Wells' syndrome is a multifaceted dermatosis with a wide morphological spectrum, ranging from characteristic cellulitis-like erythema and papula to an unusual presentation of vesicles and pustules. The most important elements for diagnosis are erythemal plaques and histological picture of eosinophilic infiltration of the dermis with 'flame figures' (Plotz et al., in Hautarzt 51:182-186, 2000). Because of its original description as a distinct entity, it has come to be regarded as an abnormal eosinophilic response to a number of causative agents such as herpes simplex virus 2(HSV-2) and toxocara (Ludwig et al., in J Am Acad Dermatol 48:S60-S61, 2003; Bassukas et al., in Cases J 1:356, 2008). Concurrence of WS and malignant diseases as colon cancer, trachea squamous carcinoma, nasopharyngeal carcinoma or angioimmunoblastic lymphadenopathy has been reported (Hirsch et al., in J Dtsch Dermatol Ges 3:530-531, 2005; Renner et al., in Acta Derm Venereol 87:525-528, 2007). Autoimmune diseases, including Systemic lupus erythematosus (SLE) are multi-system disorders of unknown cause and are commonly characterized by protean cutaneous manifestations. To date, few autoimmne disease was found associated with WS except four previous reports of Churg-Strauss syndrome (CSS) and one case of ulcerative colitis (Fujimoto et al., in Clin Exp Dermatol, 2010; Sakaria et al., in J Gastroenterol 42:250-252, 2007). The coexistence of SLE and WS in one patient was not found in literature and our case is the first. Here we described the rare combination and discussed the treatment strategy for this condition.