Scleredema

Scleredema is a form of cutaneous mucinosis characterised by woody induration and hardening of the skin that arise mainly as a result of infection but also in diabetes, and which spontaneously clears in months or years. The term scleredema is a misnomer because neither sclerosis nor oedema is found on microscopic examination. 

It is important to note that scleredema is a separate entity to scleroderma. 

This chapter is set out as follows:

Acute onset

• Mainly affects middle-aged women and children

• An acute onset with a preceding infection is the most common presentation, although some cases arise spontaneously. Streptococcus pyogenes appears to be the most common pathogen, arising mainly as an URTI, but also as cellulitis. Other infections include measles and mumps

• Hardening of the skin of the face and neck develops quickly and spreads to the upper trunk and arms in a symmetrical fashion. Hyperpigmentation is a common feature
  

• There can be loss of facial expression and difficulties in swallowing 

• This type of scleredema usually improves spontaneously over six months to two years

Insidious onset

• ​A more insidious onset is associated with diabetes (sometimes referred to as scleredema adultorum of Buschke, or, scleredema diabeticorum), and sometimes a paraproteinaemia, which can be secondary to multiple myeloma. It can also be associated with other connective tissue disorders such as Sjögren's syndrome and rheumatoid arthritis 

• Scleredema secondary to diabetes occurs particularly in adult men, and is very persistent. The main sites affected are the skin of the neck and upper back that slowly thicken over months or years

• In addition to routine bloods, including for diabetes mellitus, immunoglobulin and plasma electrophoresis levels need to be assessed as scleredema can occasionally be associated with paraproteinaemia and multiple myeloma, as well as Waldenstom’s macroglobulinaemia

• Scleredema may disappear after months to a few years, but some cases, such as those secondary to diabetes mellitus may persist for many years

• No effective treatment is known although many have been tried