Want to take quizzes and track your credits?
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.
Read the answer below and download your certificate.
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
Sign in to take quiz and track your certificates
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
Corresponding Author: Tejas Vishwanath, MD, Department of Dermatology, Rajiv Gandhi Medical College, Chhatrapati Shivaji Maharaj Hospital, Kalwa, Thane, Maharahtra 400605, India (email@example.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.
You currently have no searches saved.