There are three types of scleredema, a type of cutaneous mucinosis of unknown cause. Scleredema should not be confused with ‘scleroderma’, in which the skin is fibrotic (morphoea and systemic sclerosis).
Scleredema presents with symmetrical hardening and thickening of the dermal or middle layer of the skin with mucin deposits between collagen bundles. The diagnosis is confirmed by skin biopsy.
The affected areas are firm and woody plaques, sometimes slightly red or brown and often with a ‘peau d'orange’ (orange-skin) appearance. The face may appear expressionless and there can be difficulty opening the mouth.
All three types of scleredema can restrict movement but otherwise seldom have serious consequences. Occasionally swallowing and speech can be affected and other organs involved – the eyes, tongue, parotid gland, muscles, joints and heart.
Scleredema may be associated with systemic diseases. These include:
- Diabetes mellitus
- Sjögren syndrome
- Rheumatoid arthritis
- Multiple myeloma
- Malignant insulinoma
- HIV infection.
How do the three types of scleredema differ?
The acute type of scleredema typically starts with an infection, most often Streptococcus pyogenes, the cause of tonsillitis. It mainly affects middle aged women and children. Hardening of the skin of the face and neck quickly develops and spreads to the upper trunk and arms. It usually improves spontaneously over six months to two years.
This type of scleredema is not associated with infection. It starts more slowly and persists. Abnormal levels of an abnormal paraprotein (immunoglobulin) in the blood may occur, sometimes due to multiple myeloma.
‘Scleredema adultorum of Buschke’, also called ‘scleredema diabeticorum’, occurs in diabetics, particularly adult men, and is very persistent. The skin of the neck and upper back slowly thickens over months or years.
The best way to treat scleredema is unknown, because of its rarity. Some benefit has been reported with the following: