Scholarly article on topic 'Red scrotum syndrome: A form of corticosteroid addiction, a variant of erythermalgia, or a distinct entity?'

Red scrotum syndrome: A form of corticosteroid addiction, a variant of erythermalgia, or a distinct entity? Academic research paper on "Clinical medicine"

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Academic research paper on topic "Red scrotum syndrome: A form of corticosteroid addiction, a variant of erythermalgia, or a distinct entity?"

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DERMATOLOGICA SINICA xxx (2016) 1-2

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Dermatológica Sinica

journal homepage: http://www.derm-sinica.com

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Red scrotum syndrome: A form of corticosteroid addiction, a variant of erythermalgia, or a distinct entity?

Dear Editor,

Red scrotum syndrome (RSS),1-5 first described by Fisher,1 is a chronic cutaneous condition characterized by persistent erythema and usually associated with itching and/or burning sensation and hyperalgesia.1-5 It always involves the anterior half of the scrotum, and may extend to its posterior part, and even to the base of the penis.1 e5 No extragenital involvement has been described previously. To date, fewer than 30 cases have been reported.1-5

A 70-year-old man was referred because of a 2-week history of persistently localized red scrotum associated with burning sensation and pain. The symptoms could be aggravated by warmth and relieved by cooling. During the attack, the patient did not have associated headache. He found relief by staying in a cold environment or lying down with legs apart. The patient had tinea cruris and also urticaria months earlier, which were cured by topical mi-conazole cream and antihistamines, respectively. He denied prior topical steroid use over the genital area. His past medical and family history were unremarkable.

Cutaneous examination revealed well-demarcated erythema limited mainly to the anterior part of the scrotum (Figure 1), without any specific changes or involvement elsewhere. Microscopic examination with KOH mount was negative. The complete blood count and biochemical profiles, as well as auto-antibodies and antinuclear antibody were normal or negative. He was diagnosed as RSS and started oral aspirin 100 mg twice daily and parox-etine 20 mg daily, resulting in moderate improvement until he had to stop the treatment because of severe dizziness. He then applied topical solution containing traditional Chinese medicine, benzoic acid, and salicylic acid, resulting in excellent response after 1 week of treatment. One month after stopping the medication, recurrence occurred, this time showing poor response to the prior solution and medications. Oral ibuprofen and amitriptyline were recommended, leading to excellent improvement. He was lesion free on 1-year follow up.

RSS is not infrequent, but is seldom reported and is often mis-diagnosed or underdiagnosed.2 The lesion lacks oozing, scaling, swelling, cracking, infiltration, or lichenification except for scratching-induced oozing, but may present as variable atrophy, telangiectases, and tenderness.1 e5 Histology shows nonspecific

Conflicts of interest: The authors declare that they have no financial or non-financial conflicts of interest related to the subject matter or materials discussed in this article.

changes, including: variable spongiosis, atrophy, or even normal epidermis; mild telangiectases with sparse to mild perivascular lymphocytic infiltrate in the dermis; and a picture resembling erythematotelangiectatic rosacea.2

The etiopathogenesis of RSS remains unknown.1,2 Its redness is considered to be due to vasodilatory effects of nitric oxide synthesized by the vascular endothelium.6,7 The diagnosis is based mainly on the clinical manifestations.3 As most reported cases had prolonged application of topical steroids before the diag-nosis,1-5 and some were relieved after stopping the use of topical steroids, or had severe rebound after withdrawal of the steroids,2 these suggest that topical steroids, at least partially, might be responsible for the mechanism of action in some patients,2 and some RSS might be due to steroid addiction. Narang and col-leagues2 considered that "RSS appears to be a manifestation of corticosteroid misuse rather than a primary disease". As not all the reported cases have prior topical steroid use, we considered that RSS is not a distinct entity, but only clinical manifestations of other cutaneous diseases: those caused by topical steroids are steroid addiction, and those occurring spontaneously, as in our patient, may be a variant of erythromelalgia, as Prevost and En-glish3 and Wollina5 suggested.

Erythromelalgia, also called erythermalgia, is characterized by sudden erythema, warmth, and burning pain; it commonly involves the extremities, and may extend to the face, neck, ears, and even the scrotum in a symmetrical manner.8,9 In rare instance, it may afflict the face and/or ears, and the vulva alone,8 or even be limited to unilateral thigh.9 The clinical features of RSS mimic those of erythromelalgia, and some RSS has a positive response to gabapentin or pregabalin2,3,5 that is commonly used in treating primary erythromelalgia. However, RSS lacks lesional warmth, and presents as persistent erythema rather than as an attacking rash; such conditions suggest that spontaneous RSS may be an atypical variant of erythromelalgia involving the scrotum. The red scalp, red ear, and red scrotum syndromes might be similar diseases or variants of erythromelal-gia that are present in different locations. However, if an isolated form of erythromelalgia of the scrotum actually exists, some of the earlier cases may have been misinterpreted as RSS instead of erythromelalgia.

Erythromalgia of the scrotum should be distinguished from erythrasma, contact dermatitis, extramammary Paget's disease, seborrheic dermatitis, and steroid addiction. Based on the clinical manifestations, making a correct diagnosis is not difficult.

http://dx.doi.org/10.1016/j.dsi.2016.01.002

1027-8117/Copyright © 2016, Taiwanese Dermatological Association. Published by Elsevier Taiwan LLC. This is an open access article under the CC BY-NC-ND license (http:// creativecommons.org/licenses/by-nc-nd/4.0/).

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Correspondence / Dermatologica Sinica xxx (2016) 1—2

Treatment for RSS is still a challenge for dermatologists. Many therapies including topical or oral corticosteroid, antidepressants alone, and antifungal and antibacterial agents have been tried, showing no benefits. Recently, doxycycline, gabapentin, and prega-bapentin showed effectiveness in its treatment.1 e5

Acknowledgments

The authors are indebted to Professor Rashmi Sarkar, M.D., Department of Dermatology, Maulana Azad Medical College and LNJP Hospital, New Delhi, India, for her assistance in revising English grammatical errors in this manuscript.

Juan-Hua Liu

Department of Dermatology, The Eastern Hospital of The First Affiliated Hospital, Sun Yat-sen University, Guangzhou 510700, China

Shao-Wen Feng

Department of Pharmacy, The Eastern Hospital of The First Affiliated Hospital, Sun Yat-sen University, Guangzhou 510700, China

Ze-Yu Luo

2011 Grade, Department of Clinical Medicine, Medical College, University of South

China, Hengyang 421001, China

Di-Qing Luo*

Department of Dermatology, The Eastern Hospital of The First Affiliated Hospital, Sun Yat-sen University, Guangzhou 510700, China

* Corresponding author. Department of Dermatology, The Eastern Hospital of The First Affiliated Hospital, Sun Yat-sen University, 183 Huangpu Road East, Guangzhou

510700, China.

E-mail addresses: luodq@mail.sysu.edu.cn, diqing.luo@medmail.com.cn (D.-Q. Luo).

1. Fisher BK. The red scrotum syndrome. Cutis 1997;60:139-41.

2. Narang T, Kumaran MS, Dogra S, Saikia UN, Kumar B. Red scrotum syndrome: idiopathic neurovascular phenomenon or steroid addiction? Sex Health 2013;10:452-5.

3. Prevost N, English 3rd JC. Case reports: red scrotal syndrome: a localized pheno-typical expression of erythromelalgia. J Drugs Dermatol 2007;6:935-6.

4. Byun JW, Hong WK, Han SH, et al. Red scrotum syndrome: successful treatment with oral doxycycline. Int J Dermatol 2012;51:362-3.

5. Wollina U. Red scrotum syndrome. J Dermatol Case Rep 2011 ;5:38-41.

6. Rapaport MJ, Lebwohl M. Corticosteroid addiction and withdrawal in the atopic: the red burning skin syndrome. Clin Dermatol 2003;21:201-14.

7. Rapaport M. Rebound vasodilation from long-term topical corticosteroid use. Arch Dermatol 2007;143:268-9.

8. Chen MC, Xu QF, Luo DQ, Li X, He DY. Erythema associated with pain and warmth on face and ears: a variant of erythermalgia or red ear syndrome? J Headache Pain 2014;15:18. http://dx.doi.org/10.1186/1129-2377-15-18.

9. Wu HH, Wang MJ, Luo DQ, Lin Q. Localized secondary erythromelalgia in an 11-year-old boy. Pediatr Dermatol 2013;30:e244-5.

Received: Sep 11, 2015 Revised: Dec 12, 2015 Accepted: Jan 23, 2016

References