Hyper Immunoglobulin E Syndrome (HIES) is a primary immunodeficiency disorder characterized by the classic triad of recurrent staphylococcal abscesses, frequent airway infections, and increased serum immunoglobulin E. It is now widely accepted as a multisystem disorder with involvement of the skeletal, connective tissue, dental, and vascular systems. Lymphoma has been reported to occur at an increased frequency. Follicu-lotropic mycosis fungoides (FMF) is a rare variant of cutaneous T-cell lymphoma in which the neoplastic T lymphocytes display tropism for the follicular epithelium. We report a case of a 14-year old Filipino male with the classic type of HIES presenting with severe eczema associated with intractable pruritus, recurrent respiratory tract infections, cold abscesses, and a serum IgE of 9,350 IU/ml. Skin biopsy also revealed findings consistent with FMF. Narrowband UVB (NB-UVB) phototherapy was initiated three times a week with continuation of supportive medication. Patient reported significant relief of pruritus and gradual improvement of eczematous lesions after one month.
Job Syndrome ;