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Neurocutaneous Disorders Phakomatoses and Hamartoneoplastic Syndromes pp 449–454Cite as

Phakomatosis Pigmentovascularis

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Abstract

Phakomatosis pigmentovascularis (PPV) is defined as the coexistence of a widespread vascular (usually capillary) nevus (nevus flammeus) and an extensive pigmentary nevus (usually of the Mongolian spot type or blue/slate/grey oculo-cutaneous melanocytosis) associated to a variety of other cutaneous nevus (e.g., anaemicus, epidermal nevus, telangiectatic nevus, nevus spilus or cutis marmorata telangiectatica) and/or extracutaneous alterations (Happle 2005, Ruiz-Maldonado et al. 1987, Vidaurri-de la Cruz et al. 2003). All types of PPV so far described have been best explained as examples of twin spotting (Happle 1999, 2005).

Keywords

  • Moyamoya Disease
  • Weber Syndrome
  • Epidermal Nevus
  • Capillary Malformation
  • Mongolian Spot

These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

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References

  • Al Robaee A, Banka N, Alfadley A (2004) Phakomatosis pigmentovascularis type IIb associated with Sturge-Weber syndrome. Pediatr Dermatol 21: 642–645.

    PubMed  CrossRef  Google Scholar 

  • Bleehen SS (1998) Disorders of skin colour. In: Champion RH, Burton JL, Burns DA, Breathnach SM (eds.) Rook/Wilkinson/Ebling Textbook of Dermatology. 6th ed. Oxford: Blackwell Science, pp. 1791–1792.

    Google Scholar 

  • Castorf M, Rinaldi R, Angelo C, Zambruno G, Grammatico P, Happle R (2008) Phacomatosis cesioflammea with unilateral lipohypoplasia. Am J Med Genet A 146: 492–495.

    Google Scholar 

  • Cho S, Choi JH, Sung KJ, Moon KC, Koh JK (2001) Phakomatosis pigmentovascularis type IIb with neurologic abnormalities. Pediatr Dermatol 18: 263.

    PubMed  CAS  Google Scholar 

  • Diociaiuti A, Guidi B, Aguilar Sanchez JA, Feliciani C, Capizzi R, Amerio P (2005) Phakomatosis pigmentovascularis type IIb: a case associated with Sturge-Weber and klippel-Trenaunay syndrome. J Am Acad matol 8: 569–572.

    Google Scholar 

  • Du LC, Delaporte E, Catteau B, Destee A, Piette F (1998) Phakomatosis pigmentovascularis Type II. Eur J Dermatol 8: 569–572.

    PubMed  CAS  Google Scholar 

  • Enjolras O, Mulliken JB (2000) Vascular malformations. In: Harper J, Oranje A, Prose N (eds.) Textbook of Dermatology. Oxford: Blackwell Science, pp. 975–976.

    Google Scholar 

  • Fernandez-Guarino M, Boixeda P, de Las Heras E, Aboin S, García-Millán C, Olasolo PJ (2008) Phakomatosis pigmentovascularis: clinical findings in 15 patients and review of the literature. J Am Acad Dermatol 58: 88–93.

    PubMed  CrossRef  Google Scholar 

  • Gomez MR (1987) Neurocutaneous diseases. A Practical Approach. Boston: Butterworths.

    Google Scholar 

  • Guiglia MC, Prendeville JS (1991) Multiple granular-cell tumors associated with giant speckled lentiginous nevus and nevus flammeus in a child. J Am Acad Dermatol 24: 359–363.

    PubMed  CrossRef  CAS  Google Scholar 

  • Hagiwara K, Uezato H, Nonaka S (1998) Phacomatosis pigmentovascularis type IIb associated with Sturge-Weber syndrome and pyogenic granulom. J Dermatol 25: 721–729.

    PubMed  CAS  Google Scholar 

  • Hall BD, Cadle RG, Morrill-Cornelius SM, Bay CA (2007) Phakomatosis pigmentovascularis: implications for severity with special reference to Mongolian spots associated with Sturge-Weber and Klippel-Trenaunay syndromes. Am J Med Genet A 143: 3047–3053.

    Google Scholar 

  • Happle R (1999) Loss of heterozygosity in human skin. J Am Acad Dermatol 14: 143–161.

    CrossRef  Google Scholar 

  • Happle R (2005) Phacomatosis pigmentovascularis revisited and reclassified. Arch Dermatol 141: 385–388.

    PubMed  CrossRef  Google Scholar 

  • Happle R, Steijlen PM (1989) Phacomatosis pigmentovascularis gedeuted als ein Phänomen der Zwillingsflecken. Hautarzt 40: 721–724.

    PubMed  CAS  Google Scholar 

  • Hasegawa Y, Yasuhara M (1985) Phakomatosis pigmentovascularis type Iva. Arch Dermatol 48: 256–258.

    Google Scholar 

  • Karabudak O, Dogan B, Basekim C, Harmanyeri Y (2007) Phacomatosis spilorosea (phacomatosis pigmentovascularis type IIIb). Australas J Dermatol 48: 256–258.

    PubMed  CrossRef  Google Scholar 

  • Kikuchi I, Okasaki M (1982) Congenital temporal alopecia in Phakomatosis pigmentovascularis. J Dermatol 9: 485–487.

    PubMed  CAS  Google Scholar 

  • Kim YC, Park HJ, Cinn YW (2002) Phakomatosis pigmento vascularis type IIa with generalized vitiligo. Br J Dermatol 147: 1028–1029.

    PubMed  CrossRef  Google Scholar 

  • Kono T, Ercöcen AR, Chan HH, Kikuchi Y, Hori H, Uezono S, Nozaki M (2003) Treatment of phakomatosis pigmento-vascularis: A combined multiple laser approach. Dermatol Surg 29: 642–646.

    PubMed  CrossRef  Google Scholar 

  • Lee CW, Choi DY, Oh YG, Yoon HS, Kim JD (2005) An infantile case of Sturge-Weber syndrome in association with Klippel-Trenaunay-Weber syndrome and phakomatosis pigmentovascularis. J Korean Med Sci 20: 1082–1084.

    PubMed  CrossRef  Google Scholar 

  • Onsun N, Inandirici A, Kural Y, Teker C, Atilganoglu U (2007) Phakomatosis pigmentovascularis type II b with bilateral hearing impairment. J Eur Acad Dermatol Venereol 21: 402.

    PubMed  CrossRef  CAS  Google Scholar 

  • Ota S, Lopez A, Ito N (1987) Phakomatosis pigmentov vascularis. Jpn J Dermatol 57: 1–3.

    Google Scholar 

  • Ruiz-Maldonado R, Tamayo L, Laterza AM, Brawn G, Lopez T (1987) Phakomatosis pigmentovascularis: A new syndrome? Report of four cases. Pediatr Dermatol 4: 189–196.

    PubMed  CrossRef  CAS  Google Scholar 

  • Saricaoglu MS, Guven D, Karakurt A, Sengun A, Ziraman I (2002) An unusual case of Sturge-Webers syndrome in association with phakomatosis pigmentovascularis and klippel-Trenaunay-Weber syndrome. Retina 22: 368–371.

    PubMed  CrossRef  Google Scholar 

  • Tsuruta D, Fukai K, Seto M, Fujitani K, Shindo K, Hamada T, Ishii M (1991) Phakomatosis pigmentovascularis (TypeIIIa) associated with Moyamoya disease. Pediatr Dermatol 16: 35–38.

    CrossRef  Google Scholar 

  • Torrelo A, Zambrano A, Happle R (2006) Large aberrant Mongolian spots coexisting with cutis marmorata telangiectatica congenita (phacomatosis pigmentovascularis type V or phacomatosis cesiomarmorata). J Eur Acad Dermatol Venereol 20: 308–310.

    PubMed  CrossRef  CAS  Google Scholar 

  • Uysal G, Guven A, Ozhan B, Ozturk MH, Mutluay AH, Tulunay O (2005) Phacomatosis pigmentovascularis with Sturge-Weber syndrome. J Dermatol 27: 467–470.

    Google Scholar 

  • Vidaurri-de la Cruz H, Tamayo-Sanchez 1, Duran-McKinster C, Orozco-Covarrubias Mde L, Ruiz-Maldonado R (2003) Phakomatosis Pigmentovascularis II and II B: Clinical findings in 24 patients. J Dermatol 30: 381–388.

    PubMed  Google Scholar 

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Authors and Affiliations

  1. Department of Dermatology, National Institute of Paediatrics, Insurgente Sur 3700-C, Mexico City, 04530, Mexico

    Ramón Ruiz-Maldonado MD, Carola Duràn-McKinster MD, Luz Orozco-Covarrubias MD & Marimar Saez-De-Ocariz MD

Authors
  1. Ramón Ruiz-Maldonado MD
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  2. Carola Duràn-McKinster MD
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  3. Luz Orozco-Covarrubias MD
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  4. Marimar Saez-De-Ocariz MD
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Editor information

Editors and Affiliations

  1. Institute of Neurological Science, National Research Council, Catania, Italy

    Martino Ruggieri

  2. University Hospital La Paz, Madrid, Spain

    Ignacio Pascual-Castroviejo

  3. Institute of Neurosurgery, Catholic University of the Sacred Heart, Rome, Italy

    Concezio Di Rocco

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Ruiz-Maldonado, R., Duràn-McKinster, C., Orozco-Covarrubias, L., Saez-De-Ocariz, M. (2008). Phakomatosis Pigmentovascularis. In: Ruggieri, M., Pascual-Castroviejo, I., Di Rocco, C. (eds) Neurocutaneous Disorders Phakomatoses and Hamartoneoplastic Syndromes. Springer, Vienna. https://doi.org/10.1007/978-3-211-69500-5_23

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  • DOI: https://doi.org/10.1007/978-3-211-69500-5_23

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