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Phakomatosis pigmentovascularis presenting with sturge-weber syndrome and klippel-trenaunay syndrome.

Sen S, Bala S, Halder C, Ahar R, Gangopadhyay A - Indian J Dermatol (2015 Jan-Feb)

Bottom Line: Phakomatosis pigmentovascularis (PPV) is a rare cutaneous disorder characterized by combination of capillary malformation and other pigmented naevi.Four types and two subtypes have been described where subtype 'a' present only with cutaneous form and subtype 'b' also with systemic association like in Sturge-Weber syndrome or Klippel-Trenaunay syndrome.Hereby, we report a case where our patient presented with port-wine stain, Nevus of Ota, Sturge-Weber syndrome, and Klippel-Trenaunay syndrome; which has made it a rare combination.

View Article: PubMed Central - PubMed

Affiliation: Department of Dermatology Venereology and Leprosy, Institute of Post-Graduate Medical Education and Research and Seth Sukhlal Karnani Memorial Hospital, Kolkata, West Bengal, India.

ABSTRACT
Phakomatosis pigmentovascularis (PPV) is a rare cutaneous disorder characterized by combination of capillary malformation and other pigmented naevi. Four types and two subtypes have been described where subtype 'a' present only with cutaneous form and subtype 'b' also with systemic association like in Sturge-Weber syndrome or Klippel-Trenaunay syndrome. Hereby, we report a case where our patient presented with port-wine stain, Nevus of Ota, Sturge-Weber syndrome, and Klippel-Trenaunay syndrome; which has made it a rare combination.

No MeSH data available.


Related in: MedlinePlus

Engorged vein over lateral aspect of left leg along with hypertrophy of lower limb
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Figure 2: Engorged vein over lateral aspect of left leg along with hypertrophy of lower limb

Mentions: A 13-year-old female presented with port-wine stain of left side of the body including face along the distribution of 5th cranial nerve, upper limb, trunk, and lower limb [Figure 1]. Additionally, this lesion was present on right side of the back as well. There was associated hypertrophy of the left lower limb with a visible vessel along the lateral aspect of the leg [Figure 2]. The left sclera showed blue discoloration [Figure 3]. There was no history of seizures, but she had subnormal intelligence. Ophthalmological examination showed raised intraocular tension in left eye but the right eye was normal. X-ray of upper and lower limb showed no bony hypertrophy. The color Doppler study of left lower limb showed absence of great and short saphenous vein. An abnormal dilated vein on lateral aspect of leg and thigh was present which was seen to drain into superficial circumflex iliac vein [Figure 4]. Superficial femoral vein was hypoplastic and popliteal vein was absent. Non-contrast computed tomography (CT) scan of brain showed focal parenchymal atrophy in the left parietal lobe region with foci of gyral calcification. Calvarial thickening predominantly in frontal and occipital region was seen. Frontal and sphenoid sinuses were found to be dilated.


Phakomatosis pigmentovascularis presenting with sturge-weber syndrome and klippel-trenaunay syndrome.

Sen S, Bala S, Halder C, Ahar R, Gangopadhyay A - Indian J Dermatol (2015 Jan-Feb)

Engorged vein over lateral aspect of left leg along with hypertrophy of lower limb
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4318068&req=5

Figure 2: Engorged vein over lateral aspect of left leg along with hypertrophy of lower limb
Mentions: A 13-year-old female presented with port-wine stain of left side of the body including face along the distribution of 5th cranial nerve, upper limb, trunk, and lower limb [Figure 1]. Additionally, this lesion was present on right side of the back as well. There was associated hypertrophy of the left lower limb with a visible vessel along the lateral aspect of the leg [Figure 2]. The left sclera showed blue discoloration [Figure 3]. There was no history of seizures, but she had subnormal intelligence. Ophthalmological examination showed raised intraocular tension in left eye but the right eye was normal. X-ray of upper and lower limb showed no bony hypertrophy. The color Doppler study of left lower limb showed absence of great and short saphenous vein. An abnormal dilated vein on lateral aspect of leg and thigh was present which was seen to drain into superficial circumflex iliac vein [Figure 4]. Superficial femoral vein was hypoplastic and popliteal vein was absent. Non-contrast computed tomography (CT) scan of brain showed focal parenchymal atrophy in the left parietal lobe region with foci of gyral calcification. Calvarial thickening predominantly in frontal and occipital region was seen. Frontal and sphenoid sinuses were found to be dilated.

Bottom Line: Phakomatosis pigmentovascularis (PPV) is a rare cutaneous disorder characterized by combination of capillary malformation and other pigmented naevi.Four types and two subtypes have been described where subtype 'a' present only with cutaneous form and subtype 'b' also with systemic association like in Sturge-Weber syndrome or Klippel-Trenaunay syndrome.Hereby, we report a case where our patient presented with port-wine stain, Nevus of Ota, Sturge-Weber syndrome, and Klippel-Trenaunay syndrome; which has made it a rare combination.

View Article: PubMed Central - PubMed

Affiliation: Department of Dermatology Venereology and Leprosy, Institute of Post-Graduate Medical Education and Research and Seth Sukhlal Karnani Memorial Hospital, Kolkata, West Bengal, India.

ABSTRACT
Phakomatosis pigmentovascularis (PPV) is a rare cutaneous disorder characterized by combination of capillary malformation and other pigmented naevi. Four types and two subtypes have been described where subtype 'a' present only with cutaneous form and subtype 'b' also with systemic association like in Sturge-Weber syndrome or Klippel-Trenaunay syndrome. Hereby, we report a case where our patient presented with port-wine stain, Nevus of Ota, Sturge-Weber syndrome, and Klippel-Trenaunay syndrome; which has made it a rare combination.

No MeSH data available.


Related in: MedlinePlus