Wells syndrome associated with Churg-Strauss syndrome

556 Letters

J AM ACAD DERMATOL SEPTEMBER 2000

Wells syndrome associated with ChurgStrauss syndrome To the Editor: Churg-Strauss syndrome (CSS), or allergic angiitis and granulomatosis, is a multisystem granulomatous vasculitis occurring in patients with asthma and characterized by eosinophilic infiltration of systemic organs and peripheral eosinophilia. Wells syndrome is a rare recurrent inflammatory dermatosis of indurated erythematous plaques with peripheral eosinophilia. The origin of Wells syndrome is unknown, but it has been reported in association with insect bites, parasites, viral infections, fungal infections, drugs, leukemic and myeloproliferative disorders, and atopic dermatitis.1-3 Rarely, it has been associated with idiopathic hypereosinophilic syndrome, suggesting that there is an abnormal response of eosinophils.4 A 43-year-old woman had multiple grouped erythematous papules on the right palm and forearm

J AM ACAD DERMATOL VOLUME 43, NUMBER 3

for 20 days before her first visit. The papules progressed to infiltrative plaques in 2 weeks. She also reported a tingling or numbness on both soles and the right palm. A skin biopsy specimen from a papule showed a dense lymphohistiocytic granulomatous infiltrate of the dermal vessels. Several granulomatous foci with prominent epithelioid and giant cells were also found in the dermis. The skin lesions gradually receded with oral corticosteroids and dapsone. However, over the next few weeks, the nerve symptoms worsened to the extent that a neuropathy developed, including paresthesias with motor weakness of the left sole and sensory loss of the 4th and 5th fingers of the right hand. A biopsy specimen from the sural nerve showed perineural vasculitis with extensive loss of myelinated fibers. The patient had been treated for bronchial asthma for 2 years. CSS was diagnosed by the combined findings of vasculitis of the skin and the peripheral nerves in an asthmatic patient. Two months later, painful edematous plaques developed on the right forearm and the dorsum of the right hand. As the edema worsened, multiple vesicles and bullae developed on the surface of the plaques. Histologic study of the edematous plaques revealed a diffuse infiltrate of eosinophils and flame figures in the entire dermis. The new skin lesions prompted the diagnosis of Wells syndrome by their clinical features of acute cellulitislike swelling and typical histopathologic findings. Complete blood cell counts revealed marked leukocytosis (20,800/mm3) with eosinophilia (55%). The leukocytosis and eosinophilia were rapidly normalized with oral corticosteroids. Serologic tests revealed an increased IgE level of 2422 IU/mL (normal, <100 IU/mL), increased C4 level of 51 mg/dL (normal, 10-40 mg/dL), and positive perinuclear anti-neutrophilic cytoplasmic antibody (normal, negative). Chest radiography revealed coarse bronchovascular lung markings in the right lower lung field, compatible with the chronic bronchial pathology of bronchial asthma. Although the two kinds of skin lesions resolved with corticosteroids and dapsone treatment, the nerve symptoms of CSS were resistant to the therapy. Combined therapy with oral prednisolone (1 mg/kg per day) and cyclophosphamide (100 mg/day) as well as intermittent pulse therapy with intravenous methylprednisolone (100 mg/day for 4 days) alleviated the sensorimotor loss gradually, but the sensation of pain at the extremities and foot dropping persisted. Wells syndrome is regarded as a distinctive disease entity manifesting itself by a recurrence of acute cellulitis-like lesions. On the other

Letters 557

hand, Wells syndrome may be a histopathologic reaction pattern in various dermatoses with tissue eosinophilia, such as pemphigoid, herpes gestationis, severe prurigo, eczema, dermatophyte infection, arthropod bites, and follicular mucinosis.5 To our knowledge, this is the first case report of Wells syndrome in association with CSS. An underlying process in CSS, mediated by eosinophils, may act in a pathogenic fashion to induce Wells syndrome. Intriguingly, Churg and Strauss6 previously suggested the possibility that Wells syndrome is related to CSS on the basis of its histopathologic changes. Recently, Wells syndrome has been reported in association with idiopathic hypereosinophilic syndrome4 and eosinophilic myositis.7 These conditions have common features including peripheral and/or tissue eosinophilia and widespread eosinophilic infiltrates in multiple organs. In our case, two conditions overlapped in the same patient with a time gap; the papular eruption of CSS developed first and was later followed by an acute cellulitis-like swelling characteristic of Wells syndrome. Therefore CSS should be included in that category of systemic diseases, which may induce Wells syndrome by the pathogenic influence of infiltrating eosinophils in the skin. Seung-Chul Lee, MD, PhD Sham-Shik Shin, MD Jee-Bum Lee, MD Young-Ho Won, MD, PhD Department of Dermatology Chonnam University Medical School 8 Hak-dong, Kwangju 501-757, Korea REFERENCES 1. Chumbly LC, Harrison EG Jr, DeRemee RA. Allergic granulomatosis and angiitis (Churg-Strauss syndrome): report and analysis of 30 cases. Mayo Clin Proc 1977;52:477-84. 2. Lanham JG, Elkon KB, Pusey CD, Hughes GR. Systemic vasculitis with asthma and eosinophilia: a clinical approach to the ChurgStrauss syndrome. Medicine 1984;63:65-81. 3. Davis MDP, Daoud MS, McEvoy MT, Su WPD. Cutaneous manifestations of Churg-Strauss syndrome: a clinicopathologic correlation. J Am Acad Dermatol 1997;37:199-203. 4. Bogenrieder T, Griese DP, Schiffner R, Büttner R, Riegger GAJ, Hohenleutner U, et al. Wells syndrome associated with idiopathic hypereosinophilic syndrome. Br J Dermatol 1997;137: 978-82. 5. Schorr WF, Tauscheck AL, Dickson KB, Melski JW. Eosinophilic cellulitis (Wells syndrome): histologic and clinical features in arthropod bite reactions. J Am Acad Dermatol 1984;11:1043-9. 6. Churg J, Strauss L. Allergic granulomatosis, allergic angiitis, and periarteritis nodosa. Am J Pathol 1951;27:277-301. 7. Trüeb RM, Lübbe J, Torricelli R, Panizzon RG, Wüthrich B, Burg G. Eosinophilic myositis with eosinophilic cellulitis-like skin lesions. Arch Dermatol 1997;133:203-6. 16/8/106516 doi:10.1067/mjd.2000.106516