Clinical casesIdiopatic angioedema treated with dapsone
Abstract
The most commonly identified causes of angioedema are medications, allergens and physical agents, but most cases of angioedema are idiopathic. Treatment depends on identification of the causative agent and, especially when the mechanism is not identified, on the clinician's knowledge and experience with innovative therapeutic regimens.
Case report
A 48-year-old man presented with a 3-month history of recurrent severe episodes of angioedema affecting the lips, tongue and throat. A fiberoptic examination revealed laryngeal edema during some episodes. He did not report abdominal pain, nausea or vomiting. No precipitating factors were identified. The patient was not receiving angiotensin-converting enzyme inhibitors.
For the previous 4 years, the patient had been receiving levothyroxine for autoimmune thyroiditis. There was no history of facial palsy or hereditary angioedema.
Allergy study
Skin prick test with aeroallergens, food, latex, Anisakis and patch test to a standard series (true test) were negative. Laboratory investigations revealed normal complete blood count (CBC), erythrocyte sedimentation rate, urinalysis, blood biochemistry, serum protein electrophoresis and serum immunoglobulins. IgE concentration was 30 UI/ml. Antiperoxidase antibodies were positive (535 UI/ml). Antinuclear antibodies serum immune complexes and rheumatoid factor were negative. Complement study during acute and asymptomatic periods revealed normal values of C1 esterase inhibitor, C1q, C3, C4, functional activity of C1 inhibitor and CH50.
No pathologic findings were observed in a lip biopsy.
The patient was treated with sedating and nonsedating H1 antihistamines and corticosteroids (prednisone 30 mg/day for 3 months) with no clinical improvement and treatment with 50 mg of dapsone daily was started. Glucose 6 phosphate dehydrogenase deficiency had previously been ruled out. The patient improved and antihistamine and corticoid treatment was withdrawn 1 month after starting the dapsone regimen. No episodes of angioedema appeared during the subsequent year. No reductions in hematologic parameters or adverse events were detected.
Dapsone may be an alternative drug in extreme cases of idiopathic angioedema in patients with poor response to conventional therapy.
Resumen
Los elementos más frecuentemente implicados en la etiología del angioedema son fármacos, alergenos y agentes físicos, si bien la mayoría de los casos son de carácter idiopático. El tratamiento se basa en la identificación del agente causal y en la experiencia del clínico en el manejo de estos cuadros, siendo preciso en algunas ocasiones recurrir a regímenes terapéuticos novedosos.
Caso clínico
Paciente varón de 48 años de edad, que 3 meses antes de acudir a nuestra consulta comienza con episodios de angioedema que afectan labios, lengua y región faringo-laríngea. La laringoscopia reveló edema laringeo durante las crisis. No presentaba síntomas digestivos acompañantes. No identificamos ningún desencadenante. No recibía inhibidores del enzima de conversión de la angiotensina (IECA).
Los últimos 4 años seguía tratamiento con levotiroxina por tiroiditis autoinmune. No refería antecedentes de parálisis facial ni angioedema hereditario.
Estudio alergológico
prick test con bateria de inhalantes, alimentos, latex, anisakis: negativo. Prueba del parche estándar (true test): negativo. No se detectaron alteraciones en hemograma, velocidad de sedimentación, bioquímica con perfil hepático, electroforesis sérica e inmunoglobulinas. IgE total 30 UI/ml. Anticuerpos antiperoxidasa: positivos (535 UI/ml). Inmunocomplejos séricos, ANA, factor reumatoide: negativos. Estudio de complemento normales (C3, C4, C1 inhibidor, actividad funcional de C1, C1 q, CH50) realizado en fase asintomática y durante las crisis: dentro de los valores normales.
No se detectaron alteraciones patológicas en biopsia de labio.
Se inicia tratamiento con antihistamínicos y corticoides (prednisona 30 mg/día) durante un periodo de 3 meses persistiendo los brotes de angioedema con la misma intensidad y frecuencia, por lo que decidimos iniciar tratamiento con dapsona 50 mg/día, previamente se descartó déficit de glucosa 6 fosfato deshidrogenasa. El paciente presentó mejoría clínica, pudiendo suspender el tratamiento con corticoides y antihistamínicos al mes de iniciar el régimen terapeútico con dapsona. En los 12 meses posteriores no presentó nuevos episodios de angioedema, tampoco detectamos efectos adversos tras dicho tratamiento, ni reducción en las 3 series hematológicas.
La dapsona puede ser un fármaco alternativo en casos de angioedema idiopático que no se controlan adecuadamente con la terapia convencional.
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