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Year : 2022  |  Volume : 67  |  Issue : 5  |  Page : 620-621
Hypertrophied nodules over a red birth mark


1 Department of Dermatology, Command Hospital Air Force, Bengaluru, Karnataka, India
2 Department of Pediatrics, Command Hospital Air Force, Bengaluru, Karnataka, India

Date of Web Publication29-Dec-2022

Correspondence Address:
Debdeep Mitra
Department of Dermatology, Command Hospital Air Force, Bangalore - 560 007, Karnataka
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijd.ijd_32_22

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How to cite this article:
Mitra D, Bhatnagar A, Bhat SA, Mitra B. Hypertrophied nodules over a red birth mark. Indian J Dermatol 2022;67:620-1

How to cite this URL:
Mitra D, Bhatnagar A, Bhat SA, Mitra B. Hypertrophied nodules over a red birth mark. Indian J Dermatol [serial online] 2022 [cited 2023 Feb 8];67:620-1. Available from: https://www.e-ijd.org/text.asp?2022/67/5/620/366119




A male patient in his 50s presented for evaluation of multiple dark coloured nodules that appeared on his left side of the face over the last year. The lesions were insidious in onset, and in the last year, they had become more numerous and larger. Rewrite Patient reported painless bleeding from large lumps on trivial trauma for the last 1 month. He also reported redness in his left eye since last week, and examination revealed a slight increase in intraocular pressure. Dermatological examination revealed a red to dark brown coloured plaque along the ophthalmic and maxillary distribution of the left trigeminal nerve and maxillary division of the right trigeminal nerve, and on the surface of the plaque, there were multiple dark red coloured nontender firm nodules of varying sizes [Figure 1]. Skin biopsy from the flat red plaque showed thin-walled round to oval vessels with many erythrocytes in the lumen [Figure 2] and skin biopsy from the recently increased nodules showed endothelial cell clusters with mixed infiltrate and septal lobulations [Figure 3]. His magnetic resonance imaging (MRI) and angiography did not reveal any structural or vascular abnormality in the brain.
Figure 1: Multiple hypertrophied nodules and erythematous plaque on the left side of the face

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Figure 2: Histopathology of a flat plaque showing thin-walled endothelium lined cavities with red blood cells (haematoxylin-eosin, original magnification ×100)

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Figure 3: Histopathology of hypertrophied nodule showing endothelial cell clusters with mixed infiltrate and septal lobulations (haematoxylin-eosin, original magnification ×100)

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   Question Top


What is your diagnosis?

Options:

  1. Fegeler syndrome
  2. Pyogenic granuloma in port-wine stain
  3. Neurofibroma
  4. Verrucous carcinoma in port-wine stain.


Answer

Pyogenic granuloma in port-wine stain.


   Discussion Top


Unlike haemangiomas, port-wine stains persist into adulthood and do not fade with time.[1] They generally appear as flat lesions, and in the majority of cases, they develop hypertrophied nodules by the fifth decade, with an average age of onset of 37 years.[2],[3] With hypertrophy and nodularity, there is a risk of spontaneous bleeding with trivial trauma. The skin biopsy of the flat plaque showed thin-walled endothelium-lined cavities with red blood cells which are pathognomic of port-wine stain [Figure 2]. Histopathology of the rapidly increasing nodules revealed a lobular pattern of vascular proliferation with inflammation and oedema resembling granulation tissue. The epidermis was thinned out and there was acanthosis and hyperkeratosis on the sides [Figure 3]. The development of a pyogenic granuloma in the hypertrophied nodules is a rare event reported in about 10 cases in the literature.[4] Pulse dye laser therapy can lead to the development of pyogenic granulomas. The co-occurrence of these two vascular abnormalities must offer some information on the pathogenesis of these poorly understood conditions. Several studies indicate that abnormalities in embryonic vasculogenesis are involved in forming vascular malformations such as port-wine stains, and these same abnormalities may predispose patients toward forming vascular hyperplasia or tumours like pyogenic granuloma.[4],[5],[6]

Fegeler syndrome is acquired port-wine stain with the appearance in adulthood and was not considered in this case as its onset was since birth. Basal cell carcinoma[7] and squamous cell carcinomas are also reported complications in nodular lesions of port-wine stain but the histopathology was characteristic of port-wine stain with pyogenic granuloma.

Despite advances in vascular laser therapy, as most lasers cannot penetrate beyond 100 μm there remains a substantial subset of patients who due to insufficient quality and quantity of treatments, continue to have port-wine stains that become nodular and hypertrophied.

Learning points

  • Port-wine stains persist throughout life
  • Hypertrophied growths are common in the port-wine stains in the face in the third to fifth decade
  • Pyogenic granuloma is a known complication in these hypertrophied growth lesions
  • Malignant transformation is a rare complication
  • Be aware of underlying high-flow vascularity so a presumptive colour Doppler to rule out arterio-venous malformation is a must before any invasive procedure including biopsy.


Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
   References Top

1.
Minkis K, Geronemus RG, Hale EK. Port-wine stain progression: A potential consequence of delayed and inadequate treatment? Lasers Surg Med 2009;41:423-6.  Back to cited text no. 1
    
2.
Geronemus RG, Ashinoff R. The medical necessity of evaluation and treatment of port-wine stains. J Dermatol Surg Oncol 1991;17:76-9.  Back to cited text no. 2
    
3.
Fölster-Holst R, Shukla R, Kassir M, Galadari H, Lotti T, Wollina U, et al. Treatment update of port-wine stain: A narrative review. J Drugs Dermatol 2021;20:515-8.  Back to cited text no. 3
    
4.
Sheehan DJ, Lesher JL Jr. Pyogenic granuloma arising within a port-wine stain. Cutis 2004;73:175-80.  Back to cited text no. 4
    
5.
Garzon MC, Enjolras O, Frieden IJ. Vascular tumours and vascular malformations: Evidence for an association. J Am Acad Dermatol 2000;42:275-9.  Back to cited text no. 5
    
6.
Huang L, Bichsel C, Norris AL, Thorpe J, Pevsner J, Alexandrescu S, et al. Endothelial GNAQ p.R183Q increases ANGPT2 (angiopoietin-2) and drives formation of enlarged blood vessels. Arterioscler Thromb Vasc Biol 2022;42:e27-43.  Back to cited text no. 6
    
7.
Silapunt S, Goldberg LH, Thurber M, Friedman PM. Basal cell carcinoma arising in a port-wine stain. Dermatol Surg 2004;30:1241-5.  Back to cited text no. 7
    


    Figures

  [Figure 1], [Figure 2], [Figure 3]



 

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