Clinical Cases in Psoriasis

This updated volume provides a guide to the diagnosis and management of psoriasis. The book examines clinical cases to help the reader work through unusual cases using best practice techniques. New chapters on erythrodermic psoriasis, severe pediatric psoriasis, psoriasis with patients with prior breast cancer, and psoriasis with patients who wish to become pregnant have been added. The treatment of psoriasis in relation to patients with hepatitis B and C, HIV, and who have received pneumococcal and herpes zoster vaccines are also discussed.

Clinical Cases in Psoriasis highlights evidence-based best practice through its multidisciplinary approach and is relevant to trainees and clinicians working within dermatology.

• Language: English
• Publisher: Springer
• Release date: Jul 26, 2019
• ISBN: 9783030187729

Book preview

© Springer Nature Switzerland AG 2019

Jashin J. Wu (ed.)Clinical Cases in PsoriasisClinical Cases in Dermatologyhttps://doi.org/10.1007/978-3-030-18772-9_1

1. 34-Year-Old with Widespread Redness and Scaly Skin

Michael P. Lee¹, Kevin K. Wu² and Jashin J. Wu³  

(1)

Eastern Virginia Medical School, Norfolk, VA, USA

(2)

Frank H. Netter MD School of Medicine at Quinnipiac University, North Haven, CT, USA

(3)

Founder and CEO, Dermatology Research and Education Foundation, Irvine, CA, USA

Jashin J. Wu

Keywords

Erythrodermic psoriasisPsoriatic arthritisCyclosporine

Case

A 34-year-old male, with a history of scaly plaques since 18-years-old, presented with generalized erythema and fine scaling of his skin. He had been on adalimumab for almost a year with suboptimal results. The patient had previously failed treatment with etanercept and methotrexate 20 mg weekly used in conjunction with other biologics. Treatment with infliximab appeared to be most effective for 5 years, but lost efficacy towards the end. Phototherapy for the patient was not attempted due to scheduling issues with his work. He also endorsed hand and right knee pain in the mornings. Patient had never had a biopsy performed nor did he have any history of malignancy. He is a former smoker and drinks alcohol socially.

On physical examination, face, neck, chest, abdomen, back, arms, legs, buttocks, and groin had well-defined erythematous scaly plaques. His palms and soles were clear. The affected body surface area was 95%.

Based on the clinical case description, what is the most likely diagnosis?

1.

Atopic dermatitis

2.

Pityriasis rubra pilaris

3.

Erythrodermic psoriasis

4.

Seborrheic dermatitis

5.

Cutaneous T cell lymphoma

Diagnosis

Erythrodermic psoriasis

Discussion

Erythrodermic psoriasis (EP) is a rare, life-threatening variant of psoriasis characterized by widespread redness, itching, pain, and scaly skin. It is the least common subtype of psoriasis occurring in less than 3% of patients, has a variable age of onset, and appears to favor males [1, 2]. Erythrodermic psoriasis typically encompasses more the 75% of the total body surface area and can also be associated with pustules and exfoliation of skin, resulting in loss of its many protective functions. Symptoms may develop rapidly over several days or gradually over weeks. With impaired skin function, patients with EP may experience dehydration, infections, hypothermia, and death from sepsis if not properly treated [3].

The pathogenesis of erythrodermic psoriasis is still under investigation. There is evidence, however, pointing to a prominent role of T lymphocytes interactions with macrophages, and it has been demonstrated that the ratio of Th1/Th2 response was significantly lower in EP compared to patients with psoriasis [4]. Patients with EP were also found to have significantly higher levels of interleukin-4 and interleukin-10 compared to both psoriasis patients and healthy controls.

It is imperative to gather a detailed history and perform a thorough exam in order diagnosis EP and initiate appropriate management. EP should be considered when a patient presents with erythema of 75% or more of body surface area involvement and skin features of psoriasis with other causes of erythroderma less likely. Patient history will usually reveal personal or family history of psoriasis, and exam may demonstrate other features consistent with psoriasis including plaques, arthralgias, and nail disease. Skin biopsy will most frequently show histological features supporting psoriasis such as hyper- and parakeratosis along with a thickened stratum spongiosum layer and reduced granular layer.

Treatment

Due to the uncommon nature of EP and limited high quality studies for disease management, treatment recommendations are derived mostly from case reports and case series. Initial management typically involves correcting any fluid or electrolyte abnormalities, treatment of infections, and hypothermia prophylaxis. After stabilization, the US National Psoriasis Foundation has advocated use of systemic medications as first line therapies including cyclosporine or infliximab. Both of these medications have demonstrated rapid onset of action for EP [5]. Other appropriate therapies include methotrexate and acitretin, although these medications typically work slower.

Cyclosporine is a calcineurin inhibitor that blocks T-cell activation by preventing transcription of interleukin-2. Due to its rapid onset of action, it is recommended for unstable cases of EP and is given orally in doses of 4–5 mg/kg per day until remission. A case series of 33 subjects demonstrated that 67% of patients had complete remission within 3 months of using cyclosporine [6]. Infliximab, a soluble anti-TNF-alpha antibody, also boasts a rapid onset of action that leads to decreased T-cell infiltration in erythrodermic psoriasis. It is given as the standard regimen for psoriasis where a 5 mg/kg dose is administered at 0, 2, 6 weeks, followed by every 8 weeks. An open-label, single site study showed 40% of patients achieving a 90% reduction in PASI and 20% of patients achieving a 75% reduction in PASI at 14 weeks of treatment [7]. Combination treatment using one of these systemic medications along with a topical therapy has been shown to be effective and is also frequently necessary to achieve remission [5].

Methotrexate and acitretin can also be used as alternative first line therapies, although they have demonstrated a slower onset of action. Methotrexate is usually administered on a weekly schedule with folic acid supplementation. A retrospective study showed a good response in 94% of patients with EP [8]. Acitretin, a vitamin A derivative, is typically given in doses of 25 to 50 mg per day, but effect is not apparent for at least 3 months [9]. Newer biologic medications including ustekinumab, ixekizumab, and secukinumab for the treatment of erythrodermic psoriasis show promising preliminary results [10–12]. Particularly ustekinumab has been demonstrated to be safe and associated with significant improvements of Psoriasis Area and Severity Index scores after 4 weeks of therapy [13]. However, data is currently limited to small studies and case reports and further investigation is needed before routine recommendation of use.

There are no head to head comparisons of the recommended rapid onset first line therapies. Treatments should be selected on an individual basis, taking into consideration side effect profiles. Cyclosporine commonly causes nephrotoxicity and hypertension, and infliximab is contraindicated during active infections. While treatment options for EP have greatly increased in the past several years, there is a need for additional controlled trials to better characterize the pathology of the disease and understand the role of each medication.

The patient from the clinical scenario was started on cyclosporine along with clobetasol topical spray and tacrolimus ointment for affected areas. Patient was counseled on risks of cyclosporine use including increased risk of lymphoma, cancer recurrence, hypertension, and impaired kidney function. He was to follow up in 1 month and was also referred to rheumatology to evaluate for possible psoriatic arthritis.

Key Points

Erythrodermic psoriasis is a rare, life-threatening variant of psoriasis that presents with generalized erythema, itching, pain, and scaly skin. It typically involves greater than 75% of surface body area.

Due to disruption of a large area of the protective skin barrier, patients with EP are prone to dehydration, electrolyte abnormalities, impaired thermoregulation, and infections.

After stabilization and correction of any associated complications, first line treatments for EP include cyclosporine or infliximab, as they act rapidly to treat the disease. Acitretin and methotrexate can also be considered, although they have a slower onset of action.

References

1.

Boyd AS, Menter A. Erythrodermic psoriasis. Precipitating factors, course, and prognosis in 50 patients. J Am Acad Dermatol. 1989;21(5 Pt 1):985–91.Crossref

2.

Lebwohl M. Psoriasis. Lancet. 2003;361(9364):1197–204. https://​doi.​org/​10.​1016/​S0140-6736(03)12954-6.CrossrefPubMed

3.

Green MS, Prystowsky JH, Cohen SR, Cohen JI, Lebwohl MG. Infectious complications of erythrodermic psoriasis. J Am Acad Dermatol. 1996;34(5. Pt 2):911–4.Crossref

4.

Zhang P, Chen HX, Duan YQ, Wang WZ, Zhang TZ, Li JW, Tu YT. Analysis of Th1/Th2 response pattern for erythrodermic psoriasis. J Huazhong Univ Sci Technolog Med Sci. 2014;34(4):596–601. https://​doi.​org/​10.​1007/​s11596-014-1322-0.CrossrefPubMed

5.

Rosenbach M, Hsu S, Korman NJ, Lebwohl MG, Young M, Bebo BF Jr, Van Voorhees AS, National Psoriasis Foundation Medical Board. Treatment of erythrodermic psoriasis: from the medical board of the National Psoriasis Foundation. J Am Acad Dermatol. 2010;62(4):655–62. https://​doi.​org/​10.​1016/​j.​jaad.​2009.​05.​048.CrossrefPubMed

6.

Management of erythrodermic psoriasis with low-dose cyclosporin. Studio Italiano Multicentrico nella Psoriasi (SIMPSO). Dermatology. 1993;187(Suppl 1):30–7. https://​doi.​org/​10.​1159/​000247289.Crossref

7.

Poulalhon N, Begon E, Lebbe C, Liote F, Lahfa M, Bengoufa D, Morel P, Dubertret L, Bachelez H. A follow-up study in 28 patients treated with infliximab for severe recalcitrant psoriasis: evidence for efficacy and high incidence of biological autoimmunity. Br J Dermatol. 2007;156(2):329–36. https://​doi.​org/​10.​1111/​j.​1365-2133.​2006.​07639.​x.CrossrefPubMed

8.

Haustein UF, Rytter M. Methotrexate in psoriasis: 26 years’ experience with low-dose long-term treatment. J Eur Acad Dermatol Venereol. 2000;14(5):382–8.Crossref

9.

Menter A, Korman NJ, Elmets CA, Feldman SR, Gelfand JM, Gordon KB, Gottlieb AB, Koo JY, Lebwohl M, Lim HW, Van Voorhees AS, Beutner KR, Bhushan R. Guidelines of care for the management of psoriasis and psoriatic arthritis: section 4. Guidelines of care for the management and treatment of psoriasis with traditional systemic agents. J Am Acad Dermatol. 2009;61(3):451–85. https://​doi.​org/​10.​1016/​j.​jaad.​2009.​03.​027.CrossrefPubMed

10.

Pescitelli L, Dini V, Gisondi P, Loconsole F, Piaserico S, Piccirillo A, Stinco G, Errichetti E, Talamonti M, Tripo L, Volpi W, Prignano F. Erythrodermic psoriasis treated with ustekinumab: an Italian multicenter retrospective analysis. J Dermatol Sci. 2015;78(2):149–51. https://​doi.​org/​10.​1016/​j.​jdermsci.​2015.​01.​005.CrossrefPubMed

11.

Saeki H, Nakagawa H, Ishii T, Morisaki Y, Aoki T, Berclaz PY, Heffernan M. Efficacy and safety of open-label ixekizumab treatment in Japanese patients with moderate-to-severe plaque psoriasis, erythrodermic psoriasis and generalized pustular psoriasis. J Eur Acad Dermatol Venereol. 2015;29(6):1148–55. https://​doi.​org/​10.​1111/​jdv.​12773.CrossrefPubMed

12.

Weng HJ, Wang TS, Tsai TF. Clinical experience of secukinumab in the treatment of erythrodermic psoriasis: a case series. Br J Dermatol. 2018;178(6):1439–40. https://​doi.​org/​10.​1111/​bjd.​16252.CrossrefPubMed

13.

Saraceno R, Talamonti M, Galluzzo M, Chiricozzi A, Costanzo A, Chimenti S. Ustekinumab treatment of erythrodermic psoriasis occurring after physical stress: a report of two cases. Case Rep Dermatol. 2013;5(3):254–9. https://​doi.​org/​10.​1159/​000348645.CrossrefPubMedPubMedCentral

© Springer Nature Switzerland AG 2019

Jashin J. Wu (ed.)Clinical Cases in PsoriasisClinical Cases in Dermatologyhttps://doi.org/10.1007/978-3-030-18772-9_2

2. 13-Year-Old with Red, Scaly Rash

Michael P. Lee¹, Kevin K. Wu² and Jashin J. Wu³  

(1)

Eastern Virginia Medical School, Norfolk, VA, USA

(2)

Frank H. Netter MD School of Medicine at Quinnipiac University, North Haven, CT, USA

(3)

Founder and CEO, Dermatology Research and Education Foundation, Irvine, CA, USA

Jashin J. Wu

Keywords

Pediatric psoriasisTopicalsCorticosteroidsVitamin D analog

Case

13-year-old female presented with red, scaly rash for several years. Patient had previously been prescribed topical creams with mild improvement of symptoms. She denied any joint stiffness or pain. Patient had a significant family history of psoriasis in her paternal uncles and aunts. She was brought in to clinic by her mother and is currently in eighth grade. With the exception of obesity, the patient was otherwise healthy and denied any recent illness, sore throat, or sick contacts.

On physical examination, erythematous scaly papules and plaques were visualized on scalp, face, neck, chest, abdomen, extremities, and back. Patient deferred exam of breasts, groin, buttocks, and bilateral upper hips. The lesions appeared worse on the back and greater than 10% of the body surface area