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This information is intended for US healthcare professionals to access current scientific information about J&J Innovative Medicine products. It is prepared by Medical Information and is not intended for promotional purposes, nor to provide medical advice.

STELARA® (ustekinumab)

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Treatment of Pityriasis Rubra Pilaris with STELARA

Last Updated: 12/27/2024

Summary

The company cannot recommend any practices, procedure, or usage that deviate from the approved labeling.
A retrospective study, case series, and case reports have described the use of STELARA in patients with pityriasis rubra pilaris (PRP).¹^-³⁰

RETROSPECTIVE STUDY

Maloney et al (2017)¹ conducted a retrospective review of cases of PRP treated at their institution. Seven of the 16 patients with a clear diagnosis of PRP were treated with biologics. Two of the 7 biologic treated patients received STELARA and are described below.

Patient 1

The patient was a 67-year-old female diagnosed with type I PRP, who was intolerant of treatment with acitretin 25 mg/day which was discontinued after 2 weeks.
After failing to show improvement with different regimens of monotherapy with adalimumab and etanercept for 5 months total, she experienced improvement in erythema and pruritus within 4 weeks of the initial dose of STELARA 90 mg.
An additional dose of STELARA 45 mg was administered at week 4.
At week 12, significant reductions in scaling and flattening of lesions with a slight decrease in involved body surface area (BSA) was observed.
At 6 months, the patient continued to show improvement.
The patient’s clinical response to STELARA treatment was considered partial, defined as improvement in erythema, scaling, and/or palmoplantar keratoderma representing a less than 75% improvement in terms of affected BSA from baseline.

Patient 2

The patient was a 68-year-old male diagnosed with type I PRP, who had a partial response (<75% improvement from baseline in terms of affected BSA) to 6 months of treatment with acitretin 25 mg/day, and acitretin 25 mg every other day, but a relapse occurred during the taper and the patient didn’t see improvement after returning to the original dose. In addition, the patient had no response to 16 weeks of treatment with methotrexate (MTX) 15-20 mg/week, had a partial response to 8 weeks of cyclosporine A 300 mg/day, and had no response to 15 weeks of apremilast 30 mg twice daily.
After failing to respond to treatment with adalimumab 40 mg every other week for 12 weeks, he received STELARA 90 mg at week 0 followed by 45 mg at week 8.
He failed to respond to treatment with STELARA, defined as disease that did not improve from baseline or worsened over the course of treatment.

case series

Pham et al (2022)² reported a case series of 2 patients with refractory type 1 PRP.

Two male patients (patient 1 aged 69 years, patient 2 aged 63 years) presented with progressive papulosquamous erythroderma and palmoplantar keratoderma.
- Patient 1 presented with orange-red erythroderma with islands of sparing and waxy palmoplantar keratoderma, developing over 4 weeks. Checkerboard parakeratosis, follicular plugging, and dermal perivascular infiltrate were observed. Prior therapies included topical corticosteroid wet dressing, oral and subcutaneous (SC) MTX, oral prednisolone, acitretin, and cyclosporine.
- Patient 2 presented with progressive scaling erythroderma with island of sparing and palmoplantar keratoderma, developing over 4-6 months and associated with significant pruritus. Patient also had a secondary congestive cardiac failure due to erythroderma. Mild acanthosis with thick suprapapillary plate, mildly lengthened rete ridges, checkerboard parakeratosis, patchy basal vacuolization, and mild superficial dermal lymphocytic/histiocytic inflammation with occasional eosinophils and neutrophils were observed. Prior therapies included topical corticosteroid wet dressing, oral prednisolone, acitretin, and cyclosporine.
STELARA was initiated for both patients at 15 months and 14 months after initial onset of PRP, respectively.
- Patient 1 received STELARA 45 mg SC at week 0, 4, 12 then every 12 weeks thereafter and reported 50% improvement in cutaneous PRP involvement within 4 weeks. Complete remission was reported at 6 months, which remained at 12 months.
- Patient 2 received STELARA 90 mg SC at week 0, 4, 12 then every 12 weeks thereafter and significant improvement of erythroderma was reported within 4 weeks. Complete clearance of body involvement was reported at 6 months. Supplemental topical therapy and topical psoralen plus ultraviolet A (PUVA) phototherapy were also utilized for palmoplantar keratoderma. Guselkumab was later initiated, replacing STELARA therapy, due to recrudescence of palmoplantar keratoderma at 12 months, along with reduced effect at the 8 week mark for erythrodermic involvement. Complete resolution of palmoplantar keratoderma was reported and sustained at 12 months.
No significant treatment-related adverse events were reported during either STELARA or guselkumab therapy.

de Brito et al (2021)³ reported 3 cases of refractory type I PRP with complete response to STELARA.

A 57-year-old female presented with a pruritic psoriasiform eruption that responded partially to treatment with retinoid or MTX. The eruption became widespread, with spared islands and palmoplantar keratoderma. The patient developed erythroderma, requiring hospitalization regardless of treatment with acitretin and cyclosporine and failed treatment with azathioprine, systemic steroids, and erythromycin. After the first STELARA injection, her skin showed signs of improvement and remained clear.
A 61-year-old male presented with a widespread scaling eruption diagnosed as type I PRP and could not receive MTX due to alcohol intake. Acitretin significantly impaired his quality of life (Dermatology Life Quality Index [DLQI], 16). The patient became erythrodermic. Following the first STELARA injection, his erythema reduced by 50%, and by the fourth STELARA injection, his skin was clear. STELARA was discontinued after 18 months, and his skin remained clear.
A 64-year-old female presented with type I PRP, with erythroderma, nail changes, orange keratoderma, and spared islands over the trunk, requiring hospitalization. No improvement was observed despite treatment with acitretin, MTX, cyclosporine, and infliximab. Four weeks after the first STELARA injection, her skin was almost clear and her DLQI reduced from 25 to 5. STELARA was discontinued after 6 months and her skin remained clear.

Lwin et al (2018)⁴ reported a mother and son with PRP due to a new missense mutation in caspase recruitment domain-containing protein 14 (CARD14) and described the response to STELARA in both.

The 49-year-old mother presented with widespread erythema and scales affecting the face, body, and limbs with a few islands of sparing. She had developed erythematous patches on both cheeks around age 6 weeks, followed by more generalized dry red scales with peeling that persisted into adulthood. She also had minor nail ridging and mild palmoplantar keratoderma.
The 20-year-old son had a similar clinical course beginning in his first few weeks of life, although his skin developed more patchy, serpiginous, dry, and thick scales with associated erythema.
A diagnosis of autosomal dominant familial PRP was made and treatment with oral retinoids, phototherapy, and topical steroids was ineffective in both patients.
Sanger sequencing of CARD14 was performed in both mother and son, as well as all unaffected members of the family. This revealed a heterozygous single-nucleotide transversion c.356T>G in exon 4, resulting in the missense mutation p.Met119Arg in both the affected mother and son but not in the other unaffected relatives.
Both mother (weight: 66 kg) and son (weight: 65 kg) were treated with STELARA 45 mg SC at weeks 0 and 4, then every 12 weeks.
Psoriasis Area and Severity Index (PASI) and DLQI were assessed for the mother and son at baseline and at 12 and 24 weeks after initiation of STELARA.
- At baseline the mother had a PASI of 25.7 and DLQI of 18, and the son had a PASI of 29.2 and DLQI of 22.
- Clinical improvement was observed within a few days of the initial loading dose and at 12 weeks the mother had a PASI <1 and DLQI of 0, while the son had a PASI of 4.8 and DLQI of 3, which persisted for a total of 24 weeks.
The clinical benefit in the mother waned around 30 weeks after initiation of STELARA but improved again after an increase in the STELARA dose from 45 mg to 90 mg every 12 weeks.
Approximately 12 weeks after the increase in STELARA dose the mother experienced normalization of fingernails symptoms.
Improvement was maintained in both mother and son beyond 8 months on treatment.

Napolitano et al (2018)⁵ evaluated the efficacy of STELARA in the treatment of patients with type I or II PRP who were refractory to conventional therapies. The primary endpoint was the clearance of skin lesions following STELARA treatment.

Five patients with adult-onset PRP were identified, 4 with type I and 1 with type II.
All patients were resistant to treatment with either MTX, cyclosporine A, acitretin, and/or topical glucocorticoids that were discontinued.
Patients were treated with STELARA 45 mg SC at weeks 0, 4, and 16.
Clinical response was evaluated monthly and up to a 15-month follow-up period.
Concomitant systemic therapy was allowed after the third dose of STELARA at week 16 if there was an absence of response. No topical treatments other than topical hydrating cream were allowed.
All patients showed erythroderma/suberythroderma, with typical small islands of spared skin, and palmoplantar keratoderma.
Two patients (cases 1 and 4) also showed nail dystrophy with onycholysis.
The type II PRP patient (case 2) showed marked ichthyosiform scaling of the face, neck, and scalp, and generalized erythroderma with eczematous areas.
An improvement of skin lesions, with a decrease in erythema, follicular hyperkeratosis, and scaling, and a mild improvement of palmoplantar keratoderma, was observed in all patients starting 4 weeks after the initial dose of STELARA.
Between the second and third dose of STELARA (weeks 4-8), 4 of 5 patients (cases 1-3 and 5) showed complete clearance of skin lesions, while case 4 showed partial improvement of skin lesions.
At the 13-15-month follow-up, no significant relapse was observed in cases 1-3 and 5 and none of these 4 patients required concurrent PRP therapies.
Case 4 required concomitant therapy with acitretin 0.2 mg/kg/day for 6 weeks starting at week 16 as palmoplantar lesions persisted after the second dose of STELARA.
No significant side effects occurred during treatment or during the follow-up period.

CASE REPORTS

Case Reports - Treatment of PRP

Publication	Patient	Case Description
Reitmajer et al (2023)⁶	A nearly 3-year-old male presented with a 4-week history of progressive follicular papules, scaly red-orange patches, and palmoplantar hyperkeratosis developing into plaques with rhagades. Initially the hands and face were affected, but the disease spread to the neck, scalp, back, and extremities. The patient complained of itching and significant pain due to the rhagades, impacting his sleep and hand mobility. Growth and psychomotor development were normal, with no syndromal involvement. A skin biopsy from the affected back area revealed plump to psoriasiform acanthosis, alternating orthokeratosis and parakeratosis in both vertical and horizontal directions, and a sparse superficial perivascular infiltration of lymphocytes, consistent with the diagnosis of PRP.	The initial treatment with topical glucocorticoids and calcipotriol showed no improvement, and the condition rapidly worsened. Following increased skin involvement and worsening pain, weight-based STELARA therapy was initiated. A noticeable improvement was observed in the first follow-up after 12 weeks. The patient tested negative for the CARD14 mutation, commonly seen in juvenile PRP subtypes. At the 1-year follow-up, a lasting response was noted with no reported side effects. No concerns regarding the quality of life were observed.
Gamonal et al (2022)⁷	A 47-year-old male presented with scaly reddish plaques on the scalp, back, chest, and limbs 15 days after a booster with the BNT162b2 COVID-19 vaccine (Pfizer-BioNtech) in October 2021. Patient had received 2 doses of CoronaVac (Sinovac Biotech) in March 2021 without intercurrences. Type I PRP was diagnosed after a skin biopsy revealed perifollicular infiltrate, psoriasiform hyperplasia with confluent parakeratosis, decreased granular layer, and lymphohistiocytic perivascular and periadnexyal infiltrate in the papillary dermis. Patient’s symptoms worsened after initial treatment of secukinumab. Dermatological examination revealed a rash involving 90% of BSA with islands of sparing on neck and legs along with palmar-plantar hyperkeratosis, onychodystrophy, and ectropion. Patient was erythrodermic and had scalp involvement with scales, as well as erythema in the genital region. The nail involvement was posterior with palmoplantar keratoderma.	Prior to the biopsy, patient was prescribed prednisolone 40 mg/day for 15 days. Secukinumab was initiated with insufficient response after 8-10 weeks. STELARA 90 mg SC at weeks 0, 4, and then every 12 weeks and acitretin 25 mg/day were initiated. Gradual improvement was noted after the second dose of STELARA. No safety concerns were noted.
Katharina et al (2022)⁸	A 5-year-old female developed koebnerizing psoriasiform skin lesions, palmoplantar hyperkeratosis, and fatigue after a respiratory tract infection. Patient was initially diagnosed with infection-triggered psoriasis and treatment was initiated. Type III PRP (clinical juvenile) was diagnosed after patient presented with worsened palmoplantar keratoderma, multiple follicular, hyperkeratotic papules coalescing into scaly, orange-red plaques on her scalp, trunk, and extremities, and islands of sparing.	Infection-triggered psoriasis was initially treated with topical glucocorticoids, systemic antibiotics, and adalimumab 40 mg at week 0 and 20 mg SC every other week. After the patient was diagnosed with type III PRP, STELARA 0.75 mg/kg was initiated. Skin involvement improved after 8 weeks. STELARA dosage was then increased to 1.5 mg/kg, used concomitantly with acitretin 10 mg 2 times a week, which yielded symptom improvement with no reported adverse effects.
DeBiasio et al (2022)⁹	A 58-year-old female developed extensive erythematous scaly eruption, starting on her face and scalp, and spreading to her torso and limbs. She had type II skin with a symmetrical rash consisting of bright red scaly plaques on the face, torso, and limbs, with follicular papules on the dorsal hand and at the advancing edge of the plaques. The scalp showed mild diffuse erythema and scale. There was an orange-red waxy palmoplantar keratoderma and nutmeg grater-like follicular papules on the knuckles. Islands of sparing were prominent on the torso. The initial BSA was 50%. The diagnosis of PRP, with a differential of psoriasis and progressive symmetric erythrokeratoderma, was supported by a skin punch biopsy which revealed parakeratosis and orthokeratosis on a vertical and horizontal plane, irregular psoriasiform hyperplasia, thickened papillary dermal plates, preserved granular layer, and a perivascular lymphocytic infiltrate in the superficial dermis.	The patient had insufficient response with acitretin 10 mg/day with betamethasone valerate ointment 0.1% twice daily. Acitretin was increased to 20 mg/day and STELARA 45 mg SC at weeks 0, 4, and every 12 weeks was initiated. Gradual improvement was noted after the second dose of STELARA and the skin almost cleared at 3 months. The palmoplantar keratoderma resolved completely. STELARA dosage was increased to 90 mg SC which resulted in scalp symptom (erythema, scale, itch, and some alopecia) improvement. Side effect of “tackiness” on the palms was noted with acitretin. Maintenance therapy included acitretin 10 mg/day and STELARA 90 mg SC every 12 weeks.
Allen et al (2021)¹⁰	A 70-year-old male presented with progressive erythroderma secondary to treatment-refractory PRP. Investigations for underlying malignancy, including CT-CAP, were unremarkable. A QuantiFERON^®-TB Gold test prior to biologic therapy confirmed latent TB.	The patient did not respond to treatments with acitretin, cyclosporine, and mycophenolate and was on MTX 20 mg SC at the time of presentation. TNFi was not given due to the risk of reactivation of latent TB. STELARA 45 mg was initiated with concomitant isoniazid. Complete clinical remission was observed after 6 months.
Nielsen et al (2020)¹¹	A 70-year-old female presented with a lifelong history of severe skin disease, with symptoms beginning before age 1 (generalized, confluent eruption of red, scaly plaques). Her disease involved primarily the face, arms, and legs, with a few islands of spared skin. Her family had a history of undiagnosed skin disorders. At age 70, a genetic diagnosis of PRP with a pathogenic CARD14 mutation was made.	Prior to genetic diagnosis, the patient experimented with different nonbiologic treatments, including acitretin, MTX, and alitretinoin; all elicited minimal response. After PRP was confirmed, STELARA 45 mg was administered at weeks 0 and 4, and every 12 weeks thereafter. An effect was noted within 3 weeks, with improvement in redness and scaling. Quality of life also improved.
Camela et al (2020)¹²	A 66-year-old female presented with generalized erythematous-desquamative eruption that first appeared on the face and décolletage then spread caudally to the trunk and limbs 2 months before dermatologic examination. Patient was erythrodermic with scattered isles of sparing within affected skin. Other presentations included intense facial oedema with bilateral ectropion, severe ear and scalp desquamation, alopecia, palmoplantar keratoderma, and nail dystrophy. The patient had no history of pre-cutaneous disease. Histological findings consisted of acanthosis of the epidermis, alternating orthokeratosis and parakeratosis in vertical and horizontal directions without intracorneal accumulation of neutrophilic granulocytes, and a moderate dermal perivascular inflammatory infiltrate. A diagnosis of erythrodermic PRP was made.	The patient refused phototherapy, and the use of acitretin or MTX was contraindicated due to comorbidities. STELARA 45 mg SC was administered monthly for 8 weeks, then every 12 weeks. Significant improvement was shown after 8 weeks of treatment. Complete clinical remission was observed at week 12, and no relapse observed within 36 weeks of follow-up.
Baskan et al (2019)¹³	A 52-year-old female presented with a 4-month-old, diffuse, pruritic, salmon-colored rash on her trunk, extremities, and face, and hyperkeratotic palms and soles associated with mild nail dystrophy. A cutaneous examination showed sparse islands of spared skin in the erythematous areas. Skin biopsy was consistent with PRP. Medical comorbidities, including any underlying immunodeficiency or malignancy, were ruled out. Thereafter, the patient was diagnosed with type I PRP.	The follicular papules persisted following treatment with cyclosporine A 3 mg/kg/day and topical glucocorticoids for 2 months. The lesions persisted and liver function deteriorated over 3 months after the treatment regimen was changed to acitretin 0.5 mg/kg/day and MTX 15 mg/week. MTX was discontinued and the acitretin dose was reduced. Treatment with STELARA 45 mg SC was initiated at weeks 0 and 4, and quarterly thereafter. The lesions disappeared completely after 6 months of STELARA treatment.
Matsuda et al (2019)¹⁴	A 72-year-old male presented with an erythematous plaque with scaling on his upper back. Under the diagnosis of psoriasis, he received topical and oral corticosteroids and topical vitamin D, but his symptoms persisted. The patient then presented with sharply demarcated scaly erythemas on his face, scalp, trunk, and extremities. Histological findings from the patient’s upper back showed moderate acanthosis with broad rete ridges, follicular hyperkeratotic plug, slight spongiosis, and mild superficial perivascular lymphocyte infiltration, but no neutrophil infiltration in the dermal papillae. The patient initiated treatment with secukinumab, a topical corticosteroid, and vitamin D. Five days after the second injection of secukinumab, his eruptions progressed to generalized erythroderma. Secukinumab was discontinued, and oral corticosteroids (prednisolone 30 mg/day) and then infliximab yielded no response. Two weeks after the second administration of infliximab, the patient had islands of normal-appearing skin among generalized erythroderma and a diagnosis of type I PRP was made.	After receiving etretinate 30 mg/day, the patient had moderate improvement of skin lesions, which was soon reversed. The patient received MTX 20 mg/week for 2 weeks, then cyclosporine 100 mg/day for 8 weeks. The patient’s lesions temporarily improved; however, cyclosporine was discontinued due to hypertension and dizziness. The patient had a short period of success with phototherapy (narrow-band UVB) and an oral phosphodiesterase 4 inhibitor. Almost 1 year after the onset of generalized erythroderma, STELARA 45 mg SC was administered at weeks 0 and 4 and then every 12 weeks. Two weeks after the first injection of STELARA, the patient showed dramatic improvement. Almost complete remission was achieved 4 weeks after the first injection of STELARA and maintained for 48 weeks.
Moreno-Suarez et al (2018)¹⁵	A 76-year-old male presented with an extensive and pruriginous salmon-colored and desquamative rash that affected 80% of his BSA, with severe palmoplantar involvement and nail dystrophy which had progressed insidiously for 2 months. Spared islands of skin were present within the erythematous areas. A skin biopsy showed hyperkeratosis alternating horizontally and vertically along with epidermal hyperplasia. Clinical and laboratory tests ruled out underlying malignancy and a diagnosis of erythroderma PRP type I was made.	The patient did not respond to several treatments, including acitretin, cyclosporine, and MTX. STELARA 45 mg SC was administered at weeks 0 and 4 and then every 12 weeks. The patient’s rash diminished significantly within 10 weeks and the patient’s body erythema decreased from >90% to 10%. The patient’s keratoderma and nail dystrophy drastically improved. Twenty weeks after the first STELARA injection, only isolated erythematosquamous plaques were present in the trunk.
Bonomo et al (2018)¹⁶	A 7-year-old girl with biopsy-proven PRP. She developed symptoms at 7 months of age, with persistent erythematous plaques generalized over the trunk and extremities. Topical steroids had been initiated for a primary diagnosis of psoriasis. However, the recurrence of the lesions prompted a punch biopsy that was consistent with a diagnosis of classical juvenile PRP. Continued treatment with topical medications (hydrocortisone cream, topical retinoids, and topical vitamin D analogues) provided minor improvement. Over the next 6 years, the patient continued to have lesions and upon presentation the patient had diffuse erythematous scaly patches covering approximately 80% of her BSA, with areas of normal skin and hyperkeratosis of the palms and soles.	Cyclosporine 100 mg 3 times weekly was administered, along with biweekly phototherapy and a topical regimen of tacrolimus 0.1% ointment, triamcinolone 0.1% ointment for the body, and hydrocortisone butyrate for the face. The patient responded well with decreased erythema and scaling at 4 and 8 weeks; however, the patient experienced a flare during the fifth month of treatment. STELARA 45 mg SC was administered at weeks 0 and 4 and then every 12 weeks. At the time of the second injection the patient’s erythema had diminished significantly from BSA 80% to BSA 20% and there was less pruritus. The dose of cyclosporine was decreased to 100 mg twice weekly. On follow-up 4 weeks after the second injection there was continued improvement and she was clear of generalized erythema and only continued to have some hyperkeratosis of the hands. Cyclosporine was decreased to once weekly and was discontinued at week 12. After 6 doses of STELARA the patient was clear of erythema and remained asymptomatic.
Ismail et al (2018)¹⁷	A 74-year-old female presented with a rash on her right upper chest which progressed to become generalized over 2 months. The rash had a follicular component with islands of sparing, and thick, orange hyperkeratosis of the palms and soles, all features of classic erythrodermic PRP. The diagnosis was confirmed by skin biopsy. Following the onset of erythroderma, the patient developed pedal edema, poor urine output, and felt shivery and unwell. She declined to be admitted to the hospital and opted for home treatment. Emollients were applied regularly (approximately 5-6 kg/week), and the patient was monitored. The patient was stabilized by week 6, but the erythroderma persisted with over 90% of the skin affected.	MTX over 3 months did not provide any discernible improvement. Subsequent treatment with acitretin 20 mg daily was discontinued after 3 months due to adverse effects and lack of efficacy. The patient’s DLQI was 21 at this stage. STELARA 45 mg was administered at weeks 0, 4, and 12. There was no initial response. However, after 6 weeks a small but significant improvement was noted. After the second dose of STELARA the rate of improvement increased with almost complete clearance by the third dose at week 12.
Aragón-Miguel et al (2018)¹⁸	A 30-year-old male with intense itchy erythroderma that stared 2 months prior. The lesions started on his palms and progressed to involve the entire body. Histology of the skin lesions revealed hyperparakeratosis along with epidermal hyperplasia. A diagnosis of type I adult PRP was made based on these findings.	Initial treatment with acitretin 50 mg/day combined with PUVA 3 times per week for a total of 8 weeks was ineffective. STELARA 45 mg was administered at week 0 and 4, and then every 12 weeks. After 4 weeks of treatment, improvement was observed with reduction of the erythema, scaling, and itching. The skin was completely clear after 9 months.
Paganelli et al (2017)¹⁹	A 78-year-old female with a long-standing history of classical adult-type (type I) PRP, confirmed histologically. Despite an initial benefit with topical and oral retinoids, phototherapy (PUVA and narrow-band UVB), cyclosporine, and MTX, loss of efficacy was observed over time with each therapy. The patient experienced worsening of disease. Biosimilar infliximab (CT-P13) was started at standard doses (5 mg/kg at 0, 2, and 6 weeks, then every 8 weeks); however, the patient did not show a significant clinical response at the end of the induction phase.	STELARA 45 mg SC was initiated at weeks 0 and 4, and then every 12 weeks. The patient, who was HLA-Cw6 positive, had a marked improvement within 4 weeks of the first injection, with a dramatic decrease in scaling and erythema. Long-term control of the disease was achieved after 34 weeks of treatment. Hyperkeratosis of the stratum corneum, irregularly shaped keratinocytes in the epidermis, and dilated vessels in the dermis, present at baseline with reflectance confocal microscopy imaging, were absent at follow-up visits, while a regular “honeycomb” architecture of the epidermis was restored and inflammatory infiltrate was reduced. Results were confirmed by optical coherence tomography imaging, which showed a reduction in both the hyper-reflectance of the cornified layer and the vascularization of the papillary dermis, markedly evident before the initiation of the treatment.
Abbreviations: BSA, body surface area; CARD14, caspase recruitment domain-containing protein 14; COVID-19, coronavirus disease 2019; CT-CAP, computed tomography of the chest, abdomen, and pelvis; DLQI, Dermatology Life Quality Index; HLA, human leukocyte antigen; MTX, methotrexate; PRP, pityriasis rubra pilaris; PUVA, psoralen plus ultraviolet A; SC, subcutaneous; TB, tuberculosis; TNFi, tumor necrosis factor receptor inhibitor; UVB, ultraviolet B.

LITERATURE SEARCH

A literature search of MEDLINE^®, EMBASE^®, BIOSIS Previews^®, and DERWENT^® (and/or other resources, including internal/external databases) was conducted on 25 December 2024.

Relevant literature from 2017 to date has been summarized in this response. Additional case reports were identified in literature searches, but are not summarized.²⁰^-³⁰

References

Show

1	Maloney NJ, Hisaw LD, Worswick S. Refractory pityriasis rubra pilaris treated with etanercept, adalimumab, or ustekinumab: a retrospective investigation. Dermatol Ther. 2017;30(6):e12559.
2	Pham JP, Allen N, Smith A. Efficacy of ustekinumab and guselkumab in treatment refractory pityriasis rubra pilaris: a case series. Australas J Dermatol. 2022;63(4):522-524.
3	de Brito M, Dhawan G, Alassaf J, et al. Ustekinumab: an effective agent for refractory pityriasis rubra pilaris? Three cases with a literature review [abstract]. Br J Dermatol. 2021;184 (5): e150. Abstract 001.
4	Lwin SM, Hsu C, Liu L, et al. Beneficial effect of ustekinumab in familial pityriasis rubra pilaris with a new missense mutation in CARD14. Br J Dermatol. 2018;178(4):969-972.
5	Napolitano M, Lembo L, Fania L, et al. Ustekinumab treatment of pityriasis rubra pilaris: a report of five cases. J Dermatol. 2018;45(2):202-206.
6	Reitmajer M, Forchhammer S, Silber T. Successful treatment of CARD14‐negative juvenile pityriasis rubra pilaris with ustekinumab. [published online ahead of print December 12, 2023]. J Eur Acad Dermatol Venereol. doi:10.1111/jdv.19708.
7	Gamonal S, Marques N, Pereira H, et al. Pityriasis rubra pilaris (type I) following administration of the BNT162b2 mRNA COVID-19 vaccine: successful treatment with ustekinumab and acitretin. Dermatol Ther. 2022;35(12):e15899.
8	Katharina M, Sylvia S, Matthias B, et al. Successful treatment of a child’s pityriasis rubra pilaris (PRP) with ustekinumab and acitretin. Pediatr Dermatol. 2022;39(4):659-661.
9	DeBiasio C, Cyr J, Ayroud Y, et al. A case of classic adult pityriasis rubra pilaris successfully treated with a combination of acitretin and ustekinumab: A case report. SAGE Open Méd Case Rep. 2022;10:2050313X221093453.
10	Allen N, Smith A. Successful treatment of refractory type I pityriasis rubra pilaris with ustekinumab. Poster presented at: 53rd Annual Scientific Meeting of the Australasian College of Dermatologists (ACD); April 9-11, 2021; Virtual.
11	Nielsen R, Gram S, Bygum A. Identification of a pathogenic CARD14 mutation in a 70-year-old woman with pityriasis rubra pilaris: when genetic diagnosis influences choice of treatment strategy. BMJ Case Rep. 2021;14(1):e235287.
12	Camela E, Miano C, Caterino PD, et al. Erythrodermic pityriasis rubra pilaris treatment: two case reports and literature review. Dermatol Ther. 2020;33(6):e14223.
13	Baskan E, Hasal E. Treatment of pityriasis rubra pilaris with ustekinumab: a case report. Abstract presented at: 24th World Congress of Dermatology; June 10-15, 2019; Milan, Italy.
14	Matsuda T, Yamazaki F, Ueda‐Hayakawa I, et al. Case of pityriasis rubra pilaris progressed to generalized erythroderma following blockade of interleukin‐17A, but improved after blockade of interleukin‐12/23 p40. J Dermatol. 2019;46(1):70-72.
15	Moreno-Suarez F, Salazar-Nievas M, Aceituno-Madera P, et al. Ustekinumab an alternative therapeutic option for refractory erythroderma pytiriasis rubra pilaris disease. J Am Acad Dermatol. 2018;79(3, suppl.1):AB309.
16	Bonomo L, Raja A, Tan K, et al. Successful treatment of juvenile pityriasis rubra pilaris with ustekinumab in a 7-year-old girl. JAAD Case Rep. 2018;4(2):206-210.
17	Ismail N, Callander J, Williams M, et al. Erythrodermic pityriasis rubra pilaris managed at home: intensive community care followed by ustekinumab. Clin Exp Dermatol. 2018;43(6):749-751.
18	Aragón‐Miguel R, Prieto‐Barrios M, Calleja‐Algarra A, et al. Refractory pityriasis rubra pilaris with good response after treatment with ustekinumab. J Dtsch Dermatol Ges. 2018;16(8):1022-1025.
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