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A 22-Year-Old Man With Painful Vesicles on His Flank

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

A 22-year-old man presented with an 8-year history of painful and slightly pruritic skin lesions on his right flank. The number of lesions and degree of pain had alternately increased and decreased over the years. Occasionally the lesions would rupture and leak clear fluid. One year prior, he was hospitalized for a “skin infection” on his right flank and treated with intravenous antibiotics. The patient, a construction demolition worker, was otherwise healthy and took no medications. He had 1 lifetime female sexual partner with whom he used barrier contraception. Physical examination revealed an asymmetric cluster of lesions on the right flank measuring 14 × 12 cm at the widest margins (Figure 1). The cluster contained verrucous papules and plaques in the center and vesicles filled with clear and milky-white fluid in the periphery. Crust was partially covering some of the lesions. The surrounding skin was erythematous without warmth or induration. The remainder of the physical examination was normal except for stretch marks on the lateral abdomen.

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Lymphangioma circumscriptum

A. Biopsy the lesions

The key to the correct diagnosis in this case is a long-standing history of painful grouped vesicular and verrucous lesions in a single anatomical location, consistent with lymphangioma circumscriptum. The infection experienced by this patient was likely a secondary cellulitis, which is common in lymphangioma circumscriptum and usually caused by Staphylococcus aureus. Definitive diagnosis of lymphangioma circumscriptum requires biopsy and histopathological analysis. The disorder often appears similar to herpes simplex, herpes simplex vegetans, and herpes zoster, which can all initially be treated with valacyclovir. Of these viral lesions, only herpes simplex vegetans (a rare form of herpes simplex virus cutaneous infection in immunocompromised patients that is often recalcitrant to first-line treatment) presents with long-standing verrucous lesions. Clinical manifestations include papular eruptions, verrucous lesions, and erosive vegetative plaques, most often in the genitocrural area. Dermatitis herpetiformis, which initially can be managed with a gluten-free diet, presents as a chronic relapsing vesiculopapular rash but typically involves extensor surfaces in a symmetric distribution. Molluscum contagiosum can be treated with topical cantharadin and may have a verrucuous appearance, especially in immunocompromised patients; however, vesicles would not be present.

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Article Information

Corresponding Author: Stephen K. Tyring, MD, PhD, Center for Clinical Studies, 451 N Texas Ave, Webster, TX 77598 (styring@ccstexas.com).

Published Online: March 4, 2019. doi:10.1001/jama.2019.0107

Conflict of Interest Disclosures: None reported.

Additional Contributions: We thank the patient for providing permission to share his information.

References
1.
Patel  GA, Schwartz  RA.  Cutaneous lymphangioma circumscriptum: frog spawn on the skin.  Int J Dermatol. 2009;48(12):1290-1295. doi:10.1111/j.1365-4632.2009.04226.xPubMedGoogle ScholarCrossref
2.
Savas  JA, Ledon  J, Franca  K, Chacon  A, Zaiac  M, Nouri  K.  Carbon dioxide laser for the treatment of microcystic lymphatic malformations (lymphangioma circumscriptum): a systematic review.  Dermatol Surg. 2013;39(8):1147-1157. doi:10.1111/dsu.12220PubMedGoogle ScholarCrossref
3.
Yang  X, Jin  Y, Chen  H,  et al.  Highly selective electrocoagulation therapy: an innovative treatment for lymphangioma circumscriptum.  Dermatol Surg. 2014;40(8):899-905.PubMedGoogle Scholar
4.
Bond  J, Basheer  MH, Gordon  D.  Lymphangioma circumscriptum: pitfalls and problems in definitive management.  Dermatol Surg. 2008;34(2):271-275. doi:10.1097/00042728-200802000-00025PubMedGoogle ScholarCrossref
5.
Koçer  U, Atakan  N, Aksoy  HM, Tiftikcioglu  YO, Aksoy  B, Astarci  M.  Late-onset superficial lymphatic malformation: report of a case and review of the literature.  Dermatol Surg. 2003;29(3):291-293.PubMedGoogle Scholar
6.
Massa  AF, Menezes  N, Baptista  A, Moreira  AI, Ferreira  EO.  Cutaneous lymphangioma circumscriptum—dermoscopic features.  An Bras Dermatol. 2015;90(2):262-264. doi:10.1590/abd1806-4841.20153652PubMedGoogle ScholarCrossref
7.
Flanagan  BP, Helwig  EB.  Cutaneous lymphangioma.  Arch Dermatol. 1977;113(1):24-30. doi:10.1001/archderm.1977.01640010026002PubMedGoogle ScholarCrossref
8.
Emer  J, Gropper  J, Gallitano  S, Levitt  J.  A case of lymphangioma circumscriptum successfully treated with electrodessication following failure of pulsed dye laser.  Dermatol Online J. 2013;19(3):2.PubMedGoogle Scholar
9.
Toshyan  V, Chawla  L, Verma  S,  et al.  Lymphangioma circumscriptum: a great mimicker.  J Obstet Gynaecol India. 2016;66(suppl 2):669-671. doi:10.1007/s13224-016-0860-8PubMedGoogle ScholarCrossref
10.
Wang  JY, Liu  LF, Mao  XH.  Treatment of lymphangioma circumscriptum with topical imiquimod 5% cream.  Dermatol Surg. 2012;38(9):1566-1569. doi:10.1111/j.1524-4725.2012.02528.xPubMedGoogle ScholarCrossref
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