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Year : 2020  |  Volume : 11  |  Issue : 1  |  Page : 91-93

Congenital triangular alopecia associated with phakomatosis pigmentovascularis type ii along with Klippel Trenaunay syndrome

1 Department of Dermatology, PGIMER Dr Ram Manohar Lohia Hospital, New Delhi, India
2 Department of Pathology, PGIMER Dr Ram Manohar Lohia Hospital, New Delhi, India

Correspondence Address:
Aastha Gupta
Department of Dermatology, PGIMER Dr Ram Manohar Lohia Hospital, Baba Kharak Singh Road, New Delhi - 110 001
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/idoj.IDOJ_112_19

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Phakomatosis pigmentovascularis (PPV) is characterized by the association of a vascular nevus with a pigmentary nevus and is divided into five subtypes. PPV type II or Happle's phakomatosis cesioflammea is the most common subtype comprising of nevus flammeus along with pigmentary nevus in the form of aberrant Mongolian spots, nevus of Ota or less frequently nevus of Ito. It is estimated that around 50% of patients with PPV have systemic involvement, most frequently involving the central nervous system and eye. Other associated features include vascular abnormalities such as Sturge-Weber syndrome, and klippel trenaunay syndrome (KTS), and cutaneous lesions such as nevus anemicus (most common), cafe'-au-lait macules, generalized vitiligo and congenital triangular alopecia (CTA). There are only four reports of PPV associated with CTA in literature, and only a single previous report with associated KTS and this association has not been reported previously from India. We describe a case of a 30-year-old male having phakomatosis pigmentovascularis type II along with klippel trenaunay syndrome and associated with congenital triangular alopecia.

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