Rheumatic Manifestations of Diabetes (Mimics, Common Culprits & More)

Diabetic cheiroarthropathy can be associated with a digital sclerodactyly that looks similar to scleroderma, and thus is sometimes called pseudoscleroderma. Notably, Raynaud’s phenomenon will be absent and nailfold capillaries will be normal. Digital ulcerations of the hands will also be absent. When present, digital ulcerations should trigger evaluation for connective tissue disease.

Scleredema

Scleredema is a dermatologic condition characterized by excess collagen deposition in the skin, and has three distinct subtypes. Type I, also known as scleredema adultorum of Buschke, is often self-limited and associated with a preceding viral illness. Type II is associated with paraproteinemia and risk of myeloma, and type III, the focus of this section, is an indolent form known as scleredema diabeticorum, associated with difficult-to-control diabetes.⁶

In diabetes, hyperglycemia leads to on-enzymatic glycosylation of collagen that becomes resistant to breakdown, leading to an accumulation of abnormal collagen in the skin, which is doughy and indurated, rather than tight and shiny as seen in scleroderma.^6,8

Dr. Schiopu

Dr. Elena Schiopu, professor of medicine at the Medical College of Georgia, Augusta, with clinical and research expertise in scleroderma and myositis, often encourages her trainees to examine patients for scleredema in its usual “cape-like distribution,” looking for symmetric skin thickening along the neck, upper back and shoulders. In severe cases, the face can also be involved.

Several important features distinguish scleredema from scleroderma. Namely, scleredema spares the hands and feet, Raynaud’s will be absent, and nailfold capillaries will be normal. Systemic organ involvement is rare, but the heart, lungs and tongue can be affected. Dr. Schiopu says “pigmentary changes, which we would expect to see in scleroderma, would also be absent.”

Dr. Schiopu notes that she is often “impressed with how much scleredema can impair quality of life, with movements like showering—even breathing—impaired,” given involvement of the upper back and chest wall.

Diagnosis of scleredema is clinical, but Dr. Schiopu prefers skin biopsy to exclude other etiologies. It is also important to specifically query type II scleredema with serum protein electrophoresis (SPEP) and immunofixation, given the risk of underlying monoclonal gammopathy or myeloma.

Management of scleredema is challenging because various therapies, such as ultraviolet light, intralesional steroids, radiation and immunosuppression, have limited benefit.⁸ Dr. Schiopu explains that “the disease-modifying role here isn’t as visible. I haven’t found any immunosuppressive agents with any signal, including intravenous immunoglobulin. Additionally, diabetes itself is a comorbidity that already complicates the decision to add immunosuppression. The best option we have is tight control of diabetes.”