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A 39-year-old man is referred for evaluation of a mass on the back of his neck. He states that the condition limits the range of motion in his neck and causes discomfort, especially when he is lying on his back. The patient’s medical history includes type 1 diabetes diagnosed at age 7, hypothyroidism, hypertension, and hypercholesterolemia. The patient’s medications include insulin, levothyroxine, enalapril, and pravastatin. Physical examination reveals striking thickening of his posterior neck. No masses were noted elsewhere.
Scleredema is a rare disorder of connective tissue of unknown etiology. The condition is linked to 3 distinct pathologies: streptococcal infection, monoclonal gammopathy, and diabetes mellitus.1 All variants are characterized by nonpitting induration of the skin that is most commonly located...
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Scleredema is a rare disorder of connective tissue of unknown etiology. The condition is linked to 3 distinct pathologies: streptococcal infection, monoclonal gammopathy, and diabetes mellitus.1 All variants are characterized by nonpitting induration of the skin that is most commonly located on the neck or upper back.
Histologic examination reveals thickened collagen bundles within the dermis separated by glycosaminoglycan (mucin) deposition. Scleredema diabeticorum, as illustrated by this case, is a known complication of long-lasting and poorly controlled insulin-dependent diabetes and occurs in approximately 2.5% to 14% of patients with diabetes.1,2
Ultrasound may prove useful in gauging depth of involvement and extent of disease.3 Treatment of scleredema can be challenging, with a wide range of therapies including immunosuppressant therapy (corticosteroids, methotrexate), electron beam radiotherapy, tamoxifen, psoralen plus ultraviolet light therapies (PUVA), and ultraviolet A1 phototherapy reporting mixed and inconsistent results.4 In most cases, tighter glycemic control remains the best therapy.4
Stephen Schleicher, MD, is director of the DermDox Center for Dermatology, as well as an associate professor of medicine at Commonwealth Medical College and a clinical instructor of dermatology at Arcadia University and Kings College.
References
1. Raboudi A, Litaiem N. Scleredema (Buschke disease, scleredema of Buschke, scleredema adultorum). StatPearls [Internet]. Treasure Island, FL: StatPearls Publishing; 2019.
2. Krakowski A, Covo J, Berlin C. Diabetic scleredema. Dermatologica.1973;146(3):193-198.
3. Ha D-H, Lee MJ, Kim S-J. Ultrasonographic findings of scleredema adultorum of Buschke involving the posterior neck. Korean J Radiol. 2018;19(3):425-430.
4. Shrestha B, Sharma E, Mukhtar O, Kaler J, Thapa S, Khalid M. Scleredema diabeticorum with superimposed cellulitis and abscess formation. Case Rep Endocrinol. 2018;2018:9513768.
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