WEBER-CHRISTIAN DISEASE: DIAGNOSTIC CHALLENGES AND THERAPEUTIC ADVANCES IN A RARE DISEASE.

BACKGROUND

Weber-Christian disease (WCD) is a rare idiopathic lobular panniculitis. Its diagnosis is challenging due to its ability to mimic infections and malignancies, often leading to unnecessary surgical interventions.

CASE PRESENTATION: A 35-year-old man presented with recurrent fever and painful subcutaneous nodules in 2017. For a period of 14 months, he underwent various interventions for suspected conditions, including empyema (thoracic drainage), spinal abscess (laminectomy), and infected renal cysts (nephrectomy). In March 2018, the diagnosis was confirmed by a skin biopsy, which revealed lobular panniculitis with foamy macrophages. The patient was successfully treated with subcutaneous methotrexate (10 mg/week) and oral methylprednisolone (4-8 mg/day). The patient has remained in clinical remission for approximately 1 year following the last disease flare in March 2022.

CONCLUSION

Weber-Christian disease may present as recurrent surgical emergencies. Early deep tissue biopsy, routine histopathological examination of surgical specimens, and multidisciplinary evaluation are essential to avoid diagnostic delays. At the last follow-up (July 2023), the patient remained in remission for over one year on maintenance therapy with methotrexate and low-dose glucocorticoids.