Case Report
A 54-year-old woman with a history of primary Sjogren’s syndrome for nine years, who had been treated with prednisone and total glucosides of paeony capsule but had discontinued the medications for at least one year, was admitted to the hospital with fever and rashes. Approximately two weeks prior to her current admission, she developed crops of petechiae and purpuric macules on her lower extremities (Figure 1) and was ultimately diagnosed with hypergammaglobulinemic purpura of Waldenström (HGP), a rare complication of Sjogren’s syndrome. She began taking herbal medicine two days prior to admission, with a daily dose of approximately 200ml. The prescription included 5 g Safflower, 5 g Peach kernel, 5 g Ligusticum Chuanxiong Hort, 5 g Ephedra, 10 g Paris Rhizome, 15 g Gegen, 15 g Perilla, 5 g Cassia Twig, 10 g Atractylodes, 10 g Bergamot, 15 g Achyranthes Bidentata Blume, and 10 g Caulis Cissi. The day after starting the herbal treatment, she developed a high fever and new generalized erythematous rashes over her face and trunk. Additionally, she had painful blisters with crusting over her lips and oral mucosa, accompanied by mucopurulent secretion flowing out from her lacrimal punctum (Figure 2). Over the course of two days, the rash spread peripherally to involve more than 80% of her body surface area and progressed rapidly from discrete and confluent macules to blisters. Dermatological examination revealed that all of the rash, except that on her lower extremities, was markedly erythematous, edematous, tender, and peeling off from her body. Nikolsky’s sign was positive.
Her clinical presentation and history of taking herbal medicine strongly suggested a diagnosis of herbal medicine induced TEN(Table1). However, due to the patient’s reluctance to undergo further testing, the exact causative medicine could not be identified. Nonetheless, the clinical picture and timing of symptoms in relation to the herbal medicine intake supported this diagnosis.
Based on the patient’s medical history, laboratory data and physical examination, a diagnosis of hypergammaglobulinemic purpura of Waldenström complicated with primary Sjogren’s syndrome was made. The presence of lower extremities purpura, mild anemia (hemoglobin 104 g/L), hypergammaglobulinemia (IgG 25.56 g/L), elevated ESR (ESR 65 mm/h), and positive anti-extractable nuclear antigen antibodies, including anti-SSA, anti-SSB, and anti-Ro-52, were indicative of this diagnosis. The fact that the purpura rashes on her lower extremities were the only residual sequelae of the disease process with lesions over other parts of the body disappeared was also considered significant.
The treatment plan of stopping the herbal medicine and administering methylprednisolone(500mg/day) and immunoglobulin(20g/day) intravenously was appropriate for managing the patient’s TEN. Pain control, skin, mouth, and eye care, as well as infection prevention, are also crucial components of the management of TEN. In addition, liver and renal protection are important as certain medications used in the treatment of TEN can cause liver or kidney damage. Overall, the management plan appears to be comprehensive and appropriate for the patient’s condition.