Scleredema and diabetic sclerodactylyDepartment of Dermatology, New York University School of Medicine
Lisa Moed Gruson MD and Andrew Franks Jr MD
Dermatology Online Journal 11 (4): 3
A 40-year-old man presented with hardening of the skin of his hands and upper back, which had slowly worsened with time. His medical history included insulin-dependent diabetes mellitus since childhood. Histopathologic features of a biopsy specimen from the skin of his back showed a thick reticular dermis with collagen bundles in a haphazard array, which were separated by increased deposits of connective-tissue mucin. Scleredema and diabetic sclerodactyly are both well recognized skin findings that may occur in patients with diabetes mellitus. It is important to differentiate this condition from scleroderma. Treatment is difficult, and therefore many modalities have been used. This patient has improved with aminobenzoate, colchicine, and DMSO gel.
A 40-year-old man was referred to the Charles C. Harris Cancer Pavilion for evaluation of hardening of the skin on his fingers and back. The patient was initially evaluated by an orthopedic surgeon who noted no bone or joint abnormalities and referred him for dermatologic evaluation. The patient denied joint pain but was nonetheless having difficulty flexing his fingers and stretching his upper arms. He denied any change of color of the skin in his fingers with cold weather, difficulty swallowing, or dryness of the eyes and mouth.
Medical history includes insulin-dependent diabetes mellitus since childhood, with neuropathy, retinopathy, hypertension, and alopecia areata.
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Diffuse, woody induration of the skin of the posterior neck and upper back was present. A waxy thickening of the skin of the dorsal aspect of the hands and fingers and a prayer sign, which is the inability to appose the palmar surfaces when pressing the hands together, were noted. The patient was unable to form a fist.
A complete blood count and comprehensive metabolic panel were normal. Anti-nuclear, anti SCL-70, anti-SSA(Ro), anti-SSB(La), and anti-double-stranded DNA antibodies, C-reactive protein, and an erythrocyte sedimentation rate were normal or negative.
Histopathology revealed thickened collagen bundles within the reticular dermis. In the lower reticular dermis increased deposits of connective-tissue mucin are highlighted with a colloidal iron stain.
Diabetes mellitus has been associated with many cutaneous manifestations, including necrobiosis lipoidica, acanthosis nigricans, diabetic dermopathy, and a variety of thick skin disorders. In diabetics, there is often an increase in skin thickness. A diabetic hand syndrome has been described, which has been referred to as both diabetic sclerodactyly and the scleroderma-like syndrome . This finding was initially associated with insulin-dependent diabetes mellitus but was later noted to affect patients with non-insulin-dependent diabetes mellitus (NIDDM) as well . Previous studies noted a correlation between diabetic sclerodactyly and microvascular complications ; however, a recent study did not support this association . Scleredema is a well-defined entity of waxy, indurated skin that involves the posterior neck and upper back. This manifestation is often associated with NIDDM and obesity .
The histopathologic findings of both diabetic sclerodactyly and scleredema are similar. Histopathologic examination of the diabetic hand syndrome shows a thick dermis, increased cross-linked collagen in the reticular dermis, and small amounts of mucin . In scleredema there is also a thick reticular dermis and the collagen bundles are separated by increased mucin deposition . The pathophysiology of scleredema and the scleroderma-like syndrome of diabetes mellitus has not been fully elucidated. One hypothesis suggests that glucose may stimulate fibroblast proliferation, and synthesis of extracellular matrix components. Another possibility is that collagen degradation is inhibited due to excessive, non-enzymatic glycosylation .
It is important to distinguish between diabetic thick skin and scleroderma because scleroderma may also present with sclerodactyly and diffuse or localized thickening of the skin. In evaluating patients with these findings, a history should focus on the other related symptoms found in scleroderma, such as dysphagia, calcinosis cutis, and difficulty breathing. Patients should be evaluated for the presence of autoantibodies, such as SCL-70, antinuclear antibody, anti-SS-A(Ro), and anti-SS-B(La) antibodies. The sclerodactyly in patients with these two disorders may be distinguished by the absence of Raynaud phenomenon, atrophy, and telangiectases in patients with diabetes mellitus .
Treatment options include potent topical and intralesional glucocorticoids, strict glucose control, low-dose methotrexate, prostaglandin E, and physical therapy . Our patient is being treated with aminobenzoate 500 mg three times daily, colchicine 0.6 mg twice daily, dimethyl sulfoxide (DMSO) gel daily, and strict glucose control. On this regimen, the patient has noted increased mobility of his hands and back. Aminobenzoate is chemically pure potassium p-aminobenzoate, a member of the vitamin B complex. Potaba may increase oxygen uptake of tissue, which may prevent fibrosis . This medication has been used to treat scleroderma and Peyronie disease; however, there are no reports of its use in scleredema. Colchicine has been used in scleroderma as well. It is thought that colchicine has anti-inflammatory properties and may impede collagen synthesis by depolymerizing microtubules . DMSO may help to solubilize collagen . This medication has been used in patients with scleroderma; however, there are no reports of its use in scleredema. A recent case study also reports success with UVA-1 phototherapy in a patient with scleredema .
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