Diabetes-Related Scleredema: Pathological Characteristics
The primary pathological change in diabetes-related scleredema is thickening of the dermis with accumulation of collagen and aminoglycans.
Pathophysiology of Diabetic Scleredema
Scleredema diabeticorum (also called scleredema adultorum of Buschke) is a rare cutaneous manifestation of diabetes characterized by specific histopathological changes:
- Dermal thickening: The most prominent feature is significant thickening of the dermis 1
- Extracellular matrix changes: There is increased deposition of:
Histological Findings
The histopathology reveals:
- Thickened dermis with increased spaces between collagen bundles
- Increased deposition of mucopolysaccharides/aminoglycans in the dermis
- Fibrosis in more advanced cases 3, 4
Biochemical analysis of affected skin shows:
- Increased glycosaminoglycan content
- Mucinous materials composed primarily of hyaluronic acid 2
Clinical Presentation
Diabetic scleredema typically presents as:
- Skin thickening and hardening (induration)
- Predominant involvement of the upper back, neck, and shoulders
- Possible extension to the face, chest, and upper extremities
- In severe cases, a "cuirasse" pattern involving the trunk 5
Clinical Significance
The condition can lead to:
- Decreased mobility of the shoulders and affected areas
- Impaired respiratory function in extensive cases
- Restrictive lung pattern in severe cases involving the trunk 5
- Potential association with sleep apnea syndrome 1
Risk Factors
Diabetes-related risk factors for developing scleredema include:
- Long duration of diabetes
- Presence of microangiopathy
- Overweight/obesity
- Insulin dependence 1
Differential Diagnosis
The pathological changes must be distinguished from:
- Systemic sclerosis
- Eosinophilic fasciitis
- Cutaneous amyloidosis 3
The correct answer is B) accumulation of collagen and aminoglycans, as this represents the fundamental pathological change in the dermis that characterizes diabetes-related scleredema.
Options A (thickening of the dermis) is correct but incomplete as it doesn't specify the substances causing the thickening. Options C (spongiosis) and D (acanthosis) are incorrect as spongiosis refers to intercellular edema in the epidermis, and acanthosis refers to thickening of the epidermis - neither of which are characteristic features of diabetic scleredema.