Small fiber neuropathy (SFN) is a debilitating condition that often leads to pain and autonomic dysfunction. In the last few decades, SFN has been gaining more attention, particularly in adults. However, literature about SFN in children remains limited. The present article reports the cases of 2 adolescent girls diagnosed with SFN. The first patient (14 years of age) complained about painful itch and tingling in her legs, as well as dysautonomia symptoms for years. She also reported a red/purple-type discoloration of her legs aggravated by warmth and standing, compatible with erythromelalgia. The diagnosis of SFN was confirmed by a reduced intraepidermal nerve fiber density (IENFD) in skin biopsy sample. No underlying conditions were found. Symptomatic neuropathic pain treatment was started with moderate effect. The second patient (16 years of age) developed painful sensations in both feet and hands 6 weeks after an ICU admission for diabetic ketoacidosis, which included dysautonomia symptoms. She also exhibited some signs of erythromelalgia. The patient was diagnosed with predominant SFN (abnormal IENFD and quantitative sensory testing) as well as minor large nerve fiber involvement. Treatment with duloxetine, combined with a rehabilitation program, resulted in a marked improvement in her daily functioning. Although the SFN diagnosis in these 2 cases could be established according to the definition of SFN used in adults, additional diagnostic tools are needed that may be more appropriate for children. Additional information about the course of SFN in children may result in better treatment options.
Small Fiber Neuropathy in Children: Two Case Reports Illustrating the Importance of Recognition
POTENTIAL CONFLICT OF INTEREST: The authors have indicated they have no potential conflicts of interest to disclose.
FINANCIAL DISCLOSURE: Dr Faber reports grants from the European Union 7th Framework Programme (grant 602273), the Prinses Beatrix Spierfonds (W.OR12-01), and Grifols and Lamepro outside the submitted work. Dr Merkies received funding for research from the Talecris Talents program, the GSB CIDP Foundation International, and from the European Union 7th Framework Programme (grant 602273). In addition, a research foundation at the University of Maastricht received honoraria on behalf of Dr Merkies for participation in steering committees of the Talecris ICE Study, LFB, CSL Behring, Novartis, and Octapharma. None of the activities are related to the submitted work. The other authors have indicated they have no financial relationships relevant to this article to disclose.
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Janneke G.J. Hoeijmakers, Catharina G. Faber, Carien J. Miedema, Ingemar S.J. Merkies, Johan S.H. Vles; Small Fiber Neuropathy in Children: Two Case Reports Illustrating the Importance of Recognition. Pediatrics October 2016; 138 (4): e20161215. 10.1542/peds.2016-1215
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