Hidradenitis suppurativa-associated lymphedema: A case and review of the literature
DOI:
https://doi.org/10.5070/rhw51f12Keywords:
hidradenitis suppurativa, Lymphedema, SurgeryAbstract
Hidradenitis suppurativa is a chronic inflammatory skin disorder characterized by painful recurring pustules, nodules, abscesses, and sinus tract formation. Secondary lymphedema associated with hidradenitis suppurativa is an uncommon but severe condition, often resulting in significant morbidity. Although the exact link between lymphedema and chronic hidradenitis suppurativa is not fully understood, the chronic inflammatory process with scarring is a likely an important factor. Herein, we reported a 52-year-old man with untreated chronic hidradenitis suppurativa, nonalcoholic fatty liver disease-related cirrhosis, and secondary massive scrotal lymphedema, complicated by cellulitis and sepsis. Despite appropriate treatment, the patient’s condition deteriorated and he unfortunately passed away two weeks after hospitalization. Additionally, we reviewed the literature on hidradenitis suppurativa-associated lymphedema, analyzing data from 51 patients. The majority were men with long-standing severe hidradenitis suppurativa. Surgical intervention was identified as the most commonly effective treatment modality, offering substantial improvements in function and appearance. However, some patients experienced postsurgery adverse events. In conclusion, this report highlights the importance of early detection and management of hidradenitis suppurativa and its complications to prevent patients' significant morbidity.
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1. Girouard SD, Falk RH, Rennke HG, et al. Hidradenitis suppurativa resulting in systemic amyloid A amyloidosis: A case report and review of the literature. Dermatol Online J. 2012;18:2. [PMID: 22345175].
2. Yuan JT, Naik HB. Complications of hidradenitis suppurativa. Semin Cutan Med Surg. 2017;36:79-85. [PMID: 29096875].
3. Jemec GB, Heidenheim M, Nielsen NH. The prevalence of hidradenitis suppurativa and its potential precursor lesions. J Am Acad Dermatol. 1996;35:191-4. [PMID: 8687399].
4. Fu MR, Ridner SH, Hu SH, et al. Psychosocial impact of lymphedema: A systematic review of literature from 2004 to 2011. Psychooncology. 2013;22:1466-84. [PMID: 23204029].
5. Grada AA, Phillips TJ. Lymphedema: Pathophysiology and clinical manifestations. J Am Acad Dermatol. 2017;77:1009-20. [PMID: 28780101].
6. Menter A. Recognizing and managing comorbidities and complications in hidradenitis suppurativa. Semin Cutan Med Surg. 2014;33:S54-6. [PMID: 25452635].
7. Ghalamkarpour F, Asadi-kani Z, Moradi A, et al. Acquired lymphangioma circumscriptum secondary to tuberculosis: A rare case report. Dermatol Ther. 2020;33:e13463. [PMID: 32421173].
8. Micieli R, Alavi A. Lymphedema in patients with hidradenitis suppurativa: a systematic review of published literature. Int J Dermatol. 2018;57:1471-80. [PMID: 29645633].
9. Sanchez-Diaz M, Martinez-Lopez A, Salvador-Rodriguez L, et al. The role of biologic treatment in special scenarios in hidradenitis suppurativa: Facial and nape phenotype, dissecting cellulitis of the scalp, and lymphedema. J Dermatol Treat. 2021;34:e14829. [PMID: 33245245].
10. Caposiena Caro RD, Cannizzaro M, Mazzeo M, et al. Letter to the Editor regarding “Lymphedema in patients with hidradenitis suppurativa: A systematic review of published literature”. Int J Dermatol. 2018. [PMID: 30111228].
11. Musumeci ML, Scilletta A, Sorci F, et al. Genital lymphedema associated with hidradenitis suppurativa unresponsive to adalimumab treatment. JAAD Case Rep. 2019;5:326-8. [PMID: 31112118].
12. Smith AD, Curtin GP, Allen NC, et al. Management of genital hidradenitis suppurativa and lymphoedema with the restoration of erectile function. Australas J Dermatol. 2022;63:e184-e6. [PMID: 34456785].
13. Yu Z, Sheng L, Cao W, et al. Surgical reconstruction of massive scrotal lymphedema associated with hidradenitis suppurativa: a case report. Urology. 2021;10:1800-5. [PMID: 33434717].
14. Moschella SL. Is there a role for infliximab in the current therapy of hidradenitis suppurativa? A report of three treated cases. Int J Dermatol. 2007;46:1287-91. [PMID: 17959795].
15. Bains P, Mahajan A. Case report: lymphedema vulva secondary to hidradenitis suppurativa. J Egypt Women Dermatol Soc. 2017;14. [PMID: 29155392].
16. Saunte DML, Jemec GBE. Hidradenitis Suppurativa: Advances in Diagnosis and Treatment. JAMA. 2017;318:2019-32. [PMID: 29171014].
17. Coseriu A, Prodan L, Fetti A, et al. Surgical management of massive penoscrotal lymphedema. Case report and literature review. Ann Ital Chir. 2022;11. [PMID: 35503714].
18. Snyder SN, Kolli SS, Hughes OB, et al. Hidradenitis suppurativa and scrotal elephantiasis case report. J Dermatol Nurses Assoc. 2020;12. [PMID: 33218938].
19. Hazen PG, Daoud S. Scrotal hidradenitis suppurativa with secondary lymphedema and lymphangiomata: Successful management with carbon dioxide laser excision and marsupialization. Dermatol Surg. 2015;41:431-2. [PMID: 25777486].
20. Moosbrugger EA, Mutasim DF. Hidradenitis suppurativa complicated by severe lymphedema and lymphangiectasias. J Am Acad Dermatol. 2011;64:1223-4. [PMID: 21300594].
21. Fernandes NC, Daflom MF, Cuzzi TJAbd. Genital elephantiasis secondary to hidradenitis suppurativa: A case report. J Urol. 1999;387-9. [PMID: 10371277].
22. Kok K, Lahiri A. Penile degloving: An unusual presentation of hidradenitis suppurativa. Ann R Coll Surg Engl. 2012;94:e101-2. [PMID: 22371097].
23. Jiménez Gómez N, Bernardo AA, Luque PB, et al. Refractory inguinoscrotal suppurative hidradenitis successfully treated with surgery and adalimumab: The importance of the multidisciplinary approach. Exp Dermatol. 2019;28:47. [PMID: 31701973].
24. Lelonek E, Bieniek A, Matusiak Ł, et al. Improved psychosocial status after surgery for genital elephantiasis due to hidradenitis suppurativa: A prospective study of a case series. Acta Derm Venereol. 2021;101:adv00389. [PMID: 33399480].
25. Chen ML, Odom B, Santucci RA. Surgical management of genitoperineal hidradenitis suppurativa in men. Urology. 2014;83:1412-7. [PMID: 24751256].
26. Corder B, Googe B, Velazquez A, et al. Surgical management of acquired buried penis and scrotal lymphedema: A retrospective review. J Plast Reconstr Aesthet Surg. 2023;85:18-23. [PMID: 36647324].
27. Liu X, Xie C, Zhou F, et al. Massive genital lymphedema associated with hidradenitis suppurativa: Report of 4 cases. Int J Dermatol. 2018;57:1427-32. [PMID: 29541326].
28. Sinclair E, Williams S, Andrew J, et al. Cutaneous lymphedema: case series and review of the literature. J Am Acad Dermatol. 2020;83:147-51. [PMID: 31906687].
29. Saleh SM, Nashwan AJ, Niazi AK, et al. Lymphedema of the external genitalia in hidradenitis suppurativa: a clinical dilemma. Br J Dermatol. 2021;185:1253-1256. [PMID: 33912497].
30. Ghabril M, Cummings J, Reynolds C, et al. Genital lymphedema secondary to hidradenitis suppurativa: A case report of surgical treatment. J Urol. 2016;195:1253-5. [PMID: 26678081]
31. Phillips TJ, Menter A. Advances in the management of hidradenitis suppurativa: part 2. J Am Acad Dermatol. 2016;74:879-83. [PMID: 26726472].
32. Segura J, de la Peña R, Gallego M, et al. Lymphedema of the external genitalia secondary to hidradenitis suppurativa. J Dermatol. 2015;42:523-5. [PMID: 25723657].
33. Kwok CS, Barona M, McCallum C, et al. Lymphedema associated with hidradenitis suppurativa: A review. J Clin Dermatol. 2020;31:45-55. [PMID: 31925188].
34. Wright MB, Wright J, Patel S, et al. Management of massive genital lymphedema due to hidradenitis suppurativa with surgical reconstruction: A case series. J Urol. 2022;207:580-585. [PMID: 34833847].
35. Zhang Y, Jiang Z, Song H, et al. Surgical approaches for genital elephantiasis due to hidradenitis suppurativa: A systematic review. BMC Urol. 2021;21:98. [PMID: 34181375].
36. Hong SJ, Lee W, Kim E, et al. Scrotal elephantiasis due to hidradenitis suppurativa: Case report and review of the literature. Ann Plast Surg. 2019;82:549-552. [PMID: 30328912].
37. Samartzis K, Benyounes L, Oh J, et al. Case report: Genital elephantiasis associated with hidradenitis suppurativa. J Clin Urol. 2021;15:420-423. [PMID: 34614359].
38. Mahmoudi M, Alavi A, Bewley A, et al. Complications of hidradenitis suppurativa. J Dermatol Treat. 2021;32:698-707. [PMID: 33299335].
39. Sabharwal A, Brautbar A, Shrestha V, et al. Surgical management of hidradenitis suppurativa: A systematic review of surgical approaches. JAMA Dermatol. 2021;157:1157-1164. [PMID: 34201867].
40. Seitz IA, Simoens P, Pross M, et al. Lymphedema and hidradenitis suppurativa: A rare complication. Dermatol Surg. 2020;46:1330-1334. [PMID: 32527393].
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Copyright (c) 2025 Elnaz Pourgholi, Mahboobeh Freidoon, Sahar Dadkhahfar, Elham Keykha, Hojjat Layegh, Shirin Zaresharifi (Author)

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