Topical steroid withdrawal syndrome in a mother and son: A case report

Abstract

Topical corticosteroids are first-line treatment for many dermatoses, and are generally considered safe and effective. However, topical steroid withdrawal syndrome can result from use of topical corticosteroids, and this condition is not well-known among physicians. This article reports a mother and son whose presentations of topical steroid withdrawal syndrome following the discontinuation of prolonged, high-potency topical corticosteroid use were nearly identical. This report adds to the growing body of evidence that topical steroid withdrawal syndrome is its own entity, rather than an exacerbation of the underlying dermatosis, and adds to the few pediatric reports of topical steroid withdrawal syndrome. Management for both patients involved topical corticosteroid discontinuation; however, it took approximately 2 years before the majority of their topical steroid withdrawal syndrome manifestations resolved. Increased awareness surrounding this condition is essential to facilitate topical steroid withdrawal syndrome prevention and diagnosis and to decrease topical corticosteroid phobia and increase patient–physician trust.

Introduction

Topical corticosteroids (TCSs) are used to treat conditions including, but not limited to, atopic dermatitis (AD), psoriasis, and contact dermatitis. ¹ When used judiciously, TCS are safe and effective. ¹ Prolonged use, however, may result in topical steroid withdrawal syndrome (TSWS),^1–4 also known as red skin syndrome, or topical steroid addiction.^4,5

Erythematoedematous and papulopustular TSWS are predominant variants of the condition.^2,3 In the former, erythema, scaling, papules +/− nodules, desquamation, and swelling/edema are common signs, with burning/stinging, pruritus, pain, and decreased emollient tolerance being common symptoms. ² The latter is characterized by prominent pustules, papules, +/− nodules, while burning/stinging and edema are less frequent. ²

The face and genitalia are most commonly affected, and TSWS can extend beyond the TCS-treated area.^1,2,4 Adult women are most commonly affected.^2,3 In fact, 2015 and 2021 systematic reviews found that pediatric patients only accounted for 7.1% and 16.9% of TSWS reports, respectively.^2,3

TSWS diagnosis is difficult due to lack of well-established guidelines, but should be considered when burning is a predominant symptom occurring in the context of erythema following the discontinuation of prolonged TCS use. ¹ The most effective TSWS treatment is unclear; however, TCS discontinuation is generally recommended.^2,3

TSWS can have profound impacts on quality of life physically, emotionally, socially, and financially. ⁶ Recently, TSWS has been trending on social media resulting in a growing TCS phobia, decreased adherence, and therapeutic failure.^2,7–9 This is likely exacerbated by the fact that TSWS is not recognized by many physicians or is misdiagnosed as an exacerbation of the original skin condition, harming patient–physician trust. ⁷

Steps are being taken to improve physicians’ knowledge of TSWS. In July of 2022, the Government of Canada made a statement regarding TSWS in a Health Product InfoWatch article, ⁴ and a recently published article in the British Journal of Dermatology highlighted the need to “bridge the gap” between patients and providers in relation to prevention, recognition, and treatment of TSWS. ⁵

This report outlines a mother and her pediatric-aged son with nearly identical presentations of TSWS following the same prolonged, potent TCS regimen. This adds to the evidence that TSWS is its own entity, rather than an exacerbation of an underlying dermatosis. Consent to publish this report was obtained from both patients.

Case report

Patient-A is a 42-year-old female treated with topical hydrocortisone for AD beginning at age 1 year. By age 6 years, Patient-A had been diagnosed with the atopic triad (AD, asthma, allergic rhinitis), and was treated with topical betamethasone valerate and hydrocortisone valerate, fluticasone inhalers, and nasal sprays. These treatments continued into early adulthood.

Due to worsening skin involvement, Patient-A was prescribed desonide and betamethasone dipropionate which were used almost daily, on the hands, wrists, feet, and ankles for 13 years. During this time, she also used other corticosteroids including dexamethasone eye drops, fluticasone inhalers, and nasal sprays.

Patient-B, the 10-year-old son of Patient-A, was diagnosed with AD at age 1 year and treated with topical hydrocortisone with fusidic acid. By age 2 years, his AD worsened, and he therefore began using the same treatment regimen as his mother, desonide and betamethasone dipropionate. TCSs were used on his trunk, hands, and face, almost daily for the subsequent 5 years.

During the 5-year overlap, when Patients-A and B both used desonide and betamethasone dipropionate, they collectively went through twelve and seven 30 g tubes of each, respectively, in approximately 2 months.

In July 2020, Patients-A and B were required to do a 2-week public health isolation related to COVID-19, during which they ran out of TCS. Subsequently, Patients-A and B presented to their local emergency department with erythema, severe burning/stinging, and swelling/edema. They were given supportive management. Others in the household during the isolation, who had not used TCS, did not experience any skin issues.

In the following weeks, based on their history and clinical presentation, both Patients-A and B were diagnosed with TSWS. Treatment included TCS discontinuation and psychological support. Burning/stinging rendered emollients intolerable for the majority of their recovery.

Throughout their recovery, Patients-A and B had widespread skin involvement, with erythema, paresthesia, edema, desquamation, and anhidrosis affecting the face, neck, back, flexural surface of the arms, wrists, and hands. They also experienced madarosis, hair shedding, and significant fatigue and emotional distress.

Patient-A is 2 years TCS free and much improved, but has intermittent intense pruritus. Patient-A uses moisturizers, ciclopirox olamine shampoo, and benzoyl peroxide cleanser. Patient-B is two-and-a-half years TCS free, and is also much improved, although significant skin changes are still present. However, he reports that he is rarely pruritic. Patient-B uses Vaseline and an antibacterial face cleanser.

A timeline of Patient-A and B’s signs/symptoms can be visualized in Figure 1. The progression of Patient-A and B’s TSWS and recovery can be seen in Figures 2 and 3.

Figure 1.

Timeline outlining the signs and symptoms of topical steroid withdrawal syndrome experienced by Patients-A and B.

Figure 2.

Photographs of the face demonstrating topical steroid withdrawal syndrome and recovery in Patient-A (A1–A6) and Patient-B (B1 and B2).

Figure 3.

Photographs of the body demonstrating topical steroid withdrawal syndrome and recovery in Patient-A (A1–A5) and Patient-B (B1 and B2). Photograph A & B1 displays a real-time comparison of topical steroid withdrawal syndrome in Patient-A (left) and Patient-B (right).

Discussion

TSWS is an adverse effect of inappropriate prolonged use of TCS.^1–3 This case highlights severe TSWS in a mother and son who used copious amounts of high-potency TCS for a prolonged duration.

The main differential diagnosis for Patients-A and B is AD exacerbation. However, given their almost identical TSWS timelines, and the fact that they improved without TCS or other AD treatments, helps support the growing evidence that TSWS is its own entity, not simply a flare of the underlying dermatosis. This is also supported by the fact that Patient-B is still experiencing skin changes, but no accompanying pruritus or sleep disturbance, as would be expected with moderate-to-severe AD.

Patients-A and B likely experienced erytheroedematous TSWS, given their prominent erythema, stinging/burning, and edema, and lack of prominent pustules, papules, and nodules. ² This is consistent with a systematic review by Hajar et al., who found that 90% of those with erythematoedematous TSWS had primary eczematous conditions like AD. ²

In addition, both patients recovered well with TCS discontinuation. Hajar et al. ² found that TCS discontinuation and supportive care were the most commonly used treatments for TSWS.

Limitations of this report include the unattainability of exact prescriptions and dates, as both Patients-A and B used TCS for an extended duration while living in multiple different countries.

In conclusion, this report supports the notion that TSWS is its own entity, and describes two patients with nearly identical presentations of TSWS following the same prolonged high-potency TCS use and abrupt discontinuation. In addition, this report adds to the limited number of pediatric TSWS case reports. More research is needed to develop evidence-based TSWS diagnostic/treatment guidelines. Given that TSWS is an adverse effect of inappropriate, prolonged use of TCS,^1–3 more awareness about this condition is needed for prevention through safe prescribing practices.