ABSTRACT
Patients with autoimmune urticaria (AIU) and positive autologous serum skin test (ASST) represent a more serious type of chronic urticaria that does not respond to treatment with antihistamines, but responds completely to systemic corticosteroids. Because of the chronic course of the disease, there is a risk of side-effects. Cyclosporin A (CsA) is an alternative treatment for patients with AIU. In order to determine the efficacy of CsA at the lowest possible dose in patients with chronic idiopathic urticaria and positive ASST, 30 patients were included in a 5-month study with a follow-up one year after the end of treatment. All patients had positive ASST before treatment and autoantibodies were present in 73%. Twenty- three patients completed the study and responded to low-dose CsA treatment. Three patients did not respond to a dose of 2.5 mg/kg CsA, and 4 patients dropped-out due to side-effects. After the first month of treatment, an improvement of 31% was noted, reaching 88% after the fifth month of treatment. The mean dose of CsA was 2.16 mg/kg for the first month and 0.55 mg/kg for the fifth month. Three to 6 months after the end of the study, the ASST was repeated and was negative in 78.3% of patients. At the one-year follow-up, 20 patients were symptom-free (87%) and 3 had relapsed (13%). CsA, even in low-doses, can be an effective and short-term treat- ment with minimum side-effects in patients with AIU.
Subject(s)
Autoimmune Diseases/drug therapy , Cyclosporine/administration & dosage , Immunosuppressive Agents/administration & dosage , Urticaria/drug therapy , Adolescent , Adult , Aged , Autoantibodies/analysis , Autoimmune Diseases/immunology , Chronic Disease , Cyclosporine/adverse effects , Female , Humans , Immunosuppressive Agents/adverse effects , Male , Middle Aged , Urticaria/immunology , Young AdultABSTRACT
BACKGROUND: Combined immunodeficiency disorders comprise a heterogeneous group of diseases characterized by both humoral and cell-mediated immunodeficiency. Cutaneous granulomas manifestations in children with combined immunodeficiency are rare. OBJECTIVE: We report the case of a 6-year-old boy who presented with disseminated cutaneous granulomas and a history of multiple infections. METHODS AND RESULTS: Laboratory evaluation revealed severe combined immunodeficiency, and deoxyribonucleic acid (DNA) analysis confirmed mutations on a gene of chromosome 19 that encodes an enzyme called Janus kinase 3 (Jak-3). Immunohistochemistry revealed expression of CD8(+) in the perivascular lymphocytic infiltrate CONCLUSION: Disseminated granulomatous lesions in children with a history of frequent infections should prompt the clinician to initiate detailed immunocompetence evaluation as they might prove to be the first manifestation of immunologic impairment.
Subject(s)
CD8-Positive T-Lymphocytes/pathology , Granuloma/complications , Severe Combined Immunodeficiency/complications , Skin Diseases/complications , Child , Granuloma/immunology , Granuloma/pathology , Humans , Janus Kinase 3/genetics , Male , Mutation , Severe Combined Immunodeficiency/genetics , Severe Combined Immunodeficiency/immunology , Skin Diseases/immunology , Skin Diseases/pathologyABSTRACT
A 13-year-old patient with typical findings of transgrediens et progrediens palmoplantar keratoderma that developed confluent and reticulated papillomatosis of Gougerot and Carteaud is presented. Although coexistence of the two disorders might be coincidental, the possibility of common pathogenetic pathways resulting in alterations of keratinization could be of investigational interest.
