Harlequin ichthyosis and juvenile idiopathic arthritis: a rare combination

Profiling Immune Expression to Consider Repurposing Therapeutics for the Ichthyoses

“…These high frequencies clustered with severity and TEWL. The peripheral blood lymphocyte skewing toward Th17/IL-22 polarity raises the question of risk of systemic comorbidities, as occurs in psoriasis, particularly cardiovascular and joint inflammation, which have received little attention in ichthyosis beyond the association of arthritis as a comorbidity of harlequin ichthyosis, a severe subset of ARCI (Beazley et al, 2011;Chan et al, 2003;Clement et al, 2007;Rajpopat et al, 2011).…”

Harlequin Ichthyosis

“…In 3 cases this was associated with an inflammatory arthritis. One case was previously reported by Clement et al 12 The patient had juvenile idiopathic arthritis and required systemic treatment with methotrexate and the anti-tumor necrosis factor drug etanercept before having bilateral hip arthroplasties at age 17 years. The second case developed an intermittent, painful swollen right knee and ankles and was treated with nonsteroidal anti-inflammatory drugs for 1 year and currently has nighttime splinting to prevent joint contractures.…”

Juvenile Idiopathic Arthritis in Harlequin Ichthyosis, a Rare Combination or the Clinical Spectrum of the Disease? Report of a Child Treated With Etanercept and Review of the Literature 

“…In a single case report worsening of erythroderma and increased cutaneous infections were reported, with poor disease control even using MTX and a biologic agent in combination. [22] In our patient, the severity of arthritis required the association of conventional and biological DMARDs with improvement of arthritis and decrease of erythema, without any infectious events reported at 18 months follow-up, nor any other adverse events.…”

“…The patient was then given MTX and Etanercept at 25 mg/week with improvement in mobility, but he needed total hip arthroplasty by the age of 17. [22] He was then given adalimumab, MTX and le unomide, but reported worsening of the cutaneous disease with cracks and increased skin infections with all previous DMARDs and biologics.…”