Assuntos
Displasia Ectodérmica/diagnóstico , Alopecia/complicações , Alopecia/diagnóstico por imagem , Criança , Displasia Ectodérmica/diagnóstico por imagem , Feminino , Doenças Genéticas Ligadas ao Cromossomo X/complicações , Doenças Genéticas Ligadas ao Cromossomo X/diagnóstico por imagem , Humanos , Couro Cabeludo/anormalidades , Couro Cabeludo/fisiopatologiaAssuntos
Linfoma Folicular/terapia , Recidiva Local de Neoplasia/tratamento farmacológico , Fotoquimioterapia/métodos , Neoplasias Cutâneas/terapia , Administração Cutânea , Ácido Aminolevulínico/administração & dosagem , Ácido Aminolevulínico/análogos & derivados , Dorso , Humanos , Linfoma Folicular/diagnóstico , Linfoma Folicular/patologia , Masculino , Pessoa de Meia-Idade , Recidiva Local de Neoplasia/diagnóstico , Radioterapia , Pele/efeitos dos fármacos , Pele/patologia , Pele/efeitos da radiação , Neoplasias Cutâneas/diagnóstico , Neoplasias Cutâneas/patologia , Resultado do TratamentoRESUMO
Hailey-Hailey disease is a rare blistering dermatosis first described in 1939 by the brothers Howard and Hugh Hailey. Its incidence is estimated at 1/50,000. The inheritance is autosomal dominant with complete penetrance, but a variable expressivity in affected family members. Clinically, Hailey-Hailey disease presents between the third and fourth decade as flaccid vesicles and blisters on erythematous skin, giving rise to erosions, fissures, and vegetations. Maceration and superinfections are frequent. The lesions are typically distributed symmetrically within intertriginous regions such as the retroauricular folds, lateral aspects of the neck, axillae, umbilicus, inguinal, and perianal regions. The disease is characterized by a chronic relapsing course with spontaneous remissions and multiple recurrences. Severe disease can be very frustrating and have a major psychological and social impact. Given the dearth of evidence-based guidelines and large clinical trials, the assessment of the efficacy and safety of treatments is difficult. Treatments include topical and systemic agents, and procedural therapy such as lasers and surgery. This review provides a systematic search of the literature with a focus on classical and emerging treatment options for Hailey-Hailey disease.