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Elephantiasis nostras verrucosa, a rare manifestation of Kaposi’s sarcoma, is a progressive cutaneous hypertrophy caused by chronic non-filarial lymphedema secondary to obstruction of the lymphatic system that can lead to severe disfigurement of parts of the body that have gravity-dependent blood flow, due to edema, fibrosis, and hyperkeratosis, especially lower extremities. Among the various conditions that can induce chronic lymphedema are tumors, trauma, radiotherapy, obesity, hypothyroidism, chronic venous stasis, and AIDS-related Kaposi’s sarcoma. Kaposi’s sarcoma is a vascular tumor associated with the presence of human gammaherpesvirus 8 that is predominantly cutaneous, locally aggressive, with metastasis, and is associated with the production of factors that favor inflammation, lymphatic obstruction, and lymphedema.

Authors: Javier Arellano[1,2], Ruben Gonzalez[1], Yamile Corredoira [3], Roxana Nuñez [2]

Affiliation:
[1] Dermatology Service, San Borja Arriarán Clinical Hospital, Santiago, Chile
[2] Department of Dermatology, Faculty of Medicine, University of Chile, Santiago, Chile
[3] Pathology Department, San Borja Arriarán Clinical Hospital, Santiago, Chile
Author address:
[1] Avenida Manuel Montt 111
Departamento 905
Providencia
Región Metropolitana
Chile

Citation: Arellano J, Gonzalez R, Corredoira Y, Nuñez R. Diagnosis of elephantiasis nostras verrucosa as a clinical manifestation of Kaposi's sarcoma. Medwave 2020;20(1):e7767 doi: 10.5867/medwave.2020.01.7767

Submission date: 18/10/2019

Acceptance date: 23/12/2019

Publication date: 20/1/2020

Origin: Not commissioned.

Type of review: Externally peer-reviewed by four reviewers, double-blind.

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1. Etemad SA, Dewan AK. Kaposi Sarcoma Updates. Dermatol Clin. 2019 Oct;37(4):505-517 | CrossRef | PubMed |
2. Dietrich CF, Chaubal N, Hoerauf A, Kling K, Piontek MS, Steffgen L, Mand S, Dong Y. Review of Dancing Parasites in Lymphatic Filariasis. Ultrasound Int Open. 2019 Mar;5(2):E65-E74. | CrossRef | PubMed |
3. Namazi N, Ershadi S, Rahbar L, Ghassemipur M. Classic Kaposi sarcoma presenting as elephantiasis nostras verrucosa. Iran J Dermatol. 2015;18(1):25-8. [on line] | Link |
4. Liaw FY, Huang CF, Wu YC, Wu BY. Elephantiasis nostras verrucosa: swelling with verrucose appearance of lower limbs. Can Fam Physician. 2012;58(10):e551-3. [on line] | Link |
5. Sathyakumar S, Suh JS, Sharp VL, Polsky B. Images in HIV/AIDS. Elephantiasis nostras verrucosa secondary to Kaposi sarcoma: a rare case. AIDS Read. 2008;18(2):81-2. | PubMed |
6. Hengge UR, Stocks K, Goos M. Acquired immune deficiency syndrome-related hyperkeratotic Kaposi's sarcoma with severe lymphoedema: report of five cases. Br J Dermatol. 2000 Mar;142(3):501-5. | CrossRef | PubMed |
7. Radu O, Pantanowitz L. Kaposi sarcoma. Arch Pathol Lab Med. 2013 Feb;137(2):289-94. | CrossRef | PubMed |
8. Bishnoi A, Vinay K, Vishwajeet V, Saikia UN, Panigrahi I, Kumaran MS. Warty Fingers and Toes in a Child With Congenital Lymphedema: Elephantiasis Nostras Verrucosa. JAMA Dermatol. 2018 Jul 1;154(7):849-850. | CrossRef | PubMed |
9. Sisto K, Khachemoune A. Elephantiasis nostras verrucosa: a review. Am J Clin Dermatol. 2008;9(3):141-6. | CrossRef | PubMed |
10. Zouboulis CC, Biczó S, Gollnick H, Reupke HJ, Rinck G, Szabó M, Fekete J, Orfanos CE. Elephantiasis nostras verrucosa: beneficial effect of oral etretinate therapy. Br J Dermatol. 1992 Oct;127(4):411-6. | CrossRef | PubMed |
11. Fredman R, Tenenhaus M. Elephantiasis nostras verrucosa. Eplasty. 2012;12:ic14. [on line] | Link |
12. Iwao F, Sato-Matsumura KC, Sawamura D, Shimizu H. Elephantiasis nostras verrucosa successfully treated by surgical debridement. Dermatol Surg. 2004 Jun;30(6):939-41. | CrossRef | PubMed |
13. Boyd J, Sloan S, Meffert J. Elephantiasis nostrum verrucosa of the abdomen: clinical results with tazarotene. J Drugs Dermatol. 2004;3(4):446-8. | PubMed |

Etemad SA, Dewan AK. Kaposi Sarcoma Updates. Dermatol Clin. 2019 Oct;37(4):505-517 | CrossRef | PubMed |

Dietrich CF, Chaubal N, Hoerauf A, Kling K, Piontek MS, Steffgen L, Mand S, Dong Y. Review of Dancing Parasites in Lymphatic Filariasis. Ultrasound Int Open. 2019 Mar;5(2):E65-E74. | CrossRef | PubMed |

Namazi N, Ershadi S, Rahbar L, Ghassemipur M. Classic Kaposi sarcoma presenting as elephantiasis nostras verrucosa. Iran J Dermatol. 2015;18(1):25-8. [on line] | Link |

Liaw FY, Huang CF, Wu YC, Wu BY. Elephantiasis nostras verrucosa: swelling with verrucose appearance of lower limbs. Can Fam Physician. 2012;58(10):e551-3. [on line] | Link |

Sathyakumar S, Suh JS, Sharp VL, Polsky B. Images in HIV/AIDS. Elephantiasis nostras verrucosa secondary to Kaposi sarcoma: a rare case. AIDS Read. 2008;18(2):81-2. | PubMed |

Hengge UR, Stocks K, Goos M. Acquired immune deficiency syndrome-related hyperkeratotic Kaposi's sarcoma with severe lymphoedema: report of five cases. Br J Dermatol. 2000 Mar;142(3):501-5. | CrossRef | PubMed |

Radu O, Pantanowitz L. Kaposi sarcoma. Arch Pathol Lab Med. 2013 Feb;137(2):289-94. | CrossRef | PubMed |

Bishnoi A, Vinay K, Vishwajeet V, Saikia UN, Panigrahi I, Kumaran MS. Warty Fingers and Toes in a Child With Congenital Lymphedema: Elephantiasis Nostras Verrucosa. JAMA Dermatol. 2018 Jul 1;154(7):849-850. | CrossRef | PubMed |

Sisto K, Khachemoune A. Elephantiasis nostras verrucosa: a review. Am J Clin Dermatol. 2008;9(3):141-6. | CrossRef | PubMed |

Zouboulis CC, Biczó S, Gollnick H, Reupke HJ, Rinck G, Szabó M, Fekete J, Orfanos CE. Elephantiasis nostras verrucosa: beneficial effect of oral etretinate therapy. Br J Dermatol. 1992 Oct;127(4):411-6. | CrossRef | PubMed |

Fredman R, Tenenhaus M. Elephantiasis nostras verrucosa. Eplasty. 2012;12:ic14. [on line] | Link |

Iwao F, Sato-Matsumura KC, Sawamura D, Shimizu H. Elephantiasis nostras verrucosa successfully treated by surgical debridement. Dermatol Surg. 2004 Jun;30(6):939-41. | CrossRef | PubMed |

Boyd J, Sloan S, Meffert J. Elephantiasis nostrum verrucosa of the abdomen: clinical results with tazarotene. J Drugs Dermatol. 2004;3(4):446-8. | PubMed |