Dermatology: Practical and Conceptual Observation | Dermatol Pract Concept 2018;8(2):2 75 DERMATOLOGY PRACTICAL & CONCEPTUAL www.derm101.com Introduction Pseudoxanthoma elasticum (PXE) is a rare inherited disease of connective tissue causing fragmentation and mineraliza- tion of elastic fibers that primarily affects the skin, retina, and cardiovascular system [1]. Classically it is characterized by multiple asymptomatic small (1–5 mm in size), yellowish coalescing papules that are symmetrically distributed on the neck and flexural body areas, such as the axillae, antecubital fossae, periumbilical, inguinal and popliteal areas [2]. Periumbilical perforating pseudoxanthoma elasticum (PPPXE) is considered a localized variant of inherited PXE based on the presence of angioid streaks (22% of cases) and flexural lesions [3]. Some believe it to be an acquired derma- tosis secondary to cutaneous trauma caused by multiple preg- nancies, obesity, and multiple abdominal surgeries or trauma resulting in elastic fiber degeneration in these patients [4,5]. Sapadin et al considered PPPXE a bridge between the pure acquired form and the pure inherited form [6]. We report on a case of acquired PPPXE with dermoscopic features. Case Presentation A 68-year-old multiparous woman presented with multiple yellowish, asymptomatic periumbilical lesions. The lesions had appeared two years ago and had slowly enlarged to form yellowish papules coalescing to form a plaque along with a few red-brown colored papules with central keratotic mate- rial (Figure 1). There was no history of similar disease in the family. There was no history of any abdominal surgery. On exami- nation, the patient was obese and had no evidence of any associated systemic disease. On cutaneous examination, multiple yellowish, asymptomatic periumbilical papules and Periumbilical perforating pseudoxanthoma elasticum: a rare case report Abhijeet K. Jha1, MD Zeeshan1, Binod K. Sinha, Anupama Singh, Pallavi Agrawal2 1 Department of Skin & VD, Patna Medical College and Hospital, Patna, Bihar, India 2 Department of Pathology, Patna Medical College and Hospital, Patna, Bihar, India Key words: periumbilical perforating pseudoxanthoma elasticum (PPPXE), dermoscopy Citation: 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: https://doi.org/10.5826/dpc.0802a02 Received: September 25, 2017; Accepted: October 24, 2017; Published: April 30, 2018 Copyright: ©2018 Jha et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited. Funding: None. Competing interests: The authors have no conflicts of interest to disclose. All authors have contributed significantly to this publication. Corresponding author: Abhijeet Kumar Jha, MD, Department Of Skin & V.D, Patna Medical College and Hospital, Patna, pin-800004, Bihar, India. Tel. +00919631444797. Email: drabhijeetjha@gmail.com Periumbilical perforating pseudoxanthoma elasticum (PPPXE) usually presents with well-defined peri- umbilical yellowish atrophic plaques with keratotic papules at the periphery. It is considered a variant of hereditary pseudoxanthoma elasticum or a localized acquired cutaneous dermatosis. The lesions usually occur in the periumbilical area in obese, multiparous women. Here, we report an additional case of periumbilical perforating pseudoxanthoma elasticum with its dermoscopic features. ABSTRACT 76 Observation | Dermatol Pract Concept 2018;8(2):2 gested to describe this condition [9]. On dermoscopy, a yel- lowish hue may be due to the elastolysis of elastic fibers or the presence of calcium deposits as in PXE [10,11]. There is no established treatment of PPPXE. Dietary calcium restriction plaques were present with few red-brown colored papules. Dermoscopy (polarized, 10x, Dermlite DL 4; 3Gen, San Juan Capistrano, CA) revealed yellowish brown structureless areas along with semicircular, curved/serpiginous yellowish-brown lines, with few linear vessels along with a keratotic plug with central crater (Figure 2 A, B). On histopathology the yellow- ish papule showed an increased number of elastic fibers in the upper and mid reticular dermis. The elastic fibers were fragmented and curled giving the appearance of raveled wool. A focus of calcification was also seen (Figure 3). Histological examination was consistent with the diagnosis of PPPXE. Cardiologic evaluation (normal electrocardiogram and echo- cardiogram) and ophthalmoscopic examination did not show any changes. Routine laboratory tests and serum lipid profile were all within normal range. Discussion The term PPPXE was first used in 1979 by Hicks [4]. Earlier it was described as pseudoxanthoma elasticum (PXE) with coexisting elastosis perforans serpiginosa (EPS). Lund and Gilbert were the first to establish it as a separate entity [7]. The term “localized acquired cutaneous pseudoxanthoma elasticum” was also proposed, as PPPXE was believed to be “acquired” and lacked “systemic involvement.” [8] Recently, the term “perforating calcific elastosis (PCE)” has been sug- Figure 1. Periumbilical yellowish-white plaque with reddish brown keratotic papules at the periphery. [Copyright: ©2018 Jha et al.] Figure 2. (A) Dermoscopy (polarized, 10x) showed yellowish brown structureless areas along with semicircular, curved/serpiginous yel- lowish-brown lines, with few linear vessels. (B) Dermoscopy (polar- ized, 10x) showed yellowish brown structureless areas along with semicircular, curved/serpiginous yellowish-brown lines with few linear vessels and keratotic plug with central crater. [Copyright: ©2018 Jha et al. A B Observation | Dermatol Pract Concept 2018;8(2):2 77 References 1. Neldner KH. Pseudoxanthoma elasticum. Clin Dermatol. 1988;6:1–159. 2. Ko JH, Shih YC, Huang YH, Yang CH. Pseudoxanthoma elasti- cum. Lancet. 2013;381:565. 3. Woo TY, Rasmussen JE. Disorders of transepidermal elimination. Part 1. Int J Dermatol. 1985;24:267–269. 4. Hicks J, Carpenter CL Jr, Reed RJ. Periumbilical perforating pseudoxanthoma elasticum. Arch Dermatol. 1979;115:300-303. 5. Kazakis AM, Parish WR. Periumbilical perforating pseudoxan- thoma elasticum. J Am Acad Dermatol. 1988;19:384–388. 6. Sapadin AN, Lebwohl MG, Teich SA, Phelps RG, DiCostanzo D, Cohen SR. Periumbilical pseudoxanthoma elasticum associated with chronic renal failure and angioid streaks—apparent regres- sion with hemodialysis. J Am Acad Dermatol. 1998;39:338-344. 7. Lund HZ, Gilbert CF. Perforating pseudoxanthoma elasticum: Its distinction from elastosis perforans serpiginosa. Arch Pathol Lab Med. 1976;100:544-546. 8. Neldner KH, Martinez-Hernandez A. Localized acquired cu- taneous pseudoxanthoma elasticum. J Am Acad Dermatol. 1992;26:642-644. 9. Lopes LC, Lobo L, Bajanca R. Perforating calcific elastosis. J Eur Acad Dermatol Venereol. 2003;17:206-207. 10. Oiso N, Kato M, Kawada A. Fibroelastolytic papulosis in an elderly woman with a 30-year history: overlapping between pseu- doxanthoma elasticum-like papillary dermal elastolysis and white fibrous papulosis of the neck. Eur J Dermatol. 2014;24:688-689. 11. Nasca MR, Lacarrubba F, Caltabiano R, Verzı AE, Micali G. Perforating pseudoxanthoma elasticum with secondary elasto- sis perforans serpiginosa-like changes: Dermoscopy, confocal microscopy and histopathological correlation. J Cutan Pathol. 2016;43:10. 12. Sherer DW, Singer G, Uribarri J, et al. Oral phosphate binders in the treatment of pseudoxanthoma elasticum. J Am Acad Derma- tol. 2005;53:610-615. (800 mg/day) and oral phosphate binder have been reported to have significant clinical improvement in PXE [12]. These treatment modalities may be tried in PPPXE, as the pathologi- cal process involved in PXE and PPPXE is similar. We report PPPXE with calcinosis cutis in a rare presentation along with dermoscopic findings. Figure  3. The elastic fibers are fragmented and curled giving ap- pearance of raveled wool with a focus of calcification. (H&E, 40x). [Copyright: ©2018 Jha et al.]