Supplementary Online Content Ross NA, Chung H-J, Li Q, Andrews JP, Keller MS, Uitto J. Pityriasis rubra pilaris: a case series of patients. Published online March 9, 26. JAMA Dermatol. doi:./jamadermatol.26.9. etable. Demographics of the patients who entered the study with putative diagnosis of PRP, organized by the level of diagnostic certainty etable 2. Histopathologic features by the level of diagnostic certainty etable 3. Clinical differential diagnoses on biopsy request forms as listed by the submitting providers, by the level of diagnostic certainty etable 4. Depression and its relation to PRP, by the level of diagnostic certainty, as reported by the patients This supplementary material has been provided by the authors to give readers additional information about their work. 26 American Medical Association. All rights reserved. Downloaded From: on 6/9/28
Table S. Demographics of the patients who entered the study with putative diagnosis of PRP, organized by the Level of Diagnostic Certainty Demographic Data Level Level 2 Level 3 Level 4 TOTAL a n=5 (45 adult; 5 n=5 (2 adult; 3 n=3 (27 adult; 3 n=5 (4 adult; n= (88 adult; 2 Current Age, mean (range; median) 59 (5 to 84; 65) 53 (3 to 72; 62) 53 (7 to 82; 56) 62 (42 to 87; 6) 57 (5 to 87; 6) Female Gender n (%) 28 (56%) 6 (4%) (37%) (2%) 46 (46%) Race (n) White 44 (88%) 5 (5%) 29 (96%) 5 (%) 93 (93%) Black 3 (6%) (3%) 4 (4%) Other 2 (4%) 2 (%) No Response (2%) (%) Nations Represented 8 6 7 3 BMI b 28 (5 to 4; 27) - - - - Age at Symptom Onset (Adult c cases), mean (range; median), years 57 (25 to 8; 6) 59 (44 to 7; 6) 54 (2 to 78; 55) 5 (24 to 63; 57) 56 (2 to 8; 59) Age at Diagnosis (Adult c cases), mean, (range; median), years 58 (29 to 8; 6) 59 (44 to 7; 6) 55 (2 to 78; 56) 55 (43 to 63; 57) 57 (2 to 8; 59) Age at Symptom Onset (Pediatric d cases), mean (range; median), years 7 (2 to 3; 4) 7 (3 to ; 7) 5 (5 to 6; 5) d 7 (2 to 3; 5) Age at Diagnosis (Pediatric d cases), mean, (range; median) 6 (2 to 37; 4) (7 to 5; ) 5 (5 to 6; 5) 5 d 5 (2 to 5; 7) Correctly Diagnosed Upon Initial Presentation, n (%) 3 (26%) 6 (4%) (37%) 2 (4%) 32 (32%) Number of Biopsies Downloaded From: on 6/9/28
Reported by Patient 22 7 3 52 2 8 2 3 23 3 9 3 4 7 No Response 6 7 Time to Correct Diagnosis Among Those Initially Misdiagnosed, mean, (range; median), months 29 (.25 to 288; 2) 3 ( to 69; 5) 9 (.25 to 24; 2) 6 (2 to 2; 6) 28 (.25 to 288; 3) Adult Cases with Persistent Symptoms, n(%) of cohort Duration of Persistent Symptoms, Adult Cases, mean (range, median) Adult Cases with Resolved Symptoms, n(%) of cohort Time to Symptom Resolution, Adult Cases, mean (range, median) Pediatric Cases with Persistent Symptoms, n(%) of cohort 32 (64%) (67%) 23 (77%) 4 (8%) 69 (69%) 67 (2-384; 36) 2 (5-38; 7) 3 (2-8; 9) 67 (3-228; 9) 49 (2-384, 28) 3 (26%) 2 (3%) 4 (3%) 9 (9%) 33 (8-72; 36) 27 (24-3; 27) 29 (2-44; 3) - 32 (8-72; 3) 4 (8%) 2 (3%) 3 (%) (2%) (%) Duration of Persistent Symptoms, Pediatric Cases, mean (range, median) 24 (2-348; 96) 86 (5-56; 86) 68 (36-2; 48) 48 d (2-348; 8) Pediatric Cases with Resolved Symptoms, n(%) of cohort Resolved Symptoms, Pediatric Cases, mean (range, median) (2%) (7%) 2 (2%) 6 e 72 e - - 66 (6-72; 66) a Values represent sum totals of columns -4, except in the case of time associated metrics, expressed as mean (range plus median). b BMI, body mass index; data unavailable for patients in Levels 2-4. Downloaded From: on 6/9/28
Downloaded From: on 6/9/28 c Adult cases defined as those >9 years of age at the time of symptom onset. d Pediatric cases defined as those 9 years of age at the time of symptom onset. d Since n=, no range and median is given.
Table S2. Histopathologic features by the Level of Diagnostic Certainty a,b Features Reported Level Level 2 Level 3 Level 4 (n=5) (n=5) (n=3) (n=5) TOTAL Biopsies Analyzed 58 9 8 8 93 Number of Patients with 45 (9%) 4 (93%) 5 (7%) 5 (%) 69 (69%) Available Histopathology Report Epidermal Features, n(%) Parakeratosis 4 (82%) 2 (75%) 3 (%) 4 (8%) 6 (6%) Orthokeratosis 3 (62%) 4 (27%) 2 (7%) 37 (37%) Acanthosis 3 (6%) (67%) 2 (7%) 3 (6%) 45 (45%) Checkerboard Pattern 26 (52%) 3 (2%) 2 (7%) 3 (3%) Spongiosis 24 (48%) 7 (47%) 2 (7%) 2 (4%) 35 (35%) Hyperkeratosis 9 (38%) 7 (47%) (3%) (33%) 28 (28%) Psoriasiform Hyperplasia (22%) 2 (3%) (3%) 2 (4%) 6 (6%) Follicular Plugs 4 (28%) 2 (3%) (3%) 7 (7%) Acantholytic Dyskeratosis 4 (8%) 2 (7%) (33%) 7 (7%) Dermal Features, n(%) Alteration of Vasculature Alteration of Collagen Alteration of Elastin Inflammatory Patterns, n(%) Perivascular Epidermotropism Lichenoid Inflammatory Cells Present, n(%) Lymphocytes Histiocytes Eosinophils, rare Neutrophils Plasma cells, rare (2%) 3 (2%) 2 (7%) 5 (5%) 3 (6%) (7%) 4 (4%) 37 (74%) 2 (8%) 5 (7%) 5 (%) 59 (59%) 4 (8%) 3 (2%) (3%) 8 (8%) (2%) (3%) 2 (2%) 39 (78%) 2 (75%) 5 (7%) 5 (%) 6 (6%) 3 (26%) 4 (27%) 2 (7%) 2 (4%) 2 (2%) 5 (%) 3 (2%) (2%) 9 (9%) 4 (8%) 2 (3%) (2%) 7 (7%) (2%) (7%) (2%) 3 (3%) Downloaded From: on 6/9/28
a Percentages for each histopathologic feature are calculated based on cohort sizes and not based upon the number of biopsies examined. Histopathologic features were assumed to be absent unless specifically commented upon in the official histopathology reports b The format of the Table is adapted from Marrouche N et al. to present data in a manner that allows comparison between studies Downloaded From: on 6/9/28
Table S3. Clinical differential diagnoses on biopsy request forms as listed by the submitting providers, by the Level of Diagnostic Certainty Differential Diagnosis a Level Level 2 Level 3 Level 4 TOTAL n=5 n=5 n=3 n=5 n= Pityriasis Rubra Pilaris 2 7 2 2 3 Psoriasis 7 4 4 6 CTCL 6 2 9 Eczema 3 6 Drug Reaction 3 4 Pemphigus Foliaceus 2 PLEVA 2 PLC 2 2 Porokeratosis 2 2 Pityriasis Rosea 2 2 SCLE 2 Darier s Disease 2 2 Tinea Histiocytosis X Hypersensitivity Reaction Id Reaction Seborrheic Dermatitis a CTCL, Cutaneous T-Cell Lymphoma; PLEVA, Pityriasis Lichenoides et Varioliformis Acuta; PLC, Pityriasis Lichenoides Chronica; SCLE, Subacute Cutaneous Lupus Erythematosus Downloaded From: on 6/9/28
Table S4. Depression and its relation to PRP, by the Level of Diagnostic Certainty, as reported by the patients Level Level 2 Level 3 Level 4 TOTAL n=5 n=5 n=3 n=5 n= Frequency of Self-Reported Depression Always 2 (4%) 2 (2%) Often (22%) (7%) 2 (2%) Sometimes 5 (3%) 6 (4%) 6 (2%) (2%) 28 (28%) Rarely (2%) (7%) 5 (7%) (2%) 7 (7%) Never 6 (2%) (7%) (3%) (2%) 9 (9%) Do Not Know No Answer 6 (2%) 6 (4%) 8 (6%) 2 (4%) 32 (32%) Perceived Relation of Depression as Secondary to PRP % 8 (6%) (7%) 2 (7%) (2%) 2 (2%) -25% 4 (8%) (7%) 3 (%) (2%) 9 (9%) 26-5% 8 (6%) (7%) (3%) (2%) (%) 5-75% 5 (%) 3 (2%) 2 (7%) (%) 76-% 8 (36%) 3 (2%) 4 (3%) 25 (25%) Do Not Know No Answer 7 (4%) 6 (4%) 8 (6%) 2 (4%) 33 (33%) Downloaded From: on 6/9/28