Hemophagocytic lymphohistiocytosis in a neonate with cutis marmorata telangiectatica congenita.
نویسندگان
چکیده
In this case report we describe the first case of hemophagocytic lymphohistiocytosis with concurrent cutis marmorata telangiectatica congenita. She had pancytopenia and hepatosplenomegaly, hemophagocytic cells in spleen necropsy, and she died with respiratory failure and pseudomonas induced septicemia.
منابع مشابه
Cutis marmorata telangiectatica congenita: a case report.
Cutis marmorata telangiectatica congenita (CMTC) is a rare cutaneous vascular disorder that was first described in 1922 by Van Lohuizen (1). The clinical presentation is one of persistent cutis marmorata, phlebectasia, telangiectasia, and areas of ulcerations. The disorder shows slow clinical improvement over time. Previously, CMTC has also been described as congenital generalized phlebectasia ...
متن کاملCongenital cutis marmorata telangiectatica and syndactyly in a preterm: case report.
Cutis marmorata telangiectatica congenita (CMTC) is a rare, commonly benign, congenital, localized or generalized vascular anomaly of unknown aetiology. It is characterized by persistent cutis marmorata, telangiectasia and phlebectasia. Extracutaneous findings may be associated with CMTC in 18.8-70% of the cases. Diagnosis of the disorder is based on the clinical findings. The prognosis is good...
متن کاملCutis marmorata telangiectatica congenita.
Babies commonly present with skin anomalies at or shortly after birth. While physiological cutis marmorata is a frequently encountered condition, we report a rare entity that appears similar, but should not be confused with it. Cutis marmorata telangiectatica congenita (CMTC) is a rare, sporadic condition that presents at birth as a localised or generalised reticulated, blue-violet vascular net...
متن کاملCutis marmorata telangiectatica congenita and aberrant Mongolian spots: Type V phacomatosis pigmentovascularis or phacomatosis cesiomarmorata
CMTC: Cutis marmorata telangiectatica congenita PPV: Phacomatosis pigmentovascularis INTRODUCTION The coexistence of cutis marmorata telangiectatica congenita (CMTC) with Mongolian spots has been reported as a distinct type of phacomatosis pigmentovascularis (PPV), namely PPV type V or phacomatosis cesiomarmorata. PPV type V is a rare congenital vascular anomaly, with only 7 previous cases ment...
متن کاملFluorescein angiogram findings in a case of cutis marmorata telangiectatica congenita.
Cutis marmorata telangiectatica congenita is a well-characterized cutaneous vascular disorder with variable and rare ocular involvement. It has been reported in association with glaucoma, bilateral congenital retinal detachments, bilateral tractional retinal detachments secondary to proliferative vitreoretinopathy, and retinoblastoma. This case demonstrates novel findings of bilateral periphera...
متن کاملذخیره در منابع من
با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید
عنوان ژورنال:
- Saudi medical journal
دوره 27 11 شماره
صفحات -
تاریخ انتشار 2006