ABSTRACT
Common variable immunodeficiency is the most common severe primary immunodeficiency. Most common variable immunodeficiency patients have progressive hypogammaglobulinemia involving all immunoglobulin classes, poor or absent antibody responses, and recurrent bacterial infections, usually of the sino-respiratory tract. Some may present with complicated cutaneous infections like furunculosis (J Allergy Clin Immunol; 109: 581) or recurrent cutaneous warts. Here, we report the case of an 18-year-old male diagnosed with common variable immunodeficiency who had extensive cutaneous warts that resolved within 2 months of starting weekly infusions of subcutaneous immunoglobulin.
Subject(s)
Common Variable Immunodeficiency/complications , Warts/etiology , Adolescent , Humans , Immunoglobulins, Intravenous , Male , Warts/drug therapyABSTRACT
Angioedema occurs when there is fluid leakage into the deep dermis of the skin and underlying subcutaneous tissues. Affected individuals usually present with swelling of the face or extremities. Acquired angioedema is an uncommon but potentially life-threatening disease in the older adult population. After the individual is cleared of the initial danger period, a thorough workup for an underlying etiology must be done. We report a 62-year-old male presenting with significant tongue swelling who was diagnosed with acquired angioedema. He had autoantibodies to C1 esterase inhibitor and was subsequently diagnosed with a lymphoma. Angioedema should be recognized by clinicians as a potential presentation of a more ominous malignancy.
Subject(s)
Angioedema/blood , Autoantibodies/blood , Complement C1 Inhibitor Protein/immunology , Lymphoma/diagnosis , Tongue Diseases/blood , Acute Disease , Angioedema/etiology , Diagnosis, Differential , Humans , Lymphoma/blood , Lymphoma/complications , Male , Middle Aged , Tongue Diseases/etiologyABSTRACT
Common variable immunodeficiency (CVID) is a primary immunodeficiency characterized by hypogammaglobulinemia, poor antibody responses, and recurrent bacterial infections, usually of the sinorespiratory tract. A not uncommon complication is granuloma of the lungs, spleen, liver, and/or skin. We report the case of an 18-year-old boy with CVID and chronic granulomas of the left arm (since 13 years of age) refractory to treatment with antibiotics, intravenous immunoglobulin, antifungal agents, systemic and intralesional steroids, IFN-gamma, cyclosporine, methotrexate, hydroxychloroquine, localized radiation therapy, and surgical excision. The lesions improved after treatment with the systemic administration of the TNF-alpha inhibitor etanercept for 1 year. Etanercept prevents soluble TNF from binding to its cell membrane receptor, leading to inhibition of its inflammatory cascade. We recommend further trials of etanercept in patients with CVID with noninfectious recalcitrant granulomas.
