Scleredema diabeticorum is a rare complication of diabetes, characterized by diffuse induration of the skin over the neck, shoulders, and upper trunk and arms, progressively leading to limited upper body motility and impaired respiratory function (1). Etiology is unknown, and histopathological findings include thickened dermis with abundant mucin deposition among large collagen bundles. No treatment is currently available.
We report a case of scleredema diabeticorum treated with frequency-modulated electromagnetic neural stimulation (FREMS), a transcutaneous electrotherapy that proved effective and safe in the treatment of symptomatic diabetic neuropathy (2). Putative mechanisms of FREMS include enhancement of microvascular blood flow (3) and vasomotor activity mediated by smooth cells (4) and release of vascular endothelial growth factor.
In April 2012, a 55-year-old man with type 1 diabetes since age 11 presented complaining of a progressive thickening and hardening of his skin, deterioration in daily life activities (Barthel Index of Activities of Daily Living 10/20), and malaise. The patient had a history of poor glucose control (HbA1c 9.3% [78 mmol/mol]), laser-treated proliferative retinopathy, and ischemic heart disease. Physical examination showed a thick and hard skin over neck, shoulders, trunk, and limbs, with impairment of trunk twisting and bending, deep breathing, and limb mobility. An ultrasound showed a widespread and uniform thickening of the dermis, with an average skin thickness of 7 mm on the back of the torso (normal 1–2 mm) estimated by CT scan. A skin biopsy from the abdominal wall showed an expanded reticular dermis with abundant mucin deposition among thickened collagen bundles (Fig. 1). Scleroderma and paraneoplastic syndromes were ruled out, and scleredema diabeticorum was diagnosed. Between April 2012 and June 2013, the patient was treated with five series of FREMS (10–15 sessions) at 3-month intervals. The thickness and hardness of the skin started improving after two series of FREMS (i.e., after 6 months), with a progressive normalization of cutaneous softness and mobility of the trunk and limbs and improvement of daily life activities (Barthel Index 17/20) over the following year. The patient progressively recovered the perception of breathing normally.
Histopathology of scleredema diabeticorum. A: Skin biopsy of a normal individual (hematoxylin-eosin stain, original magnification ×50) is shown. B and C: Skin biopsy of the patient before FREMS treatment is shown. The dermis is markedly thickened with an increased number of fibroblasts and fibrosis (B) (hematoxylin-eosin stain, original magnification ×50), and mucin deposits in the dermis (emphasized in blue) are also increased (C) (colloidal iron stain, original magnification ×100).
Histopathology of scleredema diabeticorum. A: Skin biopsy of a normal individual (hematoxylin-eosin stain, original magnification ×50) is shown. B and C: Skin biopsy of the patient before FREMS treatment is shown. The dermis is markedly thickened with an increased number of fibroblasts and fibrosis (B) (hematoxylin-eosin stain, original magnification ×50), and mucin deposits in the dermis (emphasized in blue) are also increased (C) (colloidal iron stain, original magnification ×100).
In contrast with this notable clinical improvement, ultrasound, CT scans (June 2013 and 2014), and a skin biopsy (February 2013) showed no changes in either skin thickness or histopathology. Normal skin softness and mobility of the trunk and limbs have been sustained for over 1 year after the last FREMS series.
We offered FREMS treatment to our patient with scleredema diabeticorum because of its established efficacy in diabetic neuropathy, ease to perform, and overall safety and the lack of alternative options. The mechanism of action of FREMS remains to be clarified, as is the case for many of the new and largely unexplored medical treatments based on electrical impulses (5). In our patient, we were unable to objectively measure changes in skin thickness or histopathology, although we observed an impressive and sustained clinical improvement as measured by the Barthel Index. Therefore, we suggest that FREMS may be considered to improve the clinical symptoms of scleredema diabeticorum, a rare and otherwise untreatable complication of diabetes.
Article Information
Duality of Interest. M.S. received a consulting fee from Lorenz Lifetech, manufacturer of the device delivering the FREMS electrotherapy. E.B. received lecture fees from Lorenz Lifetech. No other potential conflicts of interest relevant to this article were reported.
Author Contributions. A.G., A.P., and G.D.T. performed the FREMS treatment sessions and contributed to results analyses. N.R. performed the histopathology investigations. R.N. performed imaging investigations and interpretation. A.G. and M.S. contributed to the preparation of the manuscript and critically reviewed the manuscript. G.G. provided clinical surveillance and responsibility over the entire period of treatment. E.B. conceived the study, took responsibility for the clinical treatment, and wrote the manuscript. E.B. is the guarantor of this work and, as such, had full access to all the data in the study and takes responsibility for the integrity of the data and the accuracy of the data analysis.
