Assuntos
Centros Médicos Acadêmicos/estatística & dados numéricos , Pesquisa Biomédica/estatística & dados numéricos , Procedimentos Cirúrgicos Dermatológicos/estatística & dados numéricos , Dermatologia/estatística & dados numéricos , Prática Privada/estatística & dados numéricos , Procedimentos Cirúrgicos Dermatológicos/educação , Dermatologistas/educação , Dermatologistas/estatística & dados numéricos , Dermatologia/educação , Bolsas de Estudo/organização & administração , Bolsas de Estudo/estatística & dados numéricos , Humanos , Publicações/estatística & dados numéricos , Sociedades Médicas/organização & administração , Sociedades Médicas/estatística & dados numéricosRESUMO
Importance: Familial benign pemphigus, or Hailey-Hailey disease (HHD), is a rare and debilitating genetic dermatosis characterized by chronic, recurrent vesicles, erosions, and maceration in flexural areas. Despite the reported therapeutic modalities, such as topical and systemic corticosteroids, systemic immunomodulators, topical and systemic retinoids, and laser, HHD can still be markedly difficult to control. Objective: To assess low-dose naltrexone hydrochloride in the treatment of recalcitrant HHD. Design, Setting, and Participants: In this case series, 3 patients with biopsy-proven recalcitrant HHD were evaluated in the outpatient dermatology clinic at the Cleveland Clinic. Each patient was treated with low-dose naltrexone hydrochloride at a dosage of 1.5 to 3.0 mg per day. No laboratory monitoring was necessary. Clinical response (healing of erosions, improvement in erythema, and alleviation of pain), adverse effects, and subjective quality of life were monitored throughout the treatment. The study dates were January 2016 to January 2017. Main Outcomes and Measures: Objective clinical response as assessed by the treating dermatologist, subjective quality of life as reported by the patient, and recorded adverse effects were monitored throughout the treatment at intervals of 2 to 3 months. Results: The 3 patients included a woman in her 40s and 2 men in their 60s. Each patient exhibited at least an 80% improvement in extent of disease, with one patient demonstrating 90% clearance. All 3 patients had substantial improvement in quality of life, with one patient reporting improvement in his depression. No adverse effects were recorded. Conclusions and Relevance: Low-dose naltrexone may represent a low-cost and low-risk alternative or adjunct in the treatment of HHD.
Assuntos
Naltrexona/uso terapêutico , Antagonistas de Entorpecentes/uso terapêutico , Pênfigo Familiar Benigno/tratamento farmacológico , Qualidade de Vida , Adulto , Biópsia , Relação Dose-Resposta a Droga , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Naltrexona/administração & dosagem , Naltrexona/efeitos adversos , Antagonistas de Entorpecentes/administração & dosagem , Antagonistas de Entorpecentes/efeitos adversos , Pênfigo Familiar Benigno/diagnóstico , Pênfigo Familiar Benigno/fisiopatologia , Resultado do TratamentoRESUMO
Adrenergic urticaria is a rare type of stress-induced physical urticaria characterized by transient outbreaks of red papules surrounded by halos of hypopigmented, vasoconstricted skin. First described in 1985, there are 10 reported cases of adrenergic urticaria in the English-language medical literature. Episodes are caused by various triggers, including emotional upset, coffee, and chocolate, during which serum catecholamines and IgE are elevated, whereas histamine and serotonin levels remain within normal limits. The precise mechanisms leading to the pathogenesis of adrenergic urticaria have yet to be elucidated. Diagnosis can be made by intradermal injection of epinephrine or norepinephrine, which reproduces the characteristic rash, or by clinical observation. Trigger avoidance and oral propranolol are currently the best known treatments for adrenergic urticaria. Nonspecific therapies, including tranquilizers and antihistamines, may also ease symptoms. This article explores the pathophysiology of adrenergic urticaria and proposes a mechanism by which propranolol treats the condition.