Management of Churg-Strauss Syndrome (CSS) in the setting of Disseminated Histoplasmosis

Abstract

RATIONALE: Leukocytoclastic vasculitis (LCV) can be due to drugs, infections, connective tissue diseases, allergens, or be idiopathic. METHODS: A medical report of LCV induced by unknown cause was reviewed for possible causative factors. RESULTS: A 26-year female had conjunctivitis, pruritic dermatitis for 8 months with use of Diclofenac and prednisolone due to joints symptoms. Two years before she had birch pollen allergy without skin symptoms. The new rash did not respond to antihistamines. NSAIDS were used for the joint symptoms but worsened the rash. The patient had pruritic erythematous rashes on the face and extremities and conjunctival erythema. Leukopenia, moderate eosinophilia (1.9 G/L), increased erythrocyte sedimentation rate and fibrinogen, increased total IgE (1198 IU/ml), and increased levels of specific IgE (>0.35 kU/l) to D. pteronyssinus., D. farinae, rBetv1 and rBetv2, positive antinuclear antibody and cANCA (anti-proteinase-3) were noted. EBV was found in the pharynx, blood and saliva. Skin biopsy showed leukocytoclastic vasculitis with eosinophils and lymphocytes within the vessel walls. According to the definitions of the Chapel Hill Consensus Conference and ACR Criteria LCV was diagnosed associated with EBV, IgE and NSAIDS. Antiviral drugs, prednisolone, and antihistamines provided decreased skin and eyes findings. CONCLUSIONS: EBV in association with allergens may be associated with development of LCV. Detection of triggers of vasculitis allows etiological treatment and prevention of complications.