dermatology, and Texas Medical Association Coauthor(s): Oren Lifshitz, MD, Staff Physician, Department of Dermatology, Cleveland Clinic Foundation; Heather A Klein, BS Editors: Mary L Windle, PharmD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy, Pharmacy Editor, eMedicine.com, Inc; Thomas JA Lehman, MD, Clinical Professor of Pediatrics, Department of Pediatrics, Division of Pediatric Rheumatology, Weill-Cornell University; Chief, Hospital for Special Surgery; Daniel Rauch, MD, FAAP, Director, Pediatric Hospitalist Program, Associate Professor, Department of Pediatrics, New York University School of Medicine; Barry L Myones, MD, Associate Professor, Departments of Pediatrics and Immunology, Pediatric Rheumatology Section, Baylor College of Medicine; Director of Research, Pediatric Rheumatology Center, Texas Children's Hospital Author and Editor Disclosure Synonyms and related keywords: Weber-Christian disease, idiopathic lobular panniculitis, relapsing febrile nodular nonsuppurative panniculitis, nodular nonsuppurative panniculitis, Pfeifer-Weber-Christian syndrome INTRODUCTIONSection 2 of 10
  BackgroundIn 1892, Pfeifer first described the skin condition now known as Weber-Christian disease, or idiopathic lobular panniculitis. In 1925, Weber further depicted the syndrome. In 1928, Christian emphasized the significance of fever as part of the syndrome. Henceforth, the syndrome became known as Weber-Christian disease. The nomenclature of this and other related diseases is confusing, and some authors believe that the eponym should be abandoned and that more specific diagnoses should be made on the basis of pathogenesis or cause. Diseases such as lupus panniculitis, factitial panniculitis, panniculitis associated with pancreatic disease, histiocytic cytophagic panniculitis, and alpha1-antitrypsin deficiency panniculitis have been differentiated from Weber-Christian disease. As Weber-Christian disease is elucidated further, additional diseases will probably be identified as being distinct from Weber-Christian disease. PathophysiologyWeber-Christian disease is a skin condition that features recurring inflammation in the fat layer of the skin. The involved areas of skin manifest as recurrent crops of erythematous, sometimes tender, edematous subcutaneous nodules. The lesions are symmetric in distribution, and the thighs and lower legs are affected most frequently. Malaise, fever, and arthralgias frequently occur. Nausea, vomiting, abdominal pain, weight loss, and hepatomegaly may also occur. Because its etiology is unknown, Weber-Christian disease is often referred to as idiopathic lobular panniculitis. FrequencyUnited StatesBecause of the ambiguity of this diagnosis versus other closely related conditions, the frequency of Weber-Christian disease has not been determined. Mortality/MorbidityThe course of Weber-Christian disease is variable and depends on which organs are affected.
RaceNo racial predilection appears to exist. SexThe disease occurs more often in women, who comprise approximately 75% of reported cases. AgeWeber-Christian disease may occur in young children, but it has been reported most frequently in people in the fourth to seventh decades of life. CLINICALSection 3 of 10
HistoryPatients with Weber-Christian disease typically have cutaneous and, less frequently, systemic symptoms.
PhysicalPhysical examination reveals erythematous, edematous, and tender subcutaneous nodules.
CausesBecause its etiology is unknown, Weber-Christian disease is called idiopathic lobular panniculitis. Patients with Weber-Christian disease do not report a history of physical trauma.
DIFFERENTIALSSection 4 of 10
Polyarteritis Nodosa Sarcoidosis Systemic Lupus Erythematosus Systemic Sclerosis Vasculitis and Thrombophlebitis
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| Drug Name | Prednisone (Deltasone, Meticorten, Orasone) |
|---|---|
| Description | May decrease inflammation by reversing increased capillary permeability and suppressing PMN activity. |
| Adult Dose | 40-60 mg/d PO qd or divided bid/qid; taper over 2 wk as symptoms resolve |
| Pediatric Dose | 4-5 mg/m2/d PO; alternatively, 0.5-2 mg/kg PO divided bid/qid; taper over 2 wk as symptoms resolve |
| Contraindications | Documented hypersensitivity; viral infection; peptic ulcer disease; hepatic dysfunction; connective tissue infections; fungal or tubercular skin infections; GI ulceration or bleeding |
| Interactions | 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 |
| Pregnancy | B - Usually safe but benefits must outweigh the risks. |
| 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 |
These agents inhibit key factors that mediate immune reactions, which in turn decrease inflammatory responses. They may have potential long-term therapeutic response.
| Drug Name | Azathioprine (Imuran) |
|---|---|
| Description | Antagonizes purine metabolism and inhibits synthesis of DNA, RNA, and proteins. May decrease proliferation of immune cells, which results in lower autoimmune activity. |
| Adult Dose | 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 Dose | Initial dose: 2-5 mg/kg/d PO/IV Maintenance dose: 1-2 mg/kg/d PO/IV |
| Contraindications | Documented hypersensitivity; low levels of serum TPMT |
| Interactions | 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 |
| Pregnancy | D - Unsafe in pregnancy |
| 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 |
| Drug Name | Cyclosporine (Neoral, Sandimmune) |
|---|---|
| Description | 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 Dose | 2.5-5 mg/kg/d PO in divided doses |
| Pediatric Dose | Administer as in adults |
| Contraindications | Documented hypersensitivity; uncontrolled hypertension or malignancies; concomitant administration with PUVA or UVB radiation in psoriasis (may increase risk of cancer) |
| Interactions | 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 |
| 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 enzymes; may increase risk of infection and lymphoma; reserve IV use only for those who cannot take PO |
| Drug Name | Cyclophosphamide (Neosar, Cytoxan) |
|---|---|
| Description | 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 Dose | 2.5-3 mg/kg/d PO divided qid for nonmalignant disease |
| Pediatric Dose | Administer as in adults |
| Contraindications | Documented hypersensitivity; severely depressed bone marrow function |
| Interactions | 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 |
| Pregnancy | D - Unsafe in pregnancy |
| Precautions | Regularly examine hematologic profile (particularly neutrophils and platelets) to monitor for hematopoietic suppression; regularly examine urine for RBCs, which may precede hemorrhagic cystitis |
| Drug Name | Mycophenolate (CellCept) |
|---|---|
| Description | Inhibits inosine monophosphate dehydrogenase (IMPDH) and suppresses de novo purine synthesis by lymphocytes, thereby inhibiting their proliferation. Inhibits antibody production. |
| Adult Dose | 1 g PO bid |
| Pediatric Dose | Not established; 15-23 mg/kg PO bid suggested |
| Contraindications | Documented hypersensitivity |
| Interactions | 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 |
| 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 |
| Drug Name | Hydroxychloroquine (Plaquenil) |
|---|---|
| Description | Inhibits chemotaxis of eosinophils and locomotion of neutrophils and impairs complement-dependent antigen-antibody reactions. |
| Adult Dose | 310 mg (as base) PO qd or bid for several wk depending on response; 155-310 mg/d for prolonged maintenance therapy |
| Pediatric Dose | 3-5 mg/kg/d (as sulfate) PO qd or divided bid; not to exceed 7 mg/kg/d |
| Contraindications | Documented hypersensitivity; psoriasis; retinal and visual field changes attributable to 4-aminoquinolones |
| Interactions | Serum levels increase with cimetidine; magnesium trisilicate may decrease absorption |
| Pregnancy | C - Safety for use during pregnancy has not been established. |
| 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 |
| Drug Name | Thalidomide (Thalomid) |
|---|---|
| Description | Immunomodulatory agent that may suppress excessive production of TNF-alpha and may down-regulate selected cell-surface adhesion molecules involved in leukocyte migration. |
| Adult Dose | 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 Dose | Not established |
| Contraindications | Documented hypersensitivity |
| Interactions | 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 |
| Pregnancy | X - Contraindicated in pregnancy |
| 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 |
Several antibiotics (eg, tetracycline, clofazimine) are used for their anti-inflammatory activity.
| Drug Name | Tetracycline (Sumycin, Achromycin) |
|---|---|
| Description | 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 Dose | 250-500 mg PO q6h |
| Pediatric Dose | <8 years: Not recommended >8 years: 25-50 mg/kg/d PO divided qid; not to exceed 3 g/d |
| Contraindications | Documented hypersensitivity; severe hepatic dysfunction |
| Interactions | 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 |
| Pregnancy | D - Unsafe in pregnancy |
| 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 |
| Drug Name | Clofazimine (Lamprene) |
|---|---|
| Description | 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 upon neutrophils and monocytes in a variety of ways (eg, stimulating phagocytosis and release of lysosomal enzymes). |
| Adult Dose | 50-100 mg PO qd |
| Pediatric Dose | Not established; has been used for various disease states at a dose of 1 mg/kg/d PO |
| Contraindications | Documented hypersensitivity |
| Interactions | Dapsone may inhibit anti-inflammatory activity |
| Pregnancy | C - Safety for use during pregnancy has not been established. |
| 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 |
These agents may reduce fever, arthralgia, and pain.
| Drug Name | Aspirin (Anacin, Ascriptin, Bayer Aspirin, Bayer Buffered Aspirin) |
|---|---|
| Description | 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 up to 3 mo. |
| Adult Dose | 325-650 mg PO q4-6h; not to exceed 4 g/d |
| Pediatric Dose | 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 |
| Contraindications | 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 |
| Interactions | 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 |
| Pregnancy | D - Unsafe in pregnancy |
| 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 |
| Drug Name | Indomethacin (Indochron E-R, Indocin) |
|---|---|
| Description | Rapidly absorbed; metabolism occurs in liver by demethylation, deacetylation, and glucuronide conjugation. Inhibits prostaglandin synthesis. |
| Adult Dose | 25-50 mg PO bid/tid 75 mg SR PO bid; not to exceed 200 mg/d |
| Pediatric Dose | 1-2 mg/kg/d PO divided bid/qid; not to exceed 4 mg/kg/d or 150-200 mg/d |
| Contraindications | Documented hypersensitivity; GI bleeding; renal insufficiency |
| Interactions | 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 |
| Pregnancy | B - Usually safe but benefits must outweigh the risks. |
| 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 |
Weber-Christian Disease excerpt
Article Last Updated: Jun 14, 2006
