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Home , Anakinra, Pityriasis rubra pilaris, Psoriasis, Psoriatic arthritis

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Discussion | Acrodermatitis continua of Hallopeau, also Additional Contributions: We thank the patient for granting permission to known as acrodermatitis perstans and repens, publish this information. is a rare inflammatory pustular dermatosis of the distal fin- 1. Saunier J, Debarbieux S, Jullien D, Garnier L, Dalle S, Thomas L. Acrodermatitis continua of Hallopeau treated successfully with gers and toes. It is considered a variant of pustular and after failure of tumour necrosis factor blockade and anakinra. or, less commonly, its own -like indepen- . 2015;230(2):97-100. 1 dent entity. Precise pathophysiology and incidence 2. Kiszewski AE, De Villa D, Scheibel I, Ricachnevsky N. An infant with are unknown. Case literature suggests predominance in acrodermatitis continua of Hallopeau: successful treatment with women, but the disease affects both and, rarely, and UVB therapy. Pediatr Dermatol. 2009;26(1):105-106. children.2 3. Jo SJ, Park JY, Yoon HS, Youn JI. Case of acrodermatitis continua accompanied by psoriatic . J Dermatol. 2006;33(11):787-791. Acrodermatitis continua of Hallopeau initially presents 4. Sehgal VN, Verma P, Sharma S, et al. Acrodermatitis continua of Hallopeau: as overlying the distal digits that evolves into evolution of treatment options. Int J Dermatol. 2011;50(10):1195-1211. pustules.2 The bed is often involved, with paronychial 5. Lutz V, Lipsker D. Acitretin- and inhibitor-resistant 3 and subungual involvement and atrophic skin changes. acrodermatitis continua of Hallopeau responsive to the 1 receptor Most patients experience a chronic, relapsing course involv- antagonist anakinra. Arch Dermatol. 2012;148(3):297-299. ing the proximal digit as the condition worsens.4 Acroder- 6. Iborra M, Beltrán B, Bastida G, Aguas M, Nos P. and matitis continua of Hallopeau has been reported to both -induced psoriasis in Crohn’s disease: a paradoxical side effect. J Crohns Colitis. 2011;5(2):157-161. evolve into and stem from generalized plaque or pustular psoriasis.3 The present patient was noted to have plaque psoriasis lesions nearly 1 year after the onset of her disease. Treatment of Refractory Rubra Pilaris is a rare ,3 but distal phalanx With Novel Phosphodiesterase 4 (PDE4) Inhibitor osteolysis is an important .1 The includes infectious paro- (PRP) is an inflammatory dermato- nychia of viral, fungal, or bacterial etiology, infected contact logic disorder of unknown cause characterized by hyper- dermatitis, and dyshidrotic eczema.4 Gram stain, potassium keratotic follicular combining into salmon-colored hydroxide mount, culture, and microscopy may be useful in scaling plaques, palmoplantar , and sharply diagnosis. Histopathologically, ACH is characterized by demarcated islands of spared skin.1 We report a case -rich spongiform pustules within the , of refractory PRP treated with the immune modulator dermal , and lymphohistiocytosis.4 As in pustular apremilast. psoriasis, from the nail bed often reveals acanthosis and spongiform pustules.3 Report of a Case | A white man in his 70s presented with an Treatment with topical , , fluo- 8-month history of scaling, pink, pruritic papules, originat- rouracil, , , acitretin, cyclosporine, and ing on his back (Figure, A), which coalesced to encompass phototherapy have produced inconsistent responses. Success- nearly his entire body surface. There was extensive ery- ful treatment with tumor necrosis factor inhibitors and the IL-1 thema with scaling and waxy, hyperkeratotic scaling of inhibitor anakinra5 have been reported. However, these agents the palms and soles. are not always efficacious and may even have the potential to A punch biopsy was performed for hematoxylin-eosin incite pustular psoriasis.6 staining and demonstrated alternating and Two cases of ACH treated with concomitant ustekinumab orthokeratosis with spongiosis and mild superficial lympho- and acitretin have been reported, one successfully,1 the other cytic infiltrate. Given these findings, PRP was diagnosed. unsuccessfully.5 The present case is the first to our knowl- The patient started acitretin therapy and showed initial im- edge to be successfully treated with ustekinumab as mono- provement, but after 4 months, his disease continued to prog- therapy. Given that the literature supports ustekinumab as ress. He was transitioned to methotrexate therapy with pred- monotherapy and concomitant therapy for pustular psoria- nisone bridging. However, the methotrexate regimen was sis, ustekinumab was a reasonable choice for our patient and discontinued after 8 weeks owing to lack of response and was succeeded when other agents had failed. replaced with cyclosporine. After 4 weeks of marginal re- sponse with cyclosporine and prednisone, infliximab was Rachel M. Cymerman, BA added, based on literature reports of improvement of PRP with David E. Cohen, MD tumor necrosis factor (TNF) inhibition.2,3 The patient showed marked improvement after 1 inflixi- Author Affiliations: The Ronald O. Perelman Department of Dermatology, mab infusion of 5mg/kg. Unfortunately, 4 weeks later, he was New York University School of Medicine, New York, New York. diagnosed with small-cell lymphocytic leukemia (SLL). Be- Corresponding Author: David E. Cohen, MD, The Ronald O. Perelman cause TNF inhibitors have been associated with an increased Department of Dermatology, New York University School of Medicine, 240 E risk of ,4 infliximab therapy was discontinued. The 38th St, 11th Floor, New York, NY 10016 ([email protected]). patient sought care for SLL, and and bendamus- Published Online: November 11, 2015. doi:10.1001/jamadermatol.2015.3444. tine was initiated. After completion of the Conflict of Interest Disclosures: Dr Cohen has consulted for Ferndale, chemotherapy, his PRP worsened. , and Medimetrics and owns stock or options in Dermira, Topica, and Medimetrics; none of these companies had any role in the present article. No The challenge was to identify a PRP-directed treatment other disclosures are reported. in a patient with refractory disease and contraindication to

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Figure. A Case of Refractory Pityriasis Rubra Pilaris Before and After 8 Months of Apremilast Therapy

A Before treatment B After 8 months of treatment

Almost complete resolution is seen on the back of this elderly male patient.

TNF inhibitors. After exhausting therapeutic options, we TNF inhibition, the choice of apremilast was a logical next considered apremilast (Otezla; Celgene Corporation). The step for the patient in the present case. The is appeal- patient started with a dose of 10 mg/d and titrated over ing owing to its minimal adverse effects and monitoring 5 days to the recommended maintenance dose of 30 mg requirements.6 At 12-month follow-up, the patient twice daily. remained disease free. Four weeks later, the patient reported significant Our experience suggests that apremilast may be an improvement in symptoms. He complained of mild gastro- effective treatment for refractory PRP. Additional studies intestinal upset but denied other new symptoms. The most are necessary to further establish the role of PDE4 inhibitors common adverse effects reported with apremilast are diar- as an option for refractory PRP. rhea, , and .5 At 8- and 12-week follow-ups, he reported further decreases in body surface area involve- Ifat Zerin Krase, PharmD ment of PRP. At 6- and 8-month follow-ups, he showed Kevin Cavanaugh, MD nearly complete resolution of skin findings (Figure, B). Clara Curiel-Lewandrowski, MD

Discussion | There is no unifying etiology to the pathogenesis Author Affiliations: University of Arizona College of Medicine, Tucson (Krase); Department of Dermatology, University of Arizona, Tucson (Cavanaugh, of PRP. Current treatments are mainly empirical or based on Curiel-Lewandrowski). case reports. Systemic and methotrexate are often Corresponding Author: Clara Curiel-Lewandrowski, MD, Department of used as first-line treatments. Other therapies include aza- Dermatology, University of Arizona, 1501 N Campbell Ave, Tucson, AZ 85724 thioprine, cyclosporine, , mycophenolate ([email protected]). mofetil, analogues, and phototherapy.1,2 Published Online: November 4, 2015. doi:10.1001/jamadermatol.2015.3405. Eastham et al3 demonstrated, in the most comprehen- Conflict of Interest Disclosures: None reported. sive analysis to date, that TNF inhibition is effective for re- Additional Contributions: We are indebted to Fangru Lian, MD, Department of fractory PRP. The patient in the present case responded well Pathology, University of Arizona, Tucson, for providing the histopathologic to infliximab but could not continue therapy because he analysis for the case. She did not receive any compensation for her developed SLL. Based on the proposed immune-driven contributions. mechanism of PRP, we explored novel treatments targeting 1. Klein A, Landthaler M, Karrer S. Pityriasis rubra pilaris: a review of diagnosis regulation of inflammatory responses. and treatment. Am J Clin Dermatol. 2010;11(3):157-170. Apremilast is an oral, small-molecule inhibitor of phos- 2. Müller H, Gattringer C, Zelger B, Höpfl R, Eisendle K. Infliximab monotherapy as first-line treatment for adult-onset pityriasis rubra pilaris: case report and phodiesterase 4 (PDE4) approved for treatment of moderate review of the literature on biologic therapy. J Am Acad Dermatol. 2008;59(5) 5 to severe plaque psoriasis and psoriatic arthritis. Expressed (suppl):S65-S70. in various cell types including , PDE4 partici- 3. Eastham AB, Femia AN, Qureshi A, Vleugels RA. Treatment options for pates in regulation of immune and inflammatory processes pityriasis rubra pilaris including biologic agents: a retrospective analysis from an by regulating intracellular cyclic adenosine monophosphate academic medical center. JAMA Dermatol. 2014;150(1):92-94. (cAMP) and downstream protein kinase A pathways and 4. Askling J, Fahrbach K, Nordstrom B, Ross S, Schmid CH, Symmons D. Cancer risk with tumor necrosis factor alpha (TNF) inhibitors: meta-analysis of phosphorylating CREB (transcription factor cAMP-response randomized controlled trials of adalimumab, , and infliximab using 6 element binding protein). Activation of this pathway patient level data. Pharmacoepidemiol Drug Saf. 2011;20(2):119-130. results in downstream inhibition of proinflammatory cyto- 5. Celgene Corporation. Otezla [package insert]. Summit, NJ: Celgene Corporation; kines, including TNF. Given our patient’s improvement with 2014.

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6. Palfreeman AC, McNamee KE, McCann FE. New developments in the Author Affiliations: Department of Anesthesiology, Louisiana State University management of psoriasis and psoriatic arthritis: a focus on apremilast. Drug Des Health Science Center, New Orleans. Devel Ther. 2013;7:201-210. Corresponding Author: Xiulu Ruan, MD, Department of Anesthesiology, Louisiana State University Health Science Center, 1542 Tulane Ave, New Orleans, LA 70112 ([email protected]). COMMENT & RESPONSE Conflict of Interest Disclosures: None reported. 1. Hill D, Feldman SR. Online reviews of physicians: valuable feedback, valuable Online Reviews of Physicians advertising [published online November 25, 2015]. JAMA Dermatol. 2015;1-2. Epub To the Editor The Editorial by Hill and colleagues1 is interest- ahead of print. doi:10.1001/jamadermatol.2015.3951. ing and informative. They suggest that online reviews are a fast, 2. Segal J. The role of the Internet in doctor performance rating. Pain Physician. efficient, transparent way to obtain actionable feedback from 2009;12(3):659-664. patients.1 However, we believe one of the major problems of 3. Schlesinger M, Grob R, Shaller D, et al. Taking patients’ narratives about physician online ratings is actually the lack of transparency. clinicians from anecdote to science. N Engl J Med. 2015;373(7):675-679. It is very difficult or even impossible to tell if the rater is a pa- 4. Gray BM, Vandergrift JL, Gao GG, McCullough JS, Lipner RS. Website ratings of physicians and their quality of care. JAMA Intern Med. 2015;175(2):291-293. tient or someone posing as a patient, such as an unhappy em- 5. Zgierska A, Rabago D, Miller MM. Impact of patient satisfaction ratings on ployee or a business competitor. Furthermore, even positive physicians and clinical care. Patient Prefer Adherence. 2014;8:437-446. ratings have limited utility; the origin of shining comments might be anonymous prose of the physician.2 Not to men- tion, most of the for-profit online rating sites often create a In Reply The problems with online reviews—few data, invalid handful of posts. The size of such a sample, compared with the instruments, lack of representativeness, and lack of transpar- number of patients that any given physician takes care of, lacks ency—are all predicated on physicians not actively encourag- any statistical significance. Thus, available online reviews of ing online reviews. Should physicians choose to participate, physicians lack accountability or quality control.2 large numbers of ratings can be obtained, validated instru- In a recent publication, Schlesinger and colleagues3 opined ments can be used, representativeness can be improved, and that “Most existing websites post comments submitted from the high quality of care that physicians provide can be made any source, with no assurance that they come from real pa- transparent to the public. tients. Clinicians’ legitimate concerns about accuracy and rep- Online reviews are not going to suddenly disappear. resentativeness may discourage them from using comments More and more, the public seeks out reviews for informa- for improving clinical practice.”3 They further opined that tion on physicians.1 By leaving only unhappy outliers to “Without active policy intervention, the pernicious influ- enter ratings, physicians can become victims of online ences of comments may outweigh the positive.”3 They em- reviews. Worse, by attempting to suppress physician rat- phasize that the key is to hold narrative data to the same stan- ings, physicians would look like they had something to dard of scientific rigor. Most importantly, there should be some hide. The alternative is for physicians to embrace online assurance that narratives are representative of patient reviews and encourage our patients to participate. One com- experience.3 pany, Medical Justice, that tried to help physicians by sup- Gray and colleagues4 conducted research to investigate the pressing online ratings recognized this was a huge mistake validity of physician website ratings by measuring the asso- and now encourages online rating.2 Having more represen- ciation between US physician website ratings and traditional tative reviews results in higher scores and dramatically quality measures of clinical and patient experience. They found diminishes the impact of outliers and fake reviews.3 no evidence that physician website ratings were associated with In-office kiosks, patient portals, and verification code sys- clinical quality measures. tems can help assure that reviews come from actual patients. Nevertheless, physician online ratings may have some se- Reminders at checkout or on patient instruction sheets can in- rious unintended consequences to physicians. Zgierska and clude instructions on how patients can leave online reviews. colleagues5 have suggested that patient satisfaction survey uti- Validated online measures are feasible, and people put more lization may promote job dissatisfaction, attrition, and inap- trust in recommendations from large numbers of reviewers.4,5 propriate clinical care among some physicians. Fifty-nine per- Some fake ratings may occur, but their effect would be cent reported that their compensation was linked to patient swamped by extensive patient participation; a few negative satisfaction ratings. Seventy-eight percent reported that pa- ratings may even increase patients’ perception that positive tient satisfaction surveys moderately or severely affected their ratings are valid. job satisfaction; 28% had considered quitting their job or leav- Sticking our heads in the sand and avoiding online ing the medical profession.5 reviews because of their limitations would be a self- With these problems in mind, we question the validity of fulfilling disaster for physicians and our patients. The public online reviews related to physicians and their performance and is online, and we would do well to use the internet to get raise a concern about the potential negative impact of this feedback from our patients and make that feedback trans- largely invalid pool of data related to physicians’ work. parent to help patients see the high quality of care that we provide. Xiulu Ruan, MD Greg J. Bordelon, MD Dane Hill, MD Alan David Kaye, MD, PhD Steven R. Feldman, MD, PhD

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