Necrobiosis lipoidica in childhood: a review of literature with emphasis on therapy

Abstract

Necrobiosis lipoidica (NL) is a rare chronic granulomatous disease that manifests as sharply demarcated, telangiectatic, brownish-red plaques with atrophic yellowish centers prone to ulceration and occurs predominantly on the shins. In children, NL is extremely rare, but resistance to therapy, troublesome cosmetic appearance, painful ulcerations, and possible development of squamous cell carcinoma in long-persisting lesions are challenges during treatment. Our review includes 29 reports of NL in patients aged <18 years published from 1990 on PubMed, EMBASE, and Medline. The mean age of patients was 14.3 years, with a female predominance of 2 : 1 and a high prevalence of diabetes mellitus (80%). Data showed that potent topical steroids up to twice daily is the first-line treatment. For refractory cases, therapy can be switched to tacrolimus. Ulcerations benefit from phase-adapted wound care and anti-inflammatory medical dressings such as medical honey. Adding hyperbaric oxygenation to local or systemic therapy in difficult-to-treat ulcerated lesions can be considered. Refractory cases may be switched to topical photochemotherapy or systemic treatment with TNF-α inhibitors, systemic steroids (preferably in non-diabetic patients), pentoxifylline, or hydroxychloroquine. Necrobiosis lipoidica in childhood is difficult to treat, with a treatment failure rate of 40%. Therefore, further research through patient registries is recommended.