SKIN July 2022 Volume 6 Issue 4 (c) 2022 THE AUTHORS. Published by the National Society for Cutaneous Medicine. 328 BRIEF ARTICLE A Rare Presentation of Angiolymphoid Hyperplasia with Eosinophilia Involving the Scrotum Derrick Adams DO, FAOCD1, Kevin Mai, BS2 1 Lassen Medical Center – Red Bluff 2 College of Osteopathic Medicine of the Pacific, Western University of Health Sciences - Pomona, CA Angiolymphoid hyperplasia with eosinophilia (ALHE) is an uncommon vascular proliferation. The wide histologic variations in presentation have spawned an equally wide degree of nomenclature of ALHE. Epitheliod hemangioma, pseudopyogenic granuloma, inflammatory angiomatous nodule, popular angioplasia, subcutaneous angioblastic lymphoid hyperplasia with eosinophilia and lymphofolliculosis, intravenous atypical vascular proliferation and histiocytoid hemangioma have all been utilized by various authors over the decades.1 The lesions of ALHE are benign and typically present as papules or nodules on the face or neck.1 There is a slight predilection of females over males in ALHE with most cases occurring between the ages of 20 and 50.2 It is likely an underreported condition, therefore, true demographic information may be lacking. Clinically, ALHE presents as pink to red- brown papules or nodules on the face or neck with a tendency to form in the preauricular area. ALHE lesions often bleed easily and present as either asymptomatic or with pain, pruritis, or pulsations.3 Renal disease and peripheral eosinophilia occur in a minority of patients.4 Involvement of the oral mucosa, bone, muscle, parapharyngeal space, tongue, colon, and salivary glands are rare.5 There are less than 30 cases reported of male urogenital involvement. Here we present the third reported case of ALHE with scrotal involvement.6,7 A 17-year-old male with a history of refractory verrucous vulgaris presented for asymptomatic nodules on scrotum increasing in size and number over the last 3 years (Figure 1). The patient denied any sexual history, pain, discharge, or trauma to the area. A biopsy was taken to rule out ABSTRACT Angiolymphoid hyperplasia with eosinophilia (ALHE) is a rare vascular proliferation that typically manifests as pink to reddish brown papules or nodules. These lesions classically affect the face or neck. Involvement of other structures including the tongue, colon, salivary glands, and muscle are rare. Male urogenital involvement for example, had less than 30 cases reported. Here we present the third reported case of ALHE involving the scrotum in a 17-year-old male. INTRODUCTION CASE REPORT SKIN July 2022 Volume 6 Issue 4 (c) 2022 THE AUTHORS. Published by the National Society for Cutaneous Medicine. 329 scrotal syringomas or steatocystoma multiplex. Dense collections of lymphocytes with scattered histiocytes and eosinophils were appreciated in the dermis against a background of increased vasculature. Microbial stains to rule out an infectious trigger were negative. Angiolymphoid hyperplasia with eosinophilia was diagnosed. Treatment options were discussed with the patient, but he has yet to decide upon a course of action. Molecular clonality was not investigated due to cost constraints. Figure 1. The first description of ALHE was by Wells and Whimster in 1969.8 Pathogenesis is unknown, but there is speculation that the lesions result from trauma to an artery or vein, infection, or from uncontrolled vascular factors.9 An estimated less than 10% of patients can recall a history of localized trauma or infection. In these cases, the median interval of lesion manifestation was 30 months.9 The results from a study of 53 out of 116 patients by Olsen and Helwig suggested that ALHE may be caused by arteriovenous shunts.9 Additionally, the patient’s history of refractory verruca vulgaris may provide further insight into the pathophysiology of ALHE. Since infections have been raised as a possible origin, might there be a possible association between HPV and ALHE? Our case also raises awareness for the spectrum of presentations ALHE may manifest as when it involves the scrotum. Compared to the other two known reported cases of ALHE with scrotal involvement (Figure 2), our case is the only one that presented bilaterally on the scrotum. Hence, dismantling any notion of using unilateral/bilateral spatial arrangements as a possible differentiating factor when considering scrotal involvement of ALHE. Furthermore, when analyzed alongside previous cases, it appears ALHE of the scrotum may vary in many other characteristics such as tenderness, pruritis and size. Figure 2. Case reports of angiolymphoid hyperplasia with eosinophilia have been inconsistent and underreported due to cases being incorrectly diagnosed as Kimura’s disease (KD).9 While ALHE and KD have similar characteristics, they are now characterized as different disorders.1,9 Histologically, the main characteristic of ALHE is vascular proliferation sometimes demonstrating cobble stoning with an eosinophil infiltrate.1 KD is generally deeper with lymphoid follicles, dense fibrosis, and an eosinophil infiltrate.1 ALHE is a benign primary vascular proliferation, while KD is due to chronic inflammation and is either an allergic or autoimmune disorder.10 While a DISCUSSION SKIN July 2022 Volume 6 Issue 4 (c) 2022 THE AUTHORS. Published by the National Society for Cutaneous Medicine. 330 case report by Chong et al presents a patient demonstrating features of both conditions simultaneously, the significance of this occurrence remains unknown.11 Many treatment modalities have been used in attempts to clear ALHE lesions, but it is notoriously refractory to current lines of treatment. Surgical excision is often used after other non-invasive options have failed. Other commonly used treatments include intralesional corticosteroids and laser therapy.3 Lesions after surgical excision have a 33-50% recurrence rate. Mohs micrographic surgery has also been used for treatment with results showing promise of completely resolving lesions with no recurrence and minimal destruction of healthy tissue.12 The high cost and exclusion criteria however preclude most patients from considering this option. Other treatment modalities have included topical tacrolimus, electrocoagulation, corticosteroids, sclerotherapy, indomethacin, retinoids, cryotherapy, pentoxifylline, intravenous vinblastine sulfate, radiotherapy, interferon α-2b therapy, intralesional bleomycin, and radiofrequency excision.12 In conclusion, we are reporting only the third known case of scrotal angiolymphoid hyperplasia with eosinophilia. It is a rare but benign vascular proliferation that typically presents on the head or neck area. Due to the high rates of recurrence, many treatment modalities have been used in attempts to clear lesions. Conflict of Interest Disclosures: None Funding: None Corresponding Author: Kevin Mai, BS College of Osteopathic Medicine of the Pacific Western University of Health Sciences Pomona, CA Email: Kevin.Mai@Westernu.edu References: 1. Chun SI, Ji HG. Kimura's disease and angiolymphoid hyperplasia with eosinophilia: clinical and histopathologic differences. J Am Acad Dermatol. Dec 1992;27(6 Pt 1):954-8. doi:10.1016/0190-9622(92)70293-o 2. Azizzadeh M, Namazi MR, Dastghaib L, Sari- Aslani F. Angiolymphoid hyperplasia with eosinophilia and nephrotic syndrome. Int J Dermatol. Mar 2005;44(3):242-4. doi:10.1111/j.1365-4632.2004.02030.x 3. Adler BL, Krausz AE, Minuti A, Silverberg JI, Lev-Tov H. Epidemiology and treatment of angiolymphoid hyperplasia with eosinophilia (ALHE): A systematic review. J Am Acad Dermatol. Mar 2016;74(3):506-12.e11. doi:10.1016/j.jaad.2015.10.011 4. Ramchandani PL, Sabesan T, Hussein K. Angiolymphoid hyperplasia with eosinophilia masquerading as Kimura disease. Br J Oral Maxillofac Surg. Jun 2005;43(3):249-52. doi:10.1016/j.bjoms.2004.11.023 5. Tsuboi H, Masuzawa M, Katsuoka K. Angiolymphoid hyperplasia with eosinophilia affecting the nail bed and underlying bone. J Dermatol. Jun 2006;33(6):399-402. doi:10.1111/j.1346-8138.2006.00094.x 6. Chen JF, Gao HW, Wu BY, Tsai WC, Chiang CP. Angiolymphoid hyperplasia with eosinophilia affecting the scrotum: a rare case report with molecular evidence of T-cell clonality. J Dermatol. Apr 2010;37(4):355-9. doi:10.1111/j.1346-8138.2010.00818.x 7. Park J, Hwang SR, Song KH, Kim JI, Rhee CH, Yun SK. Angiolymphoid hyperplasia with eosinophilia affecting the scrotum of a child. Eur J Dermatol. 2013 May-Jun 2013;23(3):423-4. doi:10.1684/ejd.2013.2051 8. Wells GC, Whimster IW. Subcutaneous angiolymphoid hyperplasia with eosinophilia. Br J Dermatol. Jan 1969;81(1):1-14. doi:10.1111/j.1365-2133.1969.tb15914.x 9. Olsen TG, Helwig EB. Angiolymphoid hyperplasia with eosinophilia. A clinicopathologic study of 116 patients. J Am Acad Dermatol. May 1985;12(5 Pt 1):781-96. doi:10.1016/s0190-9622(85)70098-9 CONCLUSION SKIN July 2022 Volume 6 Issue 4 (c) 2022 THE AUTHORS. Published by the National Society for Cutaneous Medicine. 331 10. Dargent JL, Vannuffel P, Saint-Remy JM, Fisogni S, Facchetti F. Plasmacytoid dendritic cells in Kimura disease. Am J Dermatopathol. Dec 2009;31(8):854-6. doi:10.1097/DAD.0b013e3181a772c6 11. Chong WS, Thomas A, Goh CL. Kimura's disease and angiolymphoid hyperplasia with eosinophilia: two disease entities in the same patient: case report and review of the literature. Int J Dermatol. Feb 2006;45(2):139- 45. doi:10.1111/j.1365-4632.2004.02361.x 12. Miller CJ, Ioffreda MD, Ammirati CT. Mohs micrographic surgery for angiolymphoid hyperplasia with eosinophilia. Dermatol Surg. Aug 2004;30(8):1169-73. doi:10.1111/j.1524- 4725.2004.30349.x