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(ustekinumab)

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STELARA® (ustekinumab)

Medical Information

STELARA - Treatment of Ichthyosis

Last Updated: 08/12/2025

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 17 July 2025.

 

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.