Congenital coexistence of vascular and pigmentary lesions: a case of phakomatosis pigmentovascularis type IV with literature review

Rawan Alanazi; Loujain Alyousef; Sharifah Almasoud; Kadi Alsubaie; Nora Alanezi; Ahmad Almousa

doi:10.24911/IJMDC.51-1767637154

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Case Report
Online Published: 22 Jan 2026

IJMDC. 2026; 10(2): 865-868

doi: 10.24911/IJMDC.51-1767637154

Congenital coexistence of vascular and pigmentary lesions: a case of phakomatosis pigmentovascularis type IV with literature review

Rawan Alanazi, Loujain Alyousef, Sharifah Almasoud, Kadi Alsubaie, Nora Alanezi, Ahmad Almousa.

Abstract
Background: Phakomatosis pigmentovascularis (PPV) is a very unusual congenital neurocutaneous condition that involves both vascular deformities and pigmentary lesions. Despite being described in various subtypes, PPV is underdiagnosed in a clinical context and can include organ systems to varying degrees.
Case Presentation: A case of a 3-month-old male infant, who was presented with congenital depigmented macules on the distal portions of his feet and the palmar side of one representative hand. His right thigh had a capillary malformation that appeared as telangiectatic blotches. The paraffin section of the lesion showed the presence of a normal amount of melanin, with no evidence of absence. Ophthalmological and neurological evaluations did not demonstrate any significant systemic anomalies. Taking into account the combination of nevus depigmentosus, capillary malformation, and localized telangiectasia, the final diagnosis was PPV type I V.
Conclusion: PPV is an uncommon variant and might not be associated with any systemic findings. Although the case in this report demonstrated only skin lesions at this time with a good prognosis, there are many different patterns of natural history associated with PPV; thus, it is critical to have ongoing multidisciplinary follow-up to monitor the potential development of ophthalmologic and neurologic complications.

Key words: Phakomatosis pigmentovascularis, dermal melanocytosis, capillary malformation, pediatric dermatology, nevus depigmentosus, case report

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Pubmed Style

Alanazi R, Alyousef L, Almasoud S, Alsubaie K, Alanezi N, Almousa A. Congenital coexistence of vascular and pigmentary lesions: a case of phakomatosis pigmentovascularis type IV with literature review. IJMDC. 2026; 10(2): 865-868. doi:10.24911/IJMDC.51-1767637154

Web Style

Alanazi R, Alyousef L, Almasoud S, Alsubaie K, Alanezi N, Almousa A. Congenital coexistence of vascular and pigmentary lesions: a case of phakomatosis pigmentovascularis type IV with literature review. https://www.ijmdc.com/?mno=305728 [Access: June 27, 2026]. doi:10.24911/IJMDC.51-1767637154

AMA (American Medical Association) Style

Alanazi R, Alyousef L, Almasoud S, Alsubaie K, Alanezi N, Almousa A. Congenital coexistence of vascular and pigmentary lesions: a case of phakomatosis pigmentovascularis type IV with literature review. IJMDC. 2026; 10(2): 865-868. doi:10.24911/IJMDC.51-1767637154

Vancouver/ICMJE Style

Alanazi R, Alyousef L, Almasoud S, Alsubaie K, Alanezi N, Almousa A. Congenital coexistence of vascular and pigmentary lesions: a case of phakomatosis pigmentovascularis type IV with literature review. IJMDC. (2026), [cited June 27, 2026]; 10(2): 865-868. doi:10.24911/IJMDC.51-1767637154

Harvard Style

Alanazi, R., Alyousef, . L., Almasoud, . S., Alsubaie, . K., Alanezi, . N. & Almousa, . A. (2026) Congenital coexistence of vascular and pigmentary lesions: a case of phakomatosis pigmentovascularis type IV with literature review. IJMDC, 10 (2), 865-868. doi:10.24911/IJMDC.51-1767637154

Turabian Style

Alanazi, Rawan, Loujain Alyousef, Sharifah Almasoud, Kadi Alsubaie, Nora Alanezi, and Ahmad Almousa. 2026. Congenital coexistence of vascular and pigmentary lesions: a case of phakomatosis pigmentovascularis type IV with literature review. International Journal of Medicine in Developing Countries, 10 (2), 865-868. doi:10.24911/IJMDC.51-1767637154

Chicago Style

Alanazi, Rawan, Loujain Alyousef, Sharifah Almasoud, Kadi Alsubaie, Nora Alanezi, and Ahmad Almousa. "Congenital coexistence of vascular and pigmentary lesions: a case of phakomatosis pigmentovascularis type IV with literature review." International Journal of Medicine in Developing Countries 10 (2026), 865-868. doi:10.24911/IJMDC.51-1767637154

MLA (The Modern Language Association) Style

APA (American Psychological Association) Style

Alanazi, R., Alyousef, . L., Almasoud, . S., Alsubaie, . K., Alanezi, . N. & Almousa, . A. (2026) Congenital coexistence of vascular and pigmentary lesions: a case of phakomatosis pigmentovascularis type IV with literature review. International Journal of Medicine in Developing Countries, 10 (2), 865-868. doi:10.24911/IJMDC.51-1767637154

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