Inverse Psoriasis Involving Genital Skin Folds: Successful Therapy with Dapsone

Antonio Guglielmetti 0 Rodrigo Conlledo 0 Juliana Bedoya 0 Francisco Ianiszewski 0 Julio Correa 0 0 A. Guglielmetti R. Conlledo (&) J. Bedoya F. Ianiszewski J. Correa Department of Dermatology, University of Valparaso , Hontaneda 2653 Valparaso, Chile Introduction: Inverse psoriasis is a rare form of psoriasis that affects between 3% and 7% of the patients with psoriasis. It can comprise genital skin folds as part of genital psoriasis, and it is one of the most commonly seen dermatoses of this area. There are few evidence-based studies about the treatment of intertriginous psoriasis involving genital skin folds. Case Presentation: The authors present a 42-year-old female patient with erythematous plaques in the vulva, groin, and perianal region. The patient had previously received a broad range of topical and systemic therapies that had to be discontinued due to ineffectiveness or side - effects. She was treated with 100 mg dapsone daily for 10 months, showing a significant improvement of her cutaneous and mucous lesions. Complete clearance of psoriatic lesions was observed after 4 weeks of treatment. She has remained in remission for up to 2 years, using only topical therapy with tacrolimus 0.1% and calcipotriol. Discussion: Genital psoriasis is a skin disease that causes great discomfort. It is important to include examination of the genital region and to adopt this conduct in daily clinical practice. Research in this field is still poor, making no discrimination between flexural and genital psoriasis, and is based on case series and expert opinion; therefore, empirical recommendations for the treatment of genital psoriasis remain. Dapsone has been shown to be an effective and convenient alternative for the treatment of inverse psoriasis in genital skin folds, which can provide effective control of the disease. Further studies are required to determine the efficacy and safety of current therapies, and to decide whether dapsone therapy should be considered in the management of this form of psoriasis when topical and other systemic agents are not effective. Psoriasis is a chronic inflammatory skin disease involving the epidermis. It is characterized by scaly erythematous plaques and compromise of different body zones, usually with pruritus, and has a significant negative impact on quality of life [1]. The worldwide prevalence of psoriasis is estimated to be 2% [24]. Inverse psoriasis, also known as intertriginous or skin-fold psoriasis, is a form of psoriasis that presents itself as erythematous plaques with poor or nondesquamation in skin flexion folds [5]. It affects between 3% and 7% of the patients with psoriasis; however, the actual incidence is still unknown [6]. Inverse psoriasis can compromise genital skin folds as part of genital psoriasis, and it is one of the most commonly seen dermatoses of this area in both females and males [1]. In most cases, genital psoriasis can accompany plaque psoriasis lesions on other parts of the body, but it has also been reported as being isolated to the genital skin; this form of presentation is rare and occurs in only 25% of psoriatic patients [7, 8]. Local conditions of intertriginous areas, such as warmth, moisture, and friction, make it susceptible to maceration, fissuring, constant irritation, and absence of scaling, which induces the modified clinical appearance of psoriasis in flexion folds when compared with classical characteristics of psoriasis vulgaris. As a result, differential diagnosis with fungal and sexually transmitted diseases becomes difficult, and resistance to treatment is higher than in other skin zones. Treatment options are limited and difficult to determine because of the lack of evidence-based data, high sensitivity of the area involved, and increased penetration of topical treatments in this vulnerable zone, making it a challenge for the clinician. There are few evidence-based studies on the treatment of inverse psoriasis involving genital flexion folds, and data reported related to the efficacy and safety are, so far, extremely limited and only supported by expert opinion (level of evidence 5 and recommendation class D) [1]. The evidence-based recommendations suggest the use of weaker topical corticosteroids as a first-line treatment, and vitamin D preparations or tar-based treatments as second-line options [1]. There are reported cases that have used dapsone (Aczone , Allergan, Marlow, UK) as a treatment option, which has shown great effectiveness and complete remission after 4 weeks of treatment [9]. The case reported in this article is of a female patient with inverse and genital psoriasis successfully treated with dapsone. CASE PRESENTATION A 42-year-old female patient, without co-morbid conditions, presented with clinical manifestations of 2 years duration, characterized by extensive erythematous and exudative plaques in the groin and vulva, lately appearing intergluteal and perianal intertriginous plaques, and associated with secondary recurrent vulvovaginitis, which had an immensely negative impact on her quality of life and psychosexual wellbeing (Figs. 1, 2). The patient had previously received a broad range of topical and systemic therapies that had to be discontinued due to ineffectiveness or side effects. A genital skin biopsy was performed, with the results showing superficial interstitial psoriasiform spongiotic and perivascular Fig. 2 Intensive erythematous and exudative plaques in the vulvar area, associated with vaginal flow dermatitis, with signs of lichenification. The patient started treatment with 20 mg methotrexate weekly. Over the next few months the patient had irregular clinical progression with partial therapeutic response, and in addition, presented with recurrent episodes of urinary tract infections. After 4 months of treatment, the patient was changed to 500 mg mycophenolate mofetil (CellCept , Roche, Welwyn Garden City, UK) twice a day for 2 months, which induced slowly improving clinical results. Due to financial issues and after written informed consent from the patient was obtained, treatment was changed to 100 mg dapsone daily, which produced an excellent response and significant improvement, with complete clearance of cutaneous and mucous psoriatic lesions after 1 month of oral treatment, which was maintained over a period of 10 months of therapy. The patient has remained in remission for up to 2 years after treatment, using only topical therapy with tacrolimus 0.1% and calcipotriol (Figs. 3, 4). No adverse events were recorded. Scientific evidence shows that involvement of the genital skin occurs in 2940% of patients Fig. 3 Complete clearance of cutaneous and mucous psoriatic lesions 2 years after systemic therapy. The patient remained on topical treatment only (tacrolimus 0.1% and calcipotriol) Fig. 4 Perineal fold with complete clearance of psoriasis plaques 2 years after systemic therapy with psoriasis [1014]. When there is inverse psoriasis, the genita (...truncated)