A Case of Variant Turner Syndrome with Multiple Café-au-lait Spots / 대한피부과학회지

The phenotypic variability of variant Turner syndrome is wide, ranging from characteristic clinical features to those that are hardly distinguishable from the general population. A 4-year-old girl presented with multiple brownish macules and patches on the trunk and upper extremities as well as axillary freckles. Exome sequencing and chromosomal microarray testing revealed a microdeletion at Xp22.33p22.11 leading to a diagnosis of Turner syndrome. Here we describe an unusual case of variant Turner syndrome with multiple café-au-lait spots.

Multiple Cutaneous Rosai-Dorfman Disease on the Face Treated by Electrosurgery / 대한피부과학회지

Cutaneous Rosai-Dorfman disease (RDD) is a rare, benign condition mainly affecting the lymph nodes and sometimes involving the skin. RDD is notorious for its poor response to medication, and surgery is often the preferred treatment option for limited lesions. Herein, we present a case of cutaneous RDD in a 49-year-old woman with multiple facial nodules that showed minimal response to treatment with steroids, isotretinoin, and methotrexate.The diagnosis of cutaneous RDD was confirmed based on histopathological examination. The patient showed minimal response to medication; however, electrosurgery resulted in a relatively good cosmetic response. The present case demonstrates that electrosurgery can be an effective treatment option for cutaneous RDD, especially in case of patients with multiple lesions. The rarity of this disease and the limited reports on its management highlights the need for further research on the efficacy of different surgical modalities for cutaneous RDD.

Venous Insufficiency is a Clear Provoker of Pigmented Purpuric Dermatosis

Background@#Pigmented purpuric dermatosis (PPD) is a chronic disorder characterized by distinct petechial hemorrhage and brownish pigmentation. The cause of PPD is unclear, but several underlying conditions are associated with it. Previous reports suggest that venous insufficiency (VI) might be related to PPD; however, a clear correlation remains unelucidated. @*Objective@#To elucidate the causal relationship between PPD and VI. @*Methods@#A total 118 patients diagnosed with PPD in the Department of Dermatology, Pusan National University Hospital from November 2006 to July 2019 were retrospectively reviewed. Doppler ultrasonography of the lower extremities was performed in 56 PPD patients, who were then divided into two groups: PPD with and without VI. We compared the clinical features between the two groups. In the PPD with VI group, we assessed the correspondence ratios between PPD and VI lateralities, and between the PPD distribution and the veins involved. @*Results@#VI was detected in 35 of the 56 patients (62.5%). The PPD with VI group was significantly associated with wider distribution, darker coloration and longer disease duration. There was a positive correlation of laterality between PPD and VI, and between PPD distribution and the vein involved. @*Conclusion@#This findings suggest that VI is a clear provoker of PPD.