罕见的念珠形发的诊断治疗

Diagnosis and Treatment of Monilethrix-a Rare Disease

念殊形发为罕见的有家庭倾向性的毛发结构异常性疾病．国内先后报告6例及一家系调查，多为单发者．本文报告的2例为同胞兄弟，其临床特点为头发稀疏而短，脱发与断发同存，拔发试验阳性，头皮有密集的毛囊角化性丘疹．光镜下，病发如串珠状．扫描电镜下，病发明显节段状结构，宽阔与缩窄段交替，缩窄段毛小皮常有穿洞、断裂、裂沟．作者还就该病的临床特征、诊断与鉴别诊断、治疗与预后进行了讨论．作者强调扫描电镜观察对念珠形发的诊断鉴别诊断具有重要价值．

Monilethrix is a rare hereditary disease of struutural abnomality of hair. Six cases and a pedigree investigation had been reported in our country. Most of them were scatteted.We reported two brother cases.The clinical features were short and sparseness of the scalp hair, with alopecia and broken hair. Pull test was positive and had dense papules of keratosis pilaris of the scalp. Light microscopy. showed beading of the affected hair shaft. By scanning electromirror (SEM), the wide and the narrow atrophic segments were seen alternating on the affected hair. The narrow segments showed the holes, the longitudinal flutes and the irregulary fissure. Clinical significance,diagnosis and differential diagnoses, treat ment and prognosis were discussed. The SEM is an important examinations in this disease.