Diabetic Scleredema: Characteristic Features
The characteristic feature of diabetic scleredema (scleredema diabeticorum) is the accumulation of mucopolysaccharides in the skin. This condition represents a rare metabolic connective tissue manifestation of diabetes mellitus, particularly type 2 diabetes.
Pathophysiological Features
- Histopathological findings: Scleredema diabeticorum is characterized by dermal thickening with mucopolysaccharide deposits between widened collagen fibers 1, 2
- Clinical presentation: The condition manifests as thickened, stiffened, and leathery skin with a peau d'orange appearance 3
- Distribution: Commonly affects the posterior aspect of the neck, upper back, and chest wall 3, 4
Distinguishing Features from Other Conditions
Scleredema diabeticorum must be differentiated from other similar conditions:
- Scleredema: Characterized by dermal sclerosis with collagen bundle thickening 1
- Scleromyxedema: Characterized by increased fibroblasts and mucin deposition 1
- Eosinophilic fasciitis: Distinguished by thickened fascia with inflammatory infiltrate 1
Clinical Implications
- Respiratory effects: Extensive chest wall involvement can lead to external restrictive lung disease and hypoventilation due to limited chest wall movement 3
- Mobility limitations: Can cause decreased motility of the shoulders 2
- Associated complications: Patients with scleredema diabeticorum may have a higher incidence of angina pectoris compared to diabetic patients without scleredema 5
Patient Demographics and Risk Factors
- Gender predilection: More common in male patients 6, 3
- Diabetes control: Associated with poorly controlled, long-term diabetes mellitus 6, 3
- Comorbidities: Often seen in patients with obesity and established diabetic microvascular complications 3
- Prevalence: Estimated to affect approximately 2.5% of diabetic patients 5
Management Considerations
- Phototherapy: UVA1 or PUVA therapy has shown the highest, although partial, response rates 6
- Systemic treatments: Corticosteroids and immunosuppressive drugs are reserved for severe cases where phototherapy has failed 6
- Diabetes management: Strict glycemic control combined with physical therapy is recommended 6
Diabetic scleredema is a chronic condition that, while not necessarily life-threatening, can significantly impact quality of life through skin thickening and potential respiratory complications.