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Pediatric Necrobiosis Lipoidica Yields Treatment Failure Rate of 40%

Article

Researchers conducted a review of treatments for the rare disease in the pediatric population.

In pediatric patients, necrobiosis lipoidica, a rare dermatologic condition characterized by brown/red plaques and ulceration, yields a treatment failure rate of 40%.

CasanoWa Stutio/AdobeStock
CasanoWa Stutio/AdobeStock

A recent review1 of literature examined the role of treatment in patients under the age of 18 years old. Researchers noted the complexity of NL treatment due to its rarity in children and tendency to resist therapy, estimating that the condition affects 0.006% of children (1 in 1157) and is common in patients with pre-existing diabetes mellitus, affecting 0.3% of individuals with the condition.

At the time of the review’s publication, researchers noted that there are no reviews assessing the prevalence of the condition in patients under the age of 18, nor are there guidelines for treatment in this population. According to Schiefer-Niederkorn et al, the review intended to develop an algorithm for topical therapy, wound treatment, and systemic therapy.

Researchers began by conducting a comprehensive search of PubMed, EMBASE, and MEDLINE, including relevant research published from January 1, 1990, through November 30, 2021. Relevant search terms included: “necrobiosis lipoidica” and “adolescent,” “boy,” “child,” “girl,” “infant,” “juvenile,” “pediatric,” “teen,” and “young.”

The initial review yielded 113 records from PubMED and 128 from EMBASE and MEDLINE combined. Upon removal of duplicate publications, researchers were left with 157 relevant records.

Researchers screened all publications, excluding any non-relevant materials, including those not involving patients under the age of 18, not differentiating participant ages, not describing treatment/therapy or their outcomes, and not involving definitive NL diagnoses.

In total, 29 articles met research criteria and were included in the review. Of these, 27 were case reports and 2 were case series.

The review showed an average NL pediatric patient age of 14.3 years ± 2.6 years and an average disease duration of 32.6 ± 36.9 months. 80.6% of patients included in the review had also been diagnosed with diabetes mellitus.

In total, 55 different treatment regimens were reported among patients. These included:

  • Doxycycline
  • Glycemic control
  • Hydrocolloid/silicone dressing
  • Hydroxychloroquine
  • Hyperbaric oxygenation
  • Infliximab
  • Laser therapy
  • No therapy (diabetes maintenance)
  • Occlusion
  • Tacrolimus ointment
  • Topical treatments or steroids
  • Tretinoin cream
  • And more

Treatments could be divided into local therapy and systemic therapy. Of these treatments, 29.1% led to partial response. In 5 patients receiving monotherapy, for example, which included topical steroid use, NL improvement was achieved. However, 54.5% of therapies were deemed unsuccessful.

While topical steroids were considered the most common form of prescribed therapy (occurring in 43.6% of the included regimens), the majority of case reports did not specify which steroid was used for treatment.

“Overall, treating NL is challenging. One third of the reviewed cases of children were unresponsive toany therapy,” wrote Schiefer-Niederkorn et al. “Necrobiosis lipoidica in childhood is difficult to treat, with a treatment failure rate of 40%. Therefore, further research through patient registries is recommended.”

Reference

  1. Schiefer‐Niederkorn A, Sadoghi B, Binder B. Necrobiosis lipoidica in childhood: a review of literature with emphasis on therapy. J Dtsch Dermatol Ges. Published online July 3, 2023. doi:10.1111/ddg.15137
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