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.