Diabetes-Related Scleredema: Primary Characteristic
The primary characteristic of diabetes-related scleredema is thickening of the dermis with accumulation of collagen and aminoglycans. 1
Pathophysiology of Diabetes-Related Scleredema
Scleredema diabeticorum (also known as scleredema of Buschke or scleredema adultorum) is characterized by:
- Dermal thickening: The fundamental histopathological finding is increased thickness of the dermis 1, 2
- Collagen and aminoglycan deposits: The dermis contains increased deposition of collagen bundles separated by mucopolysaccharide (aminoglycan) deposits 1, 3
- Histological appearance: Widened collagen fibers separated by non-colored spots corresponding to mucopolysaccharide deposits 3
Clinical Features
Diabetes-related scleredema typically presents with:
- Hardening and thickening of the skin, particularly in the nape, back, and shoulder areas 1
- Decreased mobility of the shoulders and potential impairment of respiratory function 1
- More common in obese middle-aged men with insulin-resistant diabetes 4
- Associated with long-standing diabetes, presence of microangiopathy, and insulin dependence 1
Risk Factors
The development of scleredema diabeticorum is associated with:
- Long duration of diabetes
- Poor metabolic control
- Presence of diabetic microangiopathy
- Overweight/obesity
- Insulin therapy requirement 1
Differential Diagnosis
Scleredema must be differentiated from:
- Systemic sclerosis
- Eosinophilic fasciitis
- Cutaneous amyloidosis 2
Treatment Options
Treatment of scleredema diabeticorum is challenging with inconsistent results:
- Ultraviolet A1 phototherapy has shown substantial clinical improvement in some cases 4
- PUVA therapy combined with methotrexate has been reported as successful in certain cases 5
- Steroids and colchicine have been used with some success 2
- Radiation therapy may be considered in severe cases 1
Potential Complications
If left untreated, scleredema diabeticorum may lead to:
- Decreased mobility of the shoulders
- Impaired respiratory function
- Sleep apnea syndrome
- Association with monoclonal gammopathy in some cases 1
The correct answer to the question is A) thickening of the dermis, which is the primary characteristic of diabetes-related scleredema. While B) accumulation of collagen and aminoglycans is also correct and is part of the pathophysiology, the fundamental defining feature is the thickening of the dermis itself.