Journal of Clinical and Aesthetic Dermatology

MAY 2018

An evidence-based, peer-reviewed journal for practicing clinicians in the field of dermatology

Issue link: https://jcadonline.epubxp.com/i/979610

Contents of this Issue

Navigation

Page 46 of 55

47 JCAD JOURNAL OF CLINICAL AND AESTHETIC DERMATOLOGY May 2018 • Volume 11 • Number 5 C A S E R E P O R T (to the best of our knowledge). We propose the name "Blaschko-linear congenital mixed hemato-lymphangio-keratoma serpiginosum" to represent the distinctive combination of lesions observed in our patient. Additionally, when considering the segmental location of the lesions, it seems likely that they formed due to some somatic mutations in the different developmental stages of pluripotent mesenchimal cells, which have both angiomatous (blood vessel) and lymphangiomatous (lymphatic vessel) potentials. In our opinion, the mutations were likely responsible for dysfunctional differentiation, migration, and placement of the vascular endothelial cells in the skin. In 1976, Whimster et al 19 proposed a model of pathogenesis for LC development and reported that, during embriogenesis, abnormal lymphatic cisterns grow independently from normal lymphatics in the deep subcutaneous tissue. Contraction of smooth muscle cells that line these cisterns cause dilatation and protrusion of lymphatic vesicles from the skin and result in the appearance of lesions. 20 In 2004, Martinez-Menchon et al 21 supported this hypothesis via their own imaging studies, which showed that large multi-lobular cisterns were located deep in the dermis and did not show any communication with adjacent normal lymphatics. In the light of this information, we also believe that external factors, such as trauma, friction, or cold temperatures, or internal factors, such as hormone imbalances, could be physical or biochemical triggers for an acquired transformation, proliferation, and migration to the upper layers of the pluripotent vascular rudimentary endothelial cells. Moreover, the transformed vascular endothelial cell type and degree of the transformation can be decisive in the clinical expression of the hybrid vascular malformations. Regarding the treatment of LC, various modalities have been suggested. These include surgical excision; cryosurgery; electrocautery; surface ablation with laser (e.g., CO 2 , Er:YAG, pulsed-dye); sclerotherapy with sclerosing agents like OK-432 (picibanil); and superficial radiotherapy. 22–24 However, for treatment of VH, superficial ablative procedures like electrocautery, cryosurgery, and laser ablation can lead to recurrence of the lesion due to its deeper components. 25 The importance of complete excision and grafting is indicated in the successful therapy of the lesions. 26 Recently, a topical application of imiquimod cream in the treatment of both LC 27 and hemangioma 28 has been recommended. In our case, the patient refused to undergo Er:YAG laser ablation; thus, we attempted to treat this unique combination of lesions with topical imiquimod. Unfortunately, due to lack of tolerance to the treatment by the patient and lack of improvement in the lesions, imiquimod therapy was discontinued after three months, and the patient was lost to follow-up. We hope that further studies in the fields of molecular genetics and immunobiology will elucidate the pathogenesis of such lesions. REFERENCES 1. Requena L, Sangueza OP. Cutaneous vascular anomalies. Part I: Hamartomas, malformations and dilation of preexisting vessels. J Am Acad Dermatol. 1997;37(4):523–549. 2. Chen JH, Wang KH, Hu CH, Chiu JS. Atypical angioma serpiginosum. Yonsei Med J. 2008;49(3):509–513. 3. Bayramgurler D, Filinte D, Kiran R. Angioma serpiginosum with sole involvement. Eur J Dermatol. 2008;18:708–709. 4. Aggarwal K, Gupta S, Jain VK, Marwah N. Congenital lymphangioma circumscriptum of the vulva. Indian Pediatr. 2009;46(5):428–429. 5. Mülliken JB, Glowacki J. Hemangiomas and vascular malformations in infants and children: a classification based on endothelial characteristics. Plast Reconstr Surg. 1982;69(3):412–422. 6. Debbarman P, Roy S, Kumar P. Angiokeratoma circumscriptum neviforme. Indian Pediatr. 2012;49(1):80. 7. Popadic M. Evolution of verrucous hemangioma. Indian J Dermatol Venereol Leprol. 2012;78(4):520. 8. Wendt W, Kietzman H, Schubert C, Kaiserling E. Progressive lymphangiokeratoma and angiosarcoma (Stewart-Treves syndrome) in congenital lymphedema. Hautarzt. 1988;39(3):155–160. 9. Terushkin V, Marmon S, Fischer M, et al. Verrucous lymphangioma circumscriptum. Dermatol Online J. 2012;18(12):9. 10. Amouri M, Masmoudi A, Boudaya S, et al. Acquired lymphangioma circumscriptum of the vulva. Dermatol Online J. 2007;13(4):10. 11. Gnanaraj P, Revathy V, Venugopal V, et al. Secondary lymphangioma of vulva: a report of two cases. Indian J Dermatol. 2012;57(2):149–151. 12. Harwood CA, Mortimer PS. Acquired vulval lymphangiomata mimicking genital warts. Br J Dermatol. 1993;129(3):334–336. 13. Pusztaszeri MP, Seelentag W, Bosman FT. Immunohistochemical expression of endothelial markers of CD31, CD34, von Willebrand factor, and Fli-1 in normal human tissues. J Histochem Cytochem. 2006;54(4):385–395. 14. Ghosh SK, Bandyopadhyay D, Gloshal L, Haldar S. Angiokeratoma circumscriptum naeviforme: a case report of a rare disease. Dermatol Online J. 2011;17(9):11. 15. Debelenko LV, Perez-Atayde AR, Mulliken JB, et al. D2-40 immunohistochemical analysis of pediatric vascular tumors reveals positivity in kaposiform hemangioentothelioma. Mod Pathol. 2005;18(11):1454–1460. 16. Tennant LB, Mulliken JB, Perez-Atayde AR, Kozakewich HP. Verrucous hemangioma revisited. Pediatr Dermatol. 2006;23(3):208–215. 17. Kim JH, Nam TS, Kim SH. Solitary angiokeratoma developed in one area lymphangioma circumscriptum. J Korean Med Sci. 1988;3(4):169– 170. 18. Neumann E. Some new observations on the genesis of angioma serpiginosum. Acta Derm Venereol. 1971;51(3):194–198. 19. Whimster IW. The pathology of lymphangioma circumscriptum. Br J Dermatol. 1976;94(5):473–486. 20. Terushkin V, Marmon S, Fischer M, et al. Verrucous lymphangioma circumscriptum. Dermatol Online J. 2012;18(12):9. 21. Martinez-Menchon T, Mahiques-Santos L, Febrer- Bosch I, et al. Lymphangioma circumscriptum: an example of Whimster's hypothesis. Pediatr Dermatol. 2004;21(6):652–554. 22. Aggarwal K, Gupta S, Jain VK, Marwah N. Congenital lymphangioma circumscriptum of the vulva. Indian Pediatr. 2009;46(5):428–429. 23. Mehta V, Nayak S, Balachandran C, et al. Extensive congenital vulvar lymphangioma mimicking genital warts. Indian J Dermatol. 2010;55(1):121–122. 24. Gnanaraj P, Revathy V, Venugopal V, et al. Secondary lymphangioma of vulva: a report of two cases. Indian J Dermatol. 2012;57(2):149–151. 25. Yasar A, Ermertcan AT, Bilal C, et al. Verrucous hemangioma. Indian J Dermatol Venereol Leprol. 2009;75(5):528–530. 26. Pavithra S, Mallya H, Kini H, Pai GS. Verrucous hemangioma or angiokeratoma? A missed diagnosis. Indian J Dermatol. 2011;56(5):599–600. 27. Wang JY, Liu LF, Mao XH. Treatment of lymphangioma circumscriptum with topical imiquimod %5 cream. Dermatol Surg. 2012;38(9):1566–1569. 28. Sun ZJ, Zhao YF, Zhang WF. Immune response: a possible role in the pathophysiology of hemangioma. Med Hypotheses. 2007;68(2):353–355. JCAD

Articles in this issue

Archives of this issue

view archives of Journal of Clinical and Aesthetic Dermatology - MAY 2018