Wells Syndrome Successfully Treated with Tripterygium Glycosides

Introduction

Wells syndrome (WS; George Wells, 1971), also known as eosinophilic cellulitis, is a rare inflammatory dermatosis of unknown etiology.¹ There are numerous treatment regiments available for WS, including general or local corticosteroids, immunomodulatory or immunosuppressive therapies.² Although general corticosteroids was recommended as the reference and mainstay regiment, potential adverse effects of corticosteroids and relapse after withdrawal often limit its clinical application. Chinese herbal medicine was often used as an useful complementary and alternative therapy to corticosteroids therapy in China. Tripterygium wilfordii and its preparations are extracts of Chinese herbal medicine plant Tripterygium wilfordii Hook. f., which mainly contain triptonide, tripdiolide, and triptolide.³ Tripterygium glycosides (TG) mainly comprises of triptolides, which has been proved to have anti-inflammatory and immunosuppressive effects.³ So, TG has been widely used in the long-term therapy of inflammatory, autoimmune diseases or vasculitis in China, such as psoriasis, rheumatoid arthritis, systemic lupus erythematosus and Behcet’s disease.^4,5 Here, we report a rare case of facial WS that masqueraded as bacterial cellulitis, which successfully treated with tripterygium glycosides.

Case Presentation

A 26-year-old female presented with a one-week history of pruritic, painful, facial erythematous edema covered by vesicles and yellowish crusts. Facial bacterial cellulitis was originally diagnosed by the local general practitioner, and oral antibiotic (cephalexin) was prescribed for 5 days, which did not work. No significant trigger factors, including drugs, arthropod bites or trauma, could be identified. She did not have any systemic symptoms. Clinical examination revealed edematous, erythematous plaques, vesicles and crusts on the face (Figure 1A). The blood routine test revealed a mild peripheral eosinophilia of 0.65×10⁹ cells /L (0.04~0.4×10⁹ cells/L). The following laboratory studies were within normal limits or negative: routine tests of urine and stool, bacterial cultures, hepatic and renal function tests, blood sugar, C-reactive protein, erythrocyte sedimentation rate, rheumatoid factor, antinuclear antibodies and extractable nuclear antigen. Histological findings presented as a dense dermal perivascular and interstitial lymphocytic infiltrate, some histiocytes and multinucleate giant cells, associated with many eosinophils and “flame figures” compatible with WS (Figure 1C and 1D). The patient was treated with intravenous methylprednisolone (60mg/day), resulting in a dramatic improvement in the skin lesions and blood eosinophilia within 48h. Consequently oral methylprednisolone was tapered and discontinued after 8 weeks. However, three month later, the disease relapsed. Because of weight gain and centripetal obesity, the patient refused to oral administration of methylprednisolone. Traditional Chinese Medicine tripterygium glycosides (TG) 60mg/day was prescribed and the lesions completely resolved after 4 weeks. Since then, she no longer took any medication and experienced no recurrence of disease during one  year of follow-up (Figure 1B).

Discussion

Weins et al. proposed the revised diagnostic criteria for Wells syndrome based on case series, which requires at least 2 major criteria and at least 1 minor criteria: Major criteria, (a) Typical clinical features; (b) Chronic relapsing course; (c) Exclusion of systemic disease; (d) Histology: eosinophilic infiltrates, no signs of vasculitis; Minor criteria: (a) Histology with’flame figures’; (b) Histology with granulomatous changes; (c) Peripheral eosinophilia up to 1500/μL; (d) Detectable triggering factor.⁶ Depending on history and clinicopathological correlation, the patient was diagnosed as Wells syndrome. As TG is widely used in inflammatory dermatosis, we prescribed TG to the patient while the disease relapsed, which led to completely remission without any obvious side effects.

Conclusions

In summary, we present a patient with Wells syndrome successfully treated with TG. Despite being limited, our clinical experience supports tripterygium glycosides may be one of the effective treatment alternatives in patients who are dependent on or experience side effects with corticosteroids.

Figure 1 (A) Facial edematous, erythematous plaques partially covered by vesicles and yellowish crusts. (B) The lesions completely resolved. (C) Dense eosinophilic infiltration in the dermis (HE, 40×). (D) Higher magnification with numerous eosinophils and ‘flame figures’ (HE, 200×). Degranulated eosinophils forming flame figures (black arrows).