RESUMEN
Cutis laxa is a rare disorder of elastic tissue resulting in loose, redundant, hypoelastic skin. Both acquired and inherited forms exist, some of which have significant systemic manifestations. Here, we review the various forms of cutis laxa, with focus on the inherited forms. Recent molecular studies have provided many new insights into the causes of cutis laxa and revealed greater genetic heterogeneity than previously appreciated.
Asunto(s)
Cutis Laxo/genética , Cutis Laxo/patología , Proteínas de la Matriz Extracelular/genética , Predisposición Genética a la Enfermedad/epidemiología , Anomalías Múltiples/diagnóstico , Anomalías Múltiples/epidemiología , Anomalías Múltiples/genética , Biopsia con Aguja , Cutis Laxo/epidemiología , Tejido Elástico/patología , Femenino , Humanos , Inmunohistoquímica , Incidencia , Masculino , Pronóstico , Medición de Riesgo , Índice de Severidad de la EnfermedadRESUMEN
We describe a patient who was referred for management of psoriasis unresponsive to treatment. Given the predominantly acral distribution of the patient's rash and his known diagnosis of hepatitis C, we considered the diagnosis of necrolytic acral erythema (NAE) and empirically began treatment with oral zinc sulfate. At follow-up 3 weeks later, the patient had exceptional improvement in his cutaneous disease, supporting our diagnosis of NAE. NAE is a recently described entity, and limited information is known regarding its etiology and pathogenesis; however, previous reports have been fairly consistent regarding the clinical and histopathologic presentation. We describe a patient without the classic histologic or clinical findings of previously described cases of NAE. We believe that it is important to keep the diagnosis of NAE in one's differential for a predominantly acrally distributed rash. Furthermore, we recommend additional work-up, including hepatitis C status and zinc levels, as well as possible treatment with oral zinc sulfate in patients with a potential diagnosis of NAE.
Asunto(s)
Eritema/complicaciones , Eritema/patología , Folículo Piloso/patología , Hepatitis C/complicaciones , Astringentes/administración & dosificación , Biopsia , Eritema/tratamiento farmacológico , Pie , Mano , Humanos , Masculino , Persona de Mediana Edad , Necrosis , Sulfato de Zinc/administración & dosificaciónRESUMEN
OBJECTIVE: To evaluate the efficacy of efalizumab in the treatment of oral erosive lichen planus. DESIGN: A single-center, open-label, prospective pilot study. The primary efficacy outcome measure was the change in oral mucosal surface area involvement after 12 weeks of treatment. Secondary outcome measures included the 100-mm visual analog scale (VAS) for pain and a modified Oral Health Impact Profile (OHIP-14) questionnaire. RESULTS: Four adult patients with oral erosive lichen planus were enrolled and treated with efalizumab 0.7 mg/kg subcutaneously at week 0 followed by 1.0 mg/kg weekly from week 1 to week 11. The mean reduction in the affected mucosal surface area was 71.1% (range 57.3% to 96.8%). The mean improvement in the 100-mm VAS for pain was 82%. The mean improvement in the OHIP-14 questionnaire was 69.3%. Significant adverse events included hospitalization for urticaria and a staphylococcal abscess of an artificial hip joint in one patient and drug-induced subacute cutaneous lupus in another patient.