A Quirky Case of Segmental Truncal Congenital Port-wine Stain

A 16-year-old male presented with a reddish discoloration over the left side of the abdomen since birth. Initially smaller, it gradually enlarged with age. There was no history of similar lesions elsewhere. Cutaneous examination revealed a diffuse erythematous macule over the left upper abdomen, confined to the midline, with a segmental distribution extending posteriorly to the left lumbar area [Figure 1a and b]. No other cutaneous lesions or gigantisms were noted. Dermoscopy showed erythema with linear vessels [Figure 1c]. Ultrasound of the abdomen and pelvis, thoracolumbar spine radiography, and head computed tomography were normal. A diagnosis of segmental congenital port-wine stain was made based on history, examination, and dermoscopy findings.Figure 1: Diffuse blotchy erythematous to dull red macule in a segmental distribution over the left upper abdomen (a) and left lumbar area (b). Dermoscopy (DL4) of the lesion reveals diffuse erythema with linear vessels (c)Port-wine stains (nevus flammeus) are congenital or acquired vascular malformations caused by progressive dilatation and ectasia of the superficial cutaneous vascular plexus without vascular proliferation. They are most commonly found on the face (90%), followed by the neck, trunk, and extremities.[1,2] Facial lesions typically involve the trigeminal nerve distribution but rarely cross the midline, affecting bilateral skin, eyes, or neck. Port-wine stains can occur with syndromes such as Klippel–Trenaunay and Sturge–Weber.[3] This case underscores the rarity of an isolated congenital segmental port-wine stain over the trunk, emphasizing the importance of recognizing and documenting such atypical presentations. Declaration of consent The authors certify that they have obtained all appropriate consent forms, duly signed by the parent(s)/guardian(s) of the patient. In the form, the parent(s)/guardian(s) has/have given his/her/their consent for the images and other clinical information of their child to be reported in the journal. The parents understand that the names and initials of their child/children will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed. Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest.

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Publication Details

Journal
Indian Journal of Paediatric Dermatology
Published
2025-04-01
DOI
https://doi.org/10.4103/ijpd.ijpd_188_24
Primary Topic
Genetic and rare skin diseases.
Type
article
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article

A Quirky Case of Segmental Truncal Congenital Port-wine Stain

Logamoorthy Ramamoorthy, Kaliaperumal Karthikeyan, Suganya Radhakrishnan, Ragul Raj Elango
Indian Journal of Paediatric Dermatology
Genetic and rare skin diseases.
article

A Quirky Case of Segmental Truncal Congenital Port-wine Stain

Logamoorthy Ramamoorthy, Kaliaperumal Karthikeyan, Suganya Radhakrishnan, Ragul Raj Elango
article en

Abstract

A 16-year-old male presented with a reddish discoloration over the left side of the abdomen since birth. Initially smaller, it gradually enlarged with age. There was no history of similar lesions elsewhere. Cutaneous examination revealed a diffuse erythematous macule over the left upper abdomen, confined to the midline, with a segmental distribution extending posteriorly to the left lumbar area [Figure 1a and b]. No other cutaneous lesions or gigantisms were noted. Dermoscopy showed erythema with linear vessels [Figure 1c]. Ultrasound of the abdomen and pelvis, thoracolumbar spine radiography, and head computed tomography were normal. A diagnosis of segmental congenital port-wine stain was made based on history, examination, and dermoscopy findings.Figure 1: Diffuse blotchy erythematous to dull red macule in a segmental distribution over the left upper abdomen (a) and left lumbar area (b). Dermoscopy (DL4) of the lesion reveals diffuse erythema with linear vessels (c)Port-wine stains (nevus flammeus) are congenital or acquired vascular malformations caused by progressive dilatation and ectasia of the superficial cutaneous vascular plexus without vascular proliferation. They are most commonly found on the face (90%), followed by the neck, trunk, and extremities.[1,2] Facial lesions typically involve the trigeminal nerve distribution but rarely cross the midline, affecting bilateral skin, eyes, or neck. Port-wine stains can occur with syndromes such as Klippel–Trenaunay and Sturge–Weber.[3] This case underscores the rarity of an isolated congenital segmental port-wine stain over the trunk, emphasizing the importance of recognizing and documenting such atypical presentations. Declaration of consent The authors certify that they have obtained all appropriate consent forms, duly signed by the parent(s)/guardian(s) of the patient. In the form, the parent(s)/guardian(s) has/have given his/her/their consent for the images and other clinical information of their child to be reported in the journal. The parents understand that the names and initials of their child/children will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed. Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest.

Indian Journal of Paediatric DermatologyVol. 26(2)
Sri Manakula Vinayagar Medical College and Hospital (IN)
Openalex Percentile: Top 83%
Genetic and rare skin diseases.
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