Large aberrant Mongolian spots coexisting with cutis marmorata telangiectatica congenita (phacomatosis pigmentovascularis type V or phacomatosis cesiomarmorata).
A 1-month-old boy showed cutis marmorata telangiectatica congenita (CMTC) involving a block-like pattern on his left leg and thigh and on the left side of his trunk, along with bilateral greyish-blue hyperpigmentation involving the buttocks and thighs and being in part intermingled with CMTC. This association of CMTC with Mongolian spots has been reported as a distinct type of phacomatosis pigmentovascularis (PPV), namely PPV type V. More recently, the term phacomatosis cesiomarmorata (cesius = blue) has been proposed. We believe phacomatosis cesiomarmorata is another example of twin spotting or didymosis.
Torrelo
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Department of Dermatology, Hospital del Niño Jesús, Madrid, Spain. atorrelo@aedv.es
Zambrano
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Happle
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R
D003937:Diagnosis, Differential; D006801:Humans; D007231:Infant, Newborn; D008297:Male; D049328:Mongolian Spot / Q000150:complications / Q000151:congenital / Q000175:diagnosis* / Q000473:pathology; D020752:Neurocutaneous Syndromes / Q000150:complications / Q000151:congenital / Q000175:diagnosis* / Q000473:pathology; D012878:Skin Neoplasms / Q000150:complications / Q000151:congenital / Q000175:diagnosis* / Q000473:pathology