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Phacomatosis pigmentovascularis type IIa--case report
Majoriê Mergen Segatto1, Eloísa Unfer Schmitt2, Laura Netto Hagemann2
1Federal University for Health Sciences, Porto AlegreRS, Brazil.
Anais Brasileiros De Dermatologia
|December 19, 2013
Summary
Phacomatosis Pigmentovascularis, a rare syndrome of capillary malformation and pigmentary nevus, was observed in a 2-year-old. This case, featuring nevus flammeus and an aberrant Mongolian spot, fits type IIa classification.
Area of Science:
- Dermatology
- Medical Genetics
- Pediatrics
Background:
- Phacomatosis Pigmentovascularis (PPV) is a rare congenital disorder.
- It is characterized by the co-occurrence of a capillary malformation (e.g., nevus flammeus) and a pigmentary nevus (e.g., Mongolian spot).
- Hasegawa's classification system categorizes PPV into different types based on the specific vascular and pigmentary components.
Observation:
- A case report details a 2-year-old patient with extensive nevus flammeus and an aberrant Mongolian spot.
- The patient exhibited no signs of systemic disease or other associated anomalies.
- The clinical presentation aligns with specific criteria outlined in Hasegawa's classification.
Findings:
- The observed combination of extensive nevus flammeus and an aberrant Mongolian spot in the absence of systemic involvement led to the classification of this case.
- This specific presentation was classified as type IIa Phacomatosis Pigmentovascularis.
- The classification adheres to the established diagnostic criteria of Hasegawa's system.
Implications:
- This case contributes to the understanding of Phacomatosis Pigmentovascularis spectrum and its classification.
- Accurate classification is crucial for predicting potential associated conditions and guiding clinical management.
- Further case reports are valuable for refining diagnostic criteria and understanding the variability of PPV.
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