U059 Hidradeni-s Suppura-va and Pityriasis Rubra Pilaris: Updates on Treatment DISCLOSURES I do not have any relevant relationships with industry. Scott Worswick UCLA Dermatology Director of Inpatient Dermatology 1
Pityriasis Rubra Pilaris: Differential Diagnoses, Cancer Associations and Expectations for Biologic Therapies Sco& Worswick Director of Inpa5ent Dermatology at UCLA Westwood 02-18- 2018 (special thanks to: Nolan Maloney, Lisa Hisaw)
What is your level of training 1- medical student 2- resident 3- outpa5ent dermatology a&ending 4- dermatology a&ending with significant inpa5ent experience 5- nurse prac5oner 6- other
Case #1 HPI: 53 y/o M with diffuse itchy rash slightly improved with betamethasone cream, medrol dose pack ROS: nega5ve PMH: works in educa5onal research, married Home medica5ons: MV, fish oil SH: lives in Los Angeles FH: son with atopy
Do you think this patient has 1.) Allergic Contact Dermatitis 2.) Drug Reaction 3.) Pityriasis Rubra Pilaris 4.) Follicular MF 5.) Ofuji s Syndrome 6.) Psoriasis 7.) Atopic Dermatitis
Histopathology Interface change Perivascular lymphocy5c infiltrate with sca&ered eosinophils
Clinical Course Pa5ent stopped vitamin and fish oil Resolu5on in 6 weeks
Drug Rashes Mimicking PRP (follicular prominence) Acneiform/folliculi5s Lichenoid Psoriasiform JW Byun et al. Lichenoid erup5on associated with an5tb drug: an unusual oral and follicular involvement in Am J Path 2014; 684-5. SC Shalin et al. Follicular mucinosisand MF- like drug erup5ons due to leuprolide: case report and review in J Cutan Path 2012; 1022-25.
Drugs that can trigger PRP Insulin Sorafenib Ima5nib Telaprivir vaccines T Badri et al. PRP- like erup5on following insulin therapy ini5a5on in Dermatol Pract Concept 2016; 19-21.
Sub- types of PRP Adult and pediatric Chronic and subacute Ichthyosiform/gene5c HIV- associated Griffiths WAD. Pityriasis Rubra Pilaris. Clin Exp Dermatol. 1980;5:105-112.
Case #2 HPI: 70 y/o M with chronic rash for two years dura5on (60% BSA) ROS: no weight changes, fevers or other PMH: rosacea Home medica5ons: none (but has tried and failed MTX, CsA and soriatane for this rash) SH: producer who lives in Los Angeles FH: no h/o rashes
Do you think this patient has 1.) Allergic Contact Dermatitis 2.) Drug Reaction 3.) Pityriasis Rubra Pilaris 4.) Follicular MF 5.) Ofuji s Syndrome 6.) Psoriasis 7.) Atopic Dermatitis
Clinical Course 4 SCCs in next year Trials of the following also fail: adalimumab, etanercept, apremilast, ustekinumab Be&er control with topicals only: tar- based shampoo QOD, triamcinolone 0.1% cream daily to body
What cancer has been linked to PRP in a paraneoplastic way? 1.) NHL 2.) Hodgkin s lymphoma 3.) renal cell carcinoma 4.) BCC 5.) melanoma What other cancer(s) has/have been diagnosed in pa5ents with exis5ng PRP?
What did we Sind when looking at NMSC and melanomas? 32 pa5ents in our cohort 2 antecedent tumors During on- going PRP: 2 pa5ents with SCC, 1 BCC, 1 melanoma Pa5ents with mul5ple NMSC during ac5ve PRP: 9 SCCs & BCCs in 1 pa5ent (acitre5n and MTX exposure) 2 SCCs in 1 pa5ent (no immune suppression) 4 SCCs in 1 pa5ent (aler CsA)
What systemic agent(s) have the most published data regarding efsicacy? a.) methotrexate b.) cyclosporine c.) re5noids D.) etanercept E.) ustekinumab
The treatment mainstays for Pityriasis Rubra Pilaris Topical steroids Isotre5noin/Acitre5n Phototherapy Methotrexate Cyclosporine DS Allison et al. PRP in children in JAAD; 47 (3): 386-9. CH Dicken. Treatment of classic PRP in JAAD 1994; 31:997-9. Klein A, Landthaler M, Karrer S. Pityriasis Rubra Pilaris: A Review of Diagnosis and Treatment. Am J Clin Dermatol. 2010;11(3):157-170.
Walling HW, Swick BL. PRP responding rapidly to adalimumab. Arch Dermatol. 2009;145(1):99-101. Petrof G et al.. A systema5c review of the literature on the treatment of PRP type 1 with TNF- antagonists. J Eur Acad Dermatology Venereol. 2013;27(1):131-135. Eastham AB et al.. Treatment op5ons for pityriasis rubra pilaris including biologic agents: a retrospec5ve analysis from an academic medical center. JAMA Dermatology. 2014;150(1):92-94. Garcovich S et al. Treatment of refractory adult- onset pityriasis rubra pilaris with TNF- alpha antagonists: a case series. J Eur Acad Dermatology Venereol. 2010;24(8):881-884. Chowdhary M et al. Ustekinumab as an alterna5ve treatment op5on for chronic PRP. Case Rep Dermatol. 2015;7(1):46-50. Schuster D, Pfister- Wartha A, Bruckner- Tuderman L, Schempp CM. Successful treatment of refractory PRPwith secukinumab. JAMA Dermatology. 2016;152(11):1278-1279. What to try in refractory cases? TNF- blockers Apremilast IL- 12/IL- 23 blockade IL- 17 inhibitors
Our review
Counter- Point: TNF- Inhibitors and IL- 12/23 Blockade at UCLA
My Treatment Ladder if Choosing Systemic Therapy for PRP Re5noids Etanercept ustekinumab
So is apremilast worth trying?
Learning Points: PRP Don t forget about drug reac5ons: acneiform, lichenoid, psoriasiform Also remember exposures that can triggers PRP: vaccines, HIV, insulin, sorafenib, ima5nib PRP in kids can be ichthyosiform and chronic form in adults psoriasiform or eczematous Remember cancer associa5ons: renal cell carcinoma, SCC? Most effec5ve treatments: re5noids, etanercept, ustekinumab