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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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STELARA - Treatment of Ichthyosis

Last Updated: 01/02/2025

SUMMARY

The company cannot recommend any practices, procedures, or usage that deviate from the approved labeling.
Case reports describing the use of STELARA in patients with ichthyosis are detailed below.¹^-⁶

Clinical data

Case reports describing the use of STELARA in patients with ichthyosis are described below.¹^-⁶

Primary Author and Year	Patient Narrative	Treatment and Outcomes
Viedma-Martinez et al (2023)¹	A 6-year-old male with a history of skin desquamation since birth presented with superficial fine whitish pruritic desquamation with underlying erythematous skin primarily on face and folds. Skin biopsy revealed findings consistent with ichthyosiform erythroderma. Genomic DNA revealed a homozygous mutation in the CYP4F22 gene. The patient was diagnosed with CIE due to CYP4F22 variant.	The patient was initiated on STELARA 1.2 mg/kg every 8 weeks. The patient experienced improvement of pruritus.
Foster et al (2021)²	A 28-year-old African American woman presented with a rash. Punch biopsy revealed psoriasiform epidermal changes and epidermolytic hyperkeratosis. The patient was diagnosed with EI.	The patient was initiated on cyclosporine and methotrexate. With the addition of STELARA therapy, the patient achieved symptom control.
Vakkilainen et al (2019)³	A 2-year-old male child presented with a history of pustular eruption at age 1.5 months which rapidly evolved into ichthyosiform erythroderma. Biopsy at 3 months revealed a psoriasiform reaction pattern with a dense ichthyosiform stratus corneum and neutrophilic infiltrates in the epidermis and upper dermis. At 18 months, the patient developed ichthyotic erythroderma, mild PPK, and onychodystrophy.	The patient’s skin was treated with basic emollients and topical mild corticosteroids. Low dose acitretin slightly alleviated scaling, erythema, and itch. At 22 months, STELARA 0.75 mg/kg was initiated at weeks 0, 4, and every 12 weeks thereafter. The patient’s pruritus, skin scaling, and PPK were alleviated.
Poulton et al (2019)⁴	A 14-month-old Caucasian male presented with erythematous, psoriasiform eruption that progressed to severe erythroderma. Skin biopsy revealed nonspecific psoriasiform features and the patient was diagnosed with psoriasis, pityriasis rubra pilaris, erythrokeratoderma, and ARCI. At 4 years old, the patient developed large joint inflammatory arthropathy and was initiated on nonsteroidal anti-inflammatory agents and methotrexate, with no response. Pathogenic homozygous missense variant in NIPAL4 (527C>A) was identified.	The patient was initiated on adalimumab and experienced rapid joint improvement and partial skin improvement, which eventually plateaued. STELARA was initiated at 0.75 mg/kg at weeks 0, 4, and every 12 weeks with dramatic skin improvement. STELARA dose was subsequently increased to 1.5 mg/kg every 8 weeks due to partial recurrence in the weeks preceding the 12-weekly injection. Erythema completely cleared, with only subtle dryness managed with minimal emollients.
Paller et al (2018)⁵	A 10-year-old White female with a history of total alopecia, palmar plantar calluses, woolly sparse hair, and generalized erythroderma with skin thickening was diagnosed with erythroderma/ichthyosis at age 5. At age 9, whole exome sequencing revealed a heterozygous c.1748T>C variant, resulting in a L583P change in desmoplakin 1.	By age 5, there was minimal improvement with oral corticosteroids, isotretinoin, cyclosporine, methotrexate, adalimumab, photopheresis, dapsone, or anakinra. STELARA 0.75 mg/kg was initiated at weeks 0, 4 and every 12 weeks. Two weeks after starting STELARA therapy, the patient discontinued topical corticosteroids due to decreased pruritus and discomfort. At 16 weeks, the patient experienced 58% reduction in IASI, 52% reduction in IASI-Scaling, and 66% reduction in IASI-Erythema. Continued improvement was sustained by 10 months.
Paller et al (2018)⁵ and McAleer et al (2015)⁶	A 9-year-old White male with SAM-DSP syndrome presented with a history of marked erythroderma, ichthyosis, PPK with nail dystrophy, and diffuse hypotrichosis. Skin biopsies revealed hyperkeratosis and parakeratosis overlying an acanthotic epidermis. Immunophenotyping was not performed.	There was minimal improvement with potent topical corticosteroids or corticosteroids and antimicrobial combination therapy, moderate improvement with topical tacrolimus, and anakinra was ineffective. Systemic acitretin resulted in significant improvement in PPK and ichthyosis. STELARA therapy was initiated, resulting in itch improvement after 4 weeks. At 12 weeks, there was a 59% reduction in composite IASI (from 8.2 to 3.3), 40% reduction in IASI-Scaling (from 3.5 to 2.1), and 74% reduction in IASI-Erythema (from 4.7 to 1.2). By 11 months, the patient experienced no adverse effects and showed continued improvement.
Abbreviations: ARCI, autosomal recessive congenital ichthyosis; CIE, congenital ichthyosiform erythroderma; DNA, deoxyribonucleic acid; DSP, desmoplakin gene; EI, epidermolytic ichthyosis; IASI, Ichthyosis Assessment Severity Index; PPK, palmoplantar keratoderma; SAM, severe dermatitis, multiple allergies, metabolic wasting syndrome.

Literature Search

A literature search of MEDLINE^®, EMBASE^®, BIOSIS Previews^®, and DERWENT^® (and/or other resources, including internal/external databases) was conducted on 15 July 2024.

References

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1	Viedma-Martínez M, Jiménez-Gallo D, Navarro-Navarro I, et al. Congenital ichthyosiform erythroderma due to a CYP4F22 mutation responds to ustekinumab: A case report and review of the literature. J Eur Acad Dermatol Venereol. 2023;37(3):e389-e391.
2	Foster M, Jones J, Schadt C, et al. 086 Familial tree of epidermolytic ichthyosis suggesting a novel genetic mutation. J Investig Dermatol. 2021;141(10):S163.
3	Vakkilainen S, Puhakka L, Klemetti P, et al. Novel DSP spectrin 6 region variant causes neonatal erythroderma, failure to thrive, severe herpes simplex infections and brain lesions. Acta Derm Venereol. 2019;99(9):789-796.
4	Poulton C, Gration D, Murray K, et al. Autosomal recessive congenital ichthyosis due to homozygous variants in NIPAL4 with a dramatic response to ustekinumab. Pediatr Dermatol. 2019;36(6):1002-1003.
5	Paller AS, Czarnowicki T, Renert-Yuval Y, et al. The spectrum of manifestations in desmoplakin gene (DSP) spectrin repeat 6 domain mutations: Immunophenotyping and response to ustekinumab. J Am Acad Dermatol. 2018;78(3):498-505.e2.
6	McAleer MA, Pohler E, Smith FJ, et al. Severe dermatitis, multiple allergies, and metabolic wasting syndrome caused by a novel mutation in the N-terminal plakin domain of desmoplakin. J Allergy Clin Immunol. 2015;136(5):1268-1276.