[Skip to Content]
[Skip to Content Landing]

Periumbilical Papules in a Middle-aged Woman

Educational Objective
Based on this clinical scenario and the accompanying image, understand how to arrive at a correct diagnosis.
1 Credit CME

A woman in her 50s presented with a 4- to 5-year medical history of mildly pruritic coalescent yellowish papules around the umbilicus (Figure 1A). She was obese and multiparous. Examination of the rest of the integument, systemic examination, and investigations including fundoscopy, electrocardiogram, and stool examination for occult blood revealed no significant abnormalities. Dermoscopic examination was performed (polarized mode, Dinolite) as shown in Figure 1B. We obtained a 4-mm punch biopsy from one of the papules for diagnostic clarification (Figure 1C).

Please finish quiz first before checking answer.

You answered correctly!

Read the answer below and download your certificate.

You answered incorrectly.

Read the discussion below and retake the quiz.

A. Periumbilical pseudoxanthoma elasticum

Dermoscopic findings (polarized mode, Dinolite) (Figure 1B) revealed curvilinear brownish pigment lines with accentuation of normal pigmentary network without follicular plugging or linear vessels. Histopathologic analysis was most helpful in reaching the diagnosis—it revealed predominantly middermal, clumped eosinophilic elastic fibers (Figure 1C). Verhoeff Van Gieson staining (Figure 2) showed the altered “ravelled wool” configuration of these elastic fibers. Von Kossa staining (Figure 2, inset) confirmed calcification. Even histopathologically, we were unable to find any evidence of transepidermal elimination. All these findings were suggestive of periumbilical pseudoxanthoma elasticum.

Periumbilical pseudoxanthoma elasticum is a rare, localized acquired variant of pseudoxanthoma elasticum1 purportedly owing to abdominal stretching as found in obese and multiparous women.2 When there is transepidermal elimination of calcified fragmented elastic fibers, it is also called perforating calcific elastosis, especially when limited to skin.3,4 On perforation, it presents with pruritic papules with a central dell superimposed on a yellowish plaque around the umbilicus. Systemic comorbidities (especially vascular diseases such as angina or hypertension) are reported in varied proportions of patients.5 Angioid streaks are noted in 22% of cases.5

Survey Complete!

Sign in to take quiz and track your certificates

Buy This Activity

JN Learning™ is the home for CME and MOC from the JAMA Network. Search by specialty or US state and earn AMA PRA Category 1 CME Credit™ from articles, audio, Clinical Challenges and more. Learn more about CME/MOC

Article Information

Corresponding Author: Tejas Vishwanath, MD, Department of Dermatology, Rajiv Gandhi Medical College, Chhatrapati Shivaji Maharaj Hospital, Kalwa, Thane, Maharahtra 400605, India (tejasvishwanath.igs@gmail.com).

Published Online: October 23, 2019. doi:10.1001/jamadermatol.2019.2963

Conflict of Interest Disclosures: None reported.

Additional Contributions: We thank the patient for granting permission to publish this information.

References
1.
Kumar  P, Sivasubramanian  A, Vadivel  S, Krishnaswamy  M.  Pseudoxanthoma elasticum with periumbilical perforation in a nullipara.  Indian J Dermatol. 2012;57(3):210-212. doi:10.4103/0019-5154.96195PubMedGoogle ScholarCrossref
2.
Mehta  B, Amladi  S, Nayak  C, Savant  S.  An unusual periumbilical plaque.  Indian J Dermatol Venereol Leprol. 2008;74(6):697-698. doi:10.4103/0378-6323.45139PubMedGoogle ScholarCrossref
3.
Lal  NR, Bandyopadhyay  D, Verma  R, Shukla  AK.  Perforating calcific elastosis: revisiting a rare entity.  Indian J Dermatol. 2018;63(2):186-188.PubMedGoogle Scholar
4.
Kocatürk  E, Kavala  M, Zindanci  I, Koç  M.  Periumbilical perforating pseudoxanthoma elasticum.  Indian J Dermatol Venereol Leprol. 2009;75(3):329. doi:10.4103/0378-6323.51246PubMedGoogle ScholarCrossref
5.
Budania  A, De  D, Saikia  UN, Kanwar  AJ, Mahajan  R, Yadav  S.  Periumbilical perforating pseudoxanthoma elasticum—an acquired perforating disorder.  Int J Dermatol. 2012;51(4):439-441. doi:10.1111/j.1365-4632.2011.05276.xPubMedGoogle ScholarCrossref
6.
Jha  AK, Zeeshan  MD, Sinha  BK, Singh  A, Agrawal  P.  Periumbilical perforating pseudoxanthoma elasticum: a rare case report.  Dermatol Pract Concept. 2018;8(2):75-77. doi:10.5826/dpc.0802a02PubMedGoogle ScholarCrossref
7.
Vishalakshi  V, Torsekar  RG.  Periumbilical pseudoxanthoma elasticum.  Indian J Dermatol Venereol Leprol. 2001;67(3):139-140.PubMedGoogle Scholar
8.
Valbuena  V, Assaad  D, Yeung  J.  Pseudoxanthoma elasticum-like papillary dermal elastolysis: a single case report.  J Cutan Med Surg. 2017;21(4):345-347. doi:10.1177/1203475417699407PubMedGoogle ScholarCrossref
9.
Montesu  MA, Onnis  G, Gunnella  S, Lissia  A, Satta  R.  Elastosis perforans serpiginosa: causes and associated disorders.  Eur J Dermatol. 2018;28(4):476-481.PubMedGoogle Scholar
10.
Wang  AR, Fonder  MA, Telang  GH, Bercovitch  L, Robinson-Bostom  L.  Late-onset focal dermal elastosis: an uncommon mimicker of pseudoxanthoma elasticum.  J Cutan Pathol. 2012;39(10):957-961. doi:10.1111/j.1600-0560.2012.01979.xPubMedGoogle ScholarCrossref
If you are not a JN Learning subscriber, you can either:
Subscribe to JN Learning for one year
Buy this activity
jn-learning_Modal_LoginSubscribe_Purchase
If you are not a JN Learning subscriber, you can either:
Subscribe to JN Learning for one year
Buy this activity
jn-learning_Modal_LoginSubscribe_Purchase
With a personal account, you can:
  • Access free activities and track your credits
  • Personalize content alerts
  • Customize your interests
  • Fully personalize your learning experience
Education Center Collection Sign In Modal Right

Name Your Search

Save Search
With a personal account, you can:
  • Track your credits
  • Personalize content alerts
  • Customize your interests
  • Fully personalize your learning experience
jn-learning_Modal_SaveSearch_NoAccess_Purchase

Lookup An Activity

or

My Saved Searches

You currently have no searches saved.

With a personal account, you can:
  • Access free activities and track your credits
  • Personalize content alerts
  • Customize your interests
  • Fully personalize your learning experience
Education Center Collection Sign In Modal Right
Topics
State Requirements