Scleredema diabeticorum

Abstract

Scleredema of Buschke is a rare connective tissue disease with a poorly understood pathogenesis. Three types of scleredema have been distinguished according to its association with preceding or underlying conditions. Type 1 is usually secondary to a febrile infection, type 2 is mostly associated with paraproteinemia and type 3, usually named scleredema diabeticorum, has a strict association with Diabetes mellitus. A diffuse, non-pitting swelling and induration of the skin define this disease. The skin histology is characterized by a normal or slightly thinned epidermis, and the dermis containing a decreased number of elastic fibers and thick large swollen collagen bundles separated by mucopolysaccharide deposits in the deep reticular dermis. In this report we present a 58-year-old man with scleredema diabeticorum controlled with a topical steroid cream and an optimization of glycemic control. We reviewed clinical, histopathological characteristics and the various possible treatments.

Scleredema Diabeticorum: A Rare Metabolic Connective Tissue Manifestation of Type 2 Diabetes Mellitus Causing External Restrictive Lung Disease

“…SD is a rare skin manifestation of diabetes, which can be recognized in 2.5%-14% of patients with poorly controlled disease [ 2 ]. Poorly controlled DM causes the production of advanced glycation end products, which damage the microvascular endothelium, causing endothelial dysfunction and leaky capillaries [ 2 ].…”

“…SD is a rare skin manifestation of diabetes, which can be recognized in 2.5%-14% of patients with poorly controlled disease [ 2 ]. Poorly controlled DM causes the production of advanced glycation end products, which damage the microvascular endothelium, causing endothelial dysfunction and leaky capillaries [ 2 ]. Irreversible glycation of type 1 collagen, microvascular exudation, and microvascular arteriolar thrombosis are the pathologies that occur in the connective tissue [ 2 ].…”

“…Poorly controlled diabetes can lead to arterial vascular endothelial dysfunction, causing large-to-medium arterial atheroma formation and small capillary arteriolar vasculopathy. Scleredema of Buschke is a rare connective tissue disease used to describe distinct histopathological changes occurring in the dermis and subcutaneous tissue [ 2 ]. There are three types described in the literature according to etiology.…”

“…There are three types described in the literature according to etiology. Type 1 scleredema (55%) is associated with streptococcal infection [ 2 ], type 2 scleredema (25%) is associated with paraproteinemia secondary to hematological malignancy [ 2 ], and type 3 is (3%) is scleredema diabeticorum (SD) [ 2 ]. SD is a rare complication of diabetes, which is mainly seen in type 2 DM but occasionally in type 1 DM as well [ 3 ].…”

“…There are no identified treatments for SD. However, good diabetic control and weight reduction might reduce the progression of the disease and sometimes cure it [ 2 , 4 ]. Immune-modulatory drugs such as methotrexate, cyclosporine, and topical steroids may be beneficial [ 2 ].…”