Introduction: Scleredema is a rare connective-tissue disorder characterized by diffuse, non-pitting induration of the skin. Scleredema is divided into three types. Pathogenesis is largely unknown. We present a case of a 56-year-old woman with non insulin-dependent diabetes mellitus who presented with a progressive history of thickening of the skin on her back.
Case report: A 56-year-old woman, suffered from type 2 diabetes diagnosed 15 years ago, presented with complaints of painless, progressive hardening of her upper back present for years. Recently, she was diagnosed of incipient nephropathy, high systolic blood pressure, and retinal hard exudates close to the macula treated with laser photocoagulation. Physical examination revealed a symmetrical, erythematous, indurated plaque with indistinct borders involving her posterior neck and upper back. Slightly restriction in range of motion of the shoulders and neck was noted. Her body mass index (BMI) was of 33.1 kg/m2.The punch biopsy of the skin on her back showed an increased amount of interstitial colloidal iron with positive mucin consistent with scleredema.These features supported the diagnosis of scleredema diabeticorum. Our patient was asymptomatic and described mild restriction in movement. To our knowledge, her diabetes mellitus was controlled with subcutaneous insulin injections and diet.
Discussion: Scleredema is a rare condition of unknown pathogenesis. It is characterized by symmetrical, non-pitting hardening and induration of the skin, most commonly seen on the upper back, shoulder and neck, due to excessive increase in mucin deposition between the thickened and broadened collagen bundles. In rare occasions, the disease involves the face, arms, and the rest of the trunk, but not the hands and the feet that may decrease the mobility of the affected tissues. Scleredema is classically divided into three types, distinguished by their associations with infection, monoclonal gammopathy, and diabetes mellitus (scleredema diabeticorum).The third type occurs primarily in obese, middle-aged men with a long history of diabetes mellitus, as seen in the patient described here. It may be referred to as scleredema diabeticorum.Although scleredema may be diagnosed clinically, diagnostic confirmation may be obtained with a tissue biopsy. Treatment of scleredema is challenging. In scleredema diabeticorum, it is judicious to advise control of blood glucose as a first step in treatment, although a relationship between glucose control and improvement in scleredema has not been firmly established.
Conclusion: The frequency of scleredema diabeticorum is underestimated. In most cases, scleredema diabeticorum is a self-limited, benign condition.
18 - 21 May 2019
European Society of Endocrinology