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BMJ Case Reports 2017; doi:10.1136/bcr-2017-221365
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Dermoscopy of pseudoxanthoma elasticum

  1. Anju Bhari2
  1. 1Department of Dermatology and Venereology, All India Institute of Medical Sciences, New Delhi, India
  2. 2Dr Rajendra Prasad Centre for Ophthalmic Sciences, All India Institute of Medical Sciences, New Delhi, India
  1. Correspondence to Dr Neetu Bhari, drntbhari{at}gmail.com
  • Accepted 20 October 2017
  • Published 9 November 2017

Description

A 22-year-old woman presented with complaints of gradually progressive yellowish asymptomatic papules coalescing to form plaques over the lateral aspects of the neck since last 3 years. There were no associated systemic complaints. No similar complaints were noted in any of the family members. Systemic examination was normal while mucocutaneous examination revealed the presence of symmetrically distributed yellowish monomorphic papules arranged in a linear and reticulate manner on both sides of the neck, axillae and periumbilical area (figure 1). These plaques were confluent at most of the places, occasionally studded with telangiectasias and had a pebble-like feel on palpation with poor elastic recoil. Polarised light dermoscopy at ×10 magnification of these plaques showed multiple irregular yellowish areas alternating with multiple linear vessels. These yellowish plaques coalesced to form parallel strands (figure 2A). The fundus examination was within normal limits except diffuse pigmentary degeneration (figure 2B). The routine urine and stool examination revealed no occult blood, and echocardiography was normal. Histological evaluation of the plaque showed multiple fragmented, clumped, short, curled, basophilic elasticfibres in the mid-dermis in a ravelled ball appearance (figure 2C,D).1 A final diagnosis of pseudoxanthoma elasticum (PXE) was made. Similar dermoscopic findings have been observed by Lacarrubba et al.2 PXE is a rare hereditary disease affecting connective tissue and causing mineralisation of elastic fibres in the skin, eyes, cardiovascular system and digestive system with an incidence of 1 in 50 000 people.3 We propose that dermoscopy could be a useful tool for a non-invasive prompt diagnosis of PXE for early detection and intervention.

Figure 1

Clinical examination showing yellow papules coalescing to form plaques over both sides of the neck, bilateral axillae and periumbilical area.

Figure 2

(A) Dermoscopic view (×10) of pseudoxanthoma elasticum showing linearly arranged yellowish strands intermingled with linear vessels. (B) Fundoscopic view showing absence of angioid streaks. (C, D) Histological photomicrograph (H&E; ×10, ×40) showing multiple short, curled, basophilic elastic fibres in the mid-dermis.

Learning points

  • Dermoscopy findings of pseudoxanthoma elasticum are characteristic.

  • Dermoscopy can substitute histopathology for early and confirmed diagnosis.

Footnotes

  • Contributors All authors have contributed substantially and sufficiently to the data collection and completion of this article. AS: data collection and compilation (patient management and dermoscopy), writing and reporting the paper. NB: conception and conduct of the report, supervising and writing the paper. AB: data collection and writing the paper (ophthalmoscopy).

  • Competing interests None declared.

  • Patient consent Obtained.

  • Provenance and peer review Not commissioned; externally peer reviewed.

References

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