Letters Discussion | The results of this study reveal important differ- OBSERVATION ences in the microbiota of HS lesions in obese vs nonobese pa- tients. Gut flora alterations are seen in obese patients,4,5 and Successful Treatment of Refractory Pityriasis HS has been associated with obesity. It is possible that altered Rubra Pilaris With Secukinumab gut or skin flora could have a pathogenic role in HS. Pityriasis rubra pilaris (PRP) is a rare inflammatory skin dis- Some of the limitations of the present study include the order of unknown cause. It is characterized by follicular use of retrospective data and the lack of a control group con- hyperkeratosis, scaly erythematous plaques, palmoplantar sisting of patients with no history of HS. Although these cul- keratoderma, and frequent progression to generalized tures were obtained from purulence extruding from HS le- erythroderma.1 Six types of PRP are distinguished, with type sions, the bacterial culture results could represent skin or gut 1 being the most common form in adults. Disease manage- flora contamination. Information about the specific ana- ment of PRP is challenging for lack of specific guidelines. Topi- tomic locations of HS cultures was not available. Because only cal emollients, corticosteroids, and salicylic acid alone or com- the first recorded culture of each patient was analyzed, it is un- bined with systemic retinoids, methotrexate, and tumor known if the culture results would change with time and fur- necrosis factor (TNF) inhibitors are considered to be most ther antibiotic therapy. The use of data obtained from swab- helpful.2,3 Unfortunately, PRP often resists conventional treat- based cultures may also represent a potential limitation because ment. We report the case of a 67-year-old man with refrac- DNA-based approaches to microbial analysis may yield more tory PRP who was successfully treated with secukinumab. information and lead to identification of organisms that are not cultivable. The use of more advanced microbiome tech- Report of a Case |A 67-year-old man presented with an acute gen- niques may be an important consideration for future studies. eralized, erythematous and scaly eruption with spared patches These data indicate that further research is needed to eluci- of unaffected skin on his chest (Figure 1A). He also had pal- date the role of specific bacterial species in the pathogenesis moplantar keratoderma, nail dystrophy, and severe pruritus. of HS and may suggest a role for targeted treatment of spe- Skin biopsy confirmed the clinical diagnosis of PRP. Before on- cific bacterial species in this disorder. set of the PRP, he had not taken any new medication, and there was no evidence for allergies or chronic skin disorders in his Alessandra Haskin, BA personal or family history. Laboratory tests ruled out any in- Alexander H. Fischer, MPH fection, atopic disposition, autoimmune disease, or cancer. Ginette A. Okoye, MD Treatment was started with acitretin, 35 mg/d, correspond- ing to 0.5 mg/kg of body weight. In addition, acute flares were Author Affiliations: Ms Haskin was a student at Howard University College of treated with short-term systemic corticosteroid regimens com- Medicine, Washington, DC, at the time of the study. (Haskin); Department of bined with topical class IV corticosteroids. Dermatology, The Johns Hopkins School of Medicine, Baltimore, Maryland After 5 months of treatment, he still had severe pruritus and (Fischer, Okoye). presented with erythroderma, scaling, and palmoplantar kera- Accepted for Publication: May 26, 2016. toderma. Because there were several contraindications for con- Corresponding Author: Ginette A. Okoye, MD, Department of Dermatology, ventional immunosuppressive treatments, treatment was The Johns Hopkins School of Medicine, Johns Hopkins Bayview Medical Center, 5200 Eastern Ave, Ste 2500, Baltimore, MD 21224. switched to secukinumab, a monoclonal anti–interleukin(IL)- Published Online: July 20, 2016. doi:10.1001/jamadermatol.2016.2337. 17A antibody approved for the treatment of moderate to Author Contributions: Ms Haskin and Dr Okoye had full access to all the data in severe plaque psoriasis. After giving written informed con- the study and take responsibility for the integrity of the data and the accuracy sent, the patient received 2 subcutaneous injections of of the data analysis. secukinumab, 150 mg each, once a week for 5 weeks, followed Study concept and design: Haskin, Okoye. by monthly injections. Acquisition, analysis, or interpretation of data: All authors. Drafting of the manuscript: All authors. After 3 weeks of secukinumab treatment, the scaling Statistical analysis: Fischer. and pruritus were clearly reduced, and after 8 weeks, pruri- Conflict of Interest Disclosures: None reported. tus and erythema had completely cleared (Figure 1B). Corre- 1. Matusiak Ł, Bieniek A, Szepietowski JC. Bacteriology of hidradenitis spondingly, the typical histopathological features of PRP suppurativa: which antibiotics are the treatment of choice? Acta Derm Venereol. such as hyperplasia, acantholytic dyskeratosis, and hyper- 2014;94(6):699-702. keratosis (Figure 2A) had disappeared after 8 weeks of treat- 2. Lapins J, Jarstrand C, Emtestam L. Coagulase-negative staphylococci are the ment with secukinumab (Figure 2B). After 6 months, the most common bacteria found in cultures from the deep portions of hidradenitis suppurativa lesions, as obtained by carbon dioxide laser surgery. Br J Dermatol. palmoplantar keratoderma had also disappeared, and nail 1999;140(1):90-95. growth was normal. No clinical or laboratory adverse effects 3. Revuz JE, Canoui-Poitrine F, Wolkenstein P, et al. Prevalence and factors were registered. associated with hidradenitis suppurativa: results from two case-control studies. J Am Acad Dermatol. 2008;59(4):596-601. Discussion | The treatment options for PRP are mainly based on 4. Bervoets L, Van Hoorenbeeck K, Kortleven I, et al. Differences in gut clinical observations and are partly adopted from psoriasis microbiota composition between obese and lean children: a cross-sectional therapy because psoriasis shares some clinical and histopatho- study. Gut Pathog. 2013;5(1):10. logical features with PRP.4 In the last decade, various biologi- 5. Ferrer M, Ruiz A, Lanza F, et al. Microbiota from the distal guts of lean and obese adolescents exhibit partial functional redundancy besides clear cal therapies have improved the treatment options of psoria- differences in community structure. Environ Microbiol. 2013;15(1):211-226. sis. Some case reports have also reported successful treatment 1278 JAMA Dermatology November 2016 Volume 152, Number 11 (Reprinted) jamadermatology.com Copyright 2016 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 10/01/2021 Letters Figure 1. Clinical Images Before and After Secukinumab Treatment of Pityriasis Rubra Pilaris A Before treatment B After treatment A, At presentation. B, After 8 weeks of treatment with secukinumab. Figure 2. Hematoxylin-Eosin–Stained Histopathological Images of Punch Biopsy Specimens From Pityriasis Rubra Pilaris (PRP) Lesions on the Back A Before treatment B After treatment A, The typical histopathological features of PRP are seen, such as hyperplasia, acantholytic dyskeratosis, and hyperkeratosis (original magnification ×400). B, These features had disappeared after 8 weeks of treatment with secukinumab (original magnification ×400). of PRP with TNF and IL-12/23 antagonists.3,5 We demonstrate for Author Affiliations: Department of Dermatology, Medical Center–University of Freiburg, Freiburg, Germany. the first time to our knowledge that the IL-17A inhibitor secukinumab is effective in the treatment of severe, therapy- Corresponding Author: Christoph M. Schempp, MD, PhD, Department of Dermatology, Medical Center–University of Freiburg, Hauptstr 7, D-79104 refractory PRP. Secukinumab was approved for the treatment of Freiburg, Germany ([email protected]). 6 moderate to severe plaque psoriasis in 2015. In the present pa- Correction: This article was corrected on November23, 2016, to add omitted tient with PRP, skin alterations almost completely disappeared third author’s name in print versions. after 6 months of treatment. We cannot completely exclude the Published Online: October 5, 2016. doi:10.1001/jamadermatol.2016.3885 possibility that, at least in part, spontaneous remission of PRP oc- Conflict of Interest Disclosures: None reported. curred because up to 80% of type 1 PRP type 1 is self-limited with- Additional Contributions: We thank the patient for granting permission to out treatment within 3 years after onset.2 However, we started publish this information. treatment in a phase of severe, generalized PRP with acute exac- 1. Albert MR, Mackool BT. Pityriasis rubra pilaris. Int J Dermatol. 1999;38(1):1-11. erbations, and spontaneous healing is unlikely in this case. Fur- 2. Ross NA, Chung HJ, Li Q, Andrews JP, Keller MS, Uitto J. Epidemiologic, ther studies are required to evaluate the efficacy and safety of IL-17 clinicopathologic, diagnostic, and management challenges of pityriasis rubra antagonists in larger cohorts of patients with PRP. pilaris: a case series of 100 patients. JAMA Dermatol. 2016;152(6):670-675. 3. Petrof G, Almaani N, Archer CB, Griffiths WA, Smith CH. A systematic review of the literature on the treatment of pityriasis rubra pilaris type 1 with Daniel Schuster, MD TNF-antagonists. J Eur Acad Dermatol Venereol. 2013;27(1):e131-e135. Andrea