Weber-Christian disease (WCD) is a type of panniculitis with subcutaneous nodules and fat lobule inflammation.
It is a rare inflammatory disorder that involves subcutaneous fat. Panniculitis is inflammation of the fat layer present under the skin.
Tender nodules on limbs and trunk with fever or systemic symptoms characterize this disease.
First described by Weber in 1883, Weber-Christian Disease was later elaborated by Christian in 1925.
Fever and nodules without abscess separate disease from infectious conditions. Weber-Christian disease is nodular panniculitis with unknown causes and symptoms.
Subcutaneous fat inflammation leads to nodules, scarring, and calcification formation.
Epidemiology
Weber-Christian disease is rare in adults and even rarer in children due to ambiguity with closely related conditions.
Prevalence and incidence of WCD is unknown in US and worldwide.
It is more common in women. The disease is rarely seen in the pediatric population. It is observed in people between 40 to 70 years old with 75% of cases in women after 20 years old.
Rare cases of primary panniculitis are seen by physicians only once or a few times.
Anatomy
Pathophysiology
It causes recurrent inflammation in subcutaneous fat layer occurs with erythematous, tender, edematous nodules on skin areas.
Symmetric lesions distribution on thighs and lower legs. Microscopy shows nodular inflammatory pattern in fat lobules.
Foamy macrophages and giant cells clear necrotic fat debris. The damaged fat cells release intracellular content to initiate a local inflammatory response.
Chronic inflammation/necrosis cause fibrosis and calcification of subcutaneous tissue.
Etiology
Weber-Christian disease is idiopathic lobular panniculitis with unknown cause and no trauma history reported.
High immune complex levels suggest immunologically mediated reaction in patients. WCD and alpha1-antitrypsin deficiency share abnormal regulation of inflammation.
Cyclosporine may trigger T-cell inflammatory responses, but no link with infections or post viral responses found.
Genetics
Prognostic Factors
WCD is a severe type of panniculitis with systemic symptoms.
Prognosis depends on affected organs, severity, and response to treatment.
Weber-Christian disease affects multiple organs lead to serious health complications and even death.
Patients with only skin symptoms may experience flare-ups and periods of improvement for years before resolving.
Clinical History
Collect details including chief complaints, history of present illness and medical history to understand clinical history of patient.
Physical Examination
Skin and Subcutaneous Tissue Examination
Abdominal Examination
Respiratory examination
Age group
Associated comorbidity
Associated activity
Acuity of presentation
Acute symptoms are:
Painful nodules on the extremities or trunk, High-grade fever associated with chills, night sweats, severe fatigue, malaise, and myalgia.
Chronic symptoms are:
Multiple episodes of painful nodules, chronic fatigue, malaise, and progressive weight.
Differential Diagnoses
Juvenile Systemic Sclerosis
Polyarteritis Nodosa Imaging
Pediatric Sarcoidosis
Pediatric Systemic Lupus Erythematosus
Laboratory Studies
Imaging Studies
Procedures
Histologic Findings
Staging
Treatment Paradigm
Weber-Christian disease has no consistent treatment. Corticosteroids and immunosuppressive drugs show promise in clinical settings.
Effective treatments include use of fibrinolytic agents, hydroxychloroquine, thalidomide, cyclophosphamide, tetracycline, cyclosporine, and mycophenolate.
Steroids suppress acute exacerbations effectively. NSAIDs used to reduce fever and relieve pain.
Hospitalization may be needed for severe cases with visceral organ inflammation or wound care.
Weber-Christian disease (WCD) is a type of panniculitis with subcutaneous nodules and fat lobule inflammation.
It is a rare inflammatory disorder that involves subcutaneous fat. Panniculitis is inflammation of the fat layer present under the skin.
Tender nodules on limbs and trunk with fever or systemic symptoms characterize this disease.
First described by Weber in 1883, Weber-Christian Disease was later elaborated by Christian in 1925.
Fever and nodules without abscess separate disease from infectious conditions. Weber-Christian disease is nodular panniculitis with unknown causes and symptoms.
Subcutaneous fat inflammation leads to nodules, scarring, and calcification formation.
Weber-Christian disease is rare in adults and even rarer in children due to ambiguity with closely related conditions.
Prevalence and incidence of WCD is unknown in US and worldwide.
It is more common in women. The disease is rarely seen in the pediatric population. It is observed in people between 40 to 70 years old with 75% of cases in women after 20 years old.
Rare cases of primary panniculitis are seen by physicians only once or a few times.
It causes recurrent inflammation in subcutaneous fat layer occurs with erythematous, tender, edematous nodules on skin areas.
Symmetric lesions distribution on thighs and lower legs. Microscopy shows nodular inflammatory pattern in fat lobules.
Foamy macrophages and giant cells clear necrotic fat debris. The damaged fat cells release intracellular content to initiate a local inflammatory response.
Chronic inflammation/necrosis cause fibrosis and calcification of subcutaneous tissue.
Weber-Christian disease is idiopathic lobular panniculitis with unknown cause and no trauma history reported.
High immune complex levels suggest immunologically mediated reaction in patients. WCD and alpha1-antitrypsin deficiency share abnormal regulation of inflammation.
Cyclosporine may trigger T-cell inflammatory responses, but no link with infections or post viral responses found.
WCD is a severe type of panniculitis with systemic symptoms.
Prognosis depends on affected organs, severity, and response to treatment.
Weber-Christian disease affects multiple organs lead to serious health complications and even death.
Patients with only skin symptoms may experience flare-ups and periods of improvement for years before resolving.
Collect details including chief complaints, history of present illness and medical history to understand clinical history of patient.
Skin and Subcutaneous Tissue Examination
Abdominal Examination
Respiratory examination
Acute symptoms are:
Painful nodules on the extremities or trunk, High-grade fever associated with chills, night sweats, severe fatigue, malaise, and myalgia.
Chronic symptoms are:
Multiple episodes of painful nodules, chronic fatigue, malaise, and progressive weight.
Juvenile Systemic Sclerosis
Polyarteritis Nodosa Imaging
Pediatric Sarcoidosis
Pediatric Systemic Lupus Erythematosus
Weber-Christian disease has no consistent treatment. Corticosteroids and immunosuppressive drugs show promise in clinical settings.
Effective treatments include use of fibrinolytic agents, hydroxychloroquine, thalidomide, cyclophosphamide, tetracycline, cyclosporine, and mycophenolate.
Steroids suppress acute exacerbations effectively. NSAIDs used to reduce fever and relieve pain.
Hospitalization may be needed for severe cases with visceral organ inflammation or wound care.
Pediatrics, General
Create a hygienic and infection-free environment to reduce infection control.
Avoid close contact with individuals who are sick or diagnose with contagious infections.
Patient should wear warm clothes during winter to prevent vasoconstriction and panniculitis.
Patient should participate in psychological counselling to manage their stress.
Proper awareness about WCD should be provided and its related causes with management strategies.
Appointments with a physician and preventing recurrence of disorder is an ongoing life-long effort.
Pediatrics, General
Prednisone:
It prevents inflammation and controls rate of protein synthesis to suppress migration of polymorphonuclear leukocytes.
Pediatrics, General
Azathioprine:
It decreases proliferation of immune cells to reduce autoimmune activity.
Cyclosporine:
It suppresses cellular and humoral immunity for a various organ.
Mycophenolate:
It inhibits inosine monophosphate dehydrogenase to suppress purine synthesis.
Thalidomide:
It suppresses excessive production of TNF-alpha molecules involved in leukocyte migration.
Pediatrics, General
Tetracycline:
It inhibits bacterial protein synthesis to bind with 30S ribosomal subunit.
Clofazimine:
It inhibits template function of DNA with weakly bactericidal and anti-inflammatory effects.
Pediatrics, General
Indomethacin:
It is rapidly absorbed to inhibit prostaglandin synthesis.
Pediatrics, General
No surgical intervention is indicated for WCD.
Pediatric dermatologist assists with causes of panniculitis suggest skin biopsy if needed.
Pediatric rheumatologist and infectious disease specialist consult for diagnosis and treatment plan.
Pediatrics, General
In the initial treatment phase, the goal is to control active inflammation and stabilize the patient’s vital signs.
Pharmacologic therapy is effective in the treatment phase as it includes use of corticosteroids, immunomodulators, antibiotics, and NSAIDs drugs.
In supportive care and management phase, patients should receive required attention such as lifestyle modification.
The regular follow-up visits with the physician are scheduled to check the improvement of patients along with treatment response.
Weber-Christian disease (WCD) is a type of panniculitis with subcutaneous nodules and fat lobule inflammation.
It is a rare inflammatory disorder that involves subcutaneous fat. Panniculitis is inflammation of the fat layer present under the skin.
Tender nodules on limbs and trunk with fever or systemic symptoms characterize this disease.
First described by Weber in 1883, Weber-Christian Disease was later elaborated by Christian in 1925.
Fever and nodules without abscess separate disease from infectious conditions. Weber-Christian disease is nodular panniculitis with unknown causes and symptoms.
Subcutaneous fat inflammation leads to nodules, scarring, and calcification formation.
Weber-Christian disease is rare in adults and even rarer in children due to ambiguity with closely related conditions.
Prevalence and incidence of WCD is unknown in US and worldwide.
It is more common in women. The disease is rarely seen in the pediatric population. It is observed in people between 40 to 70 years old with 75% of cases in women after 20 years old.
Rare cases of primary panniculitis are seen by physicians only once or a few times.
It causes recurrent inflammation in subcutaneous fat layer occurs with erythematous, tender, edematous nodules on skin areas.
Symmetric lesions distribution on thighs and lower legs. Microscopy shows nodular inflammatory pattern in fat lobules.
Foamy macrophages and giant cells clear necrotic fat debris. The damaged fat cells release intracellular content to initiate a local inflammatory response.
Chronic inflammation/necrosis cause fibrosis and calcification of subcutaneous tissue.
Weber-Christian disease is idiopathic lobular panniculitis with unknown cause and no trauma history reported.
High immune complex levels suggest immunologically mediated reaction in patients. WCD and alpha1-antitrypsin deficiency share abnormal regulation of inflammation.
Cyclosporine may trigger T-cell inflammatory responses, but no link with infections or post viral responses found.
WCD is a severe type of panniculitis with systemic symptoms.
Prognosis depends on affected organs, severity, and response to treatment.
Weber-Christian disease affects multiple organs lead to serious health complications and even death.
Patients with only skin symptoms may experience flare-ups and periods of improvement for years before resolving.
Collect details including chief complaints, history of present illness and medical history to understand clinical history of patient.
Skin and Subcutaneous Tissue Examination
Abdominal Examination
Respiratory examination
Acute symptoms are:
Painful nodules on the extremities or trunk, High-grade fever associated with chills, night sweats, severe fatigue, malaise, and myalgia.
Chronic symptoms are:
Multiple episodes of painful nodules, chronic fatigue, malaise, and progressive weight.
Juvenile Systemic Sclerosis
Polyarteritis Nodosa Imaging
Pediatric Sarcoidosis
Pediatric Systemic Lupus Erythematosus
Weber-Christian disease has no consistent treatment. Corticosteroids and immunosuppressive drugs show promise in clinical settings.
Effective treatments include use of fibrinolytic agents, hydroxychloroquine, thalidomide, cyclophosphamide, tetracycline, cyclosporine, and mycophenolate.
Steroids suppress acute exacerbations effectively. NSAIDs used to reduce fever and relieve pain.
Hospitalization may be needed for severe cases with visceral organ inflammation or wound care.
Pediatrics, General
Create a hygienic and infection-free environment to reduce infection control.
Avoid close contact with individuals who are sick or diagnose with contagious infections.
Patient should wear warm clothes during winter to prevent vasoconstriction and panniculitis.
Patient should participate in psychological counselling to manage their stress.
Proper awareness about WCD should be provided and its related causes with management strategies.
Appointments with a physician and preventing recurrence of disorder is an ongoing life-long effort.
Pediatrics, General
Prednisone:
It prevents inflammation and controls rate of protein synthesis to suppress migration of polymorphonuclear leukocytes.
Pediatrics, General
Azathioprine:
It decreases proliferation of immune cells to reduce autoimmune activity.
Cyclosporine:
It suppresses cellular and humoral immunity for a various organ.
Mycophenolate:
It inhibits inosine monophosphate dehydrogenase to suppress purine synthesis.
Thalidomide:
It suppresses excessive production of TNF-alpha molecules involved in leukocyte migration.
Pediatrics, General
Tetracycline:
It inhibits bacterial protein synthesis to bind with 30S ribosomal subunit.
Clofazimine:
It inhibits template function of DNA with weakly bactericidal and anti-inflammatory effects.
Pediatrics, General
Indomethacin:
It is rapidly absorbed to inhibit prostaglandin synthesis.
Pediatrics, General
No surgical intervention is indicated for WCD.
Pediatric dermatologist assists with causes of panniculitis suggest skin biopsy if needed.
Pediatric rheumatologist and infectious disease specialist consult for diagnosis and treatment plan.
Pediatrics, General
In the initial treatment phase, the goal is to control active inflammation and stabilize the patient’s vital signs.
Pharmacologic therapy is effective in the treatment phase as it includes use of corticosteroids, immunomodulators, antibiotics, and NSAIDs drugs.
In supportive care and management phase, patients should receive required attention such as lifestyle modification.
The regular follow-up visits with the physician are scheduled to check the improvement of patients along with treatment response.
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