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Red genitals: New roads to recovery

DII small banner By Warren R. Heymann, MD
July 23, 2018

Red scrotum syndrome. Sharp border between the erythematous skin and the normal scrotum skin in a 65-year-old male patient.
Credit: JAAD

I searched PubMed for Quality of Life (QoL) studies on the Red Scrotum Syndrome (RSS) and Red Vulva Syndrome (RVS); I found no such manuscripts. On a practical level, none are needed — every person I have encountered with either condition has been in misery due to any combination of pruritus, burning, or pain.

Far more literature has been published about RSS than the RVS. Although RSS has been classified as an “orphan” disease, with a prevalence of less than 1 per 2000 (1), I believe that it is much more common than the literature suggests.
The RSS is observed mostly in elderly men over the age of 60. (I despise that definition of elderly!) The disorder may follow the prolonged application of topical steroids to the scrotum. Sharply defined erythema involving the anterior scrotum, which may extend posteriorly, or on to the base of the penis, is the clinical hallmark of the process. Scaling, lichenification, oozing, or infiltrations are not observed. Histologic features are nonspecific. (2)

The differential diagnosis includes atopic dermatitis (often lichenified if chronic), allergic or irritant contact dermatitis (oozing if acute), psoriasis (elevated plaque), ichthyosis (scaly), tinea (peripherally scaly with positive KOH), syphilis (poorly demarcated with plasma cells on biopsy), Langerhans cell histiocytosis (often crusted clinically, proven histologically), (3) extramammary disease (plaque, possibly ulcerated, definitive histology), or a fixed drug eruption (round, often resolving with hyperpigmentation). Diagnosing RSS or RVS is based on excluding these other disorders by history, biopsy, cultures, or patch testing.
The pathophysiology of RSS and RVS is unknown, but there are there are three prevailing theories: 1) it is erythromelalgia-like; 2) neurogenic inflammation may be responsible; and 3) it is a rosacea-like disorder compounded by vasodilation. (4) Although the theory that prolonged use of topical steroids is causative has been popularized, they are not the sole etiologic factor; occasionally RSS patients do not present with skin atrophy or telangiectasias. Additionally, there are patients with RSS who have the disease prior to the use of topical steroids. (5)

Be realistic. Patients with RSS/RVS will be treated with topical steroids — prescribed by another colleague, you, or me. Unfortunately, they are ineffective and potentially etiologic. The key is recognizing the disorder, discontinuing the topical steroids, and trying other approaches. Using the pathophysiologic theories as a therapeutic framework, gabapentin, pregabalin, or amitriptyline may be effective for reducing the neurogenic influence that overlaps with erythromelalgia and scrotodynia/vulvodynia. Calcineurin inhibitors may be partially effective. (3,5) From a rosacea-like perspective, there have been several reports of doxycycline being worthwhile, notably for its anti-inflammatory, anti-angiogenic, anti-collagenase, and anti-matrix metalloproteinase effects. (2)

Carvedilol, a nonselective beta-blocker with anti-oxidant and anti-inflammatory activity has now been reported to be effective for both RSS and RVS (2 cases for each). The drug was only administered for a month (6.25 mg daily in the RSS patients and three times a week for those with RVS) — improvement was noted within weeks, followed by complete remission (the patients were followed for 6 months). In these reports, a baseline cardiogram was obtained, and both heart rate and blood pressure were monitored. There were no adverse effects. (4,6)

To date, there are no reports of using topical timolol or alpha-agonists (oxymetazoline, brimonidine) for patients with either RSS or RVS. If the rosacea-like vasodilation paradigm is apt, these topical agents should be effective.

Patients with these syndromes may be associated with psychiatric comorbidities such as depression, anxiety, and obsessive-compulsive disorder. (5) They see red — both literally and figuratively. Being aware of new potential therapeutic measures offers the possibility of genuinely improving their QoL.

Point to remember: Novel therapies that counter vasodilation, such as beta-blockers, may be useful therapeutic agents for patients with either the RSS or RVS.

1. Wollina U. Three orphans one should know: Red scalp, red ear, and red scrotum syndrome. J Eur Acad Dermatol Venereol 2016; 30: e169-70.
2. Byun JW, et al. Red scrotum syndrome: Successful treatment with oral doxycycline. Int J Dermatol 2012; 51: 356-63.
3. Wollina U. Red scrotum syndrome. J Dermatol Case Rep 2011; 5: 38-41.
4. Merhi R, et al. Carvedilol for the treatment of red scrotum syndrome. JAAD Case Rep 2017; 3: 464-6.
5. Miller J, Leicht S. Pregabalin in the treatment of red scrotum syndrome. Dermatol Ther 2016; 29; 244-8.
6. Hajj C, Ayoub N. Carvedilol for treatment of red vulva syndrome. JAMA Dermatol 2018

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