Fournier’s Gangrene: A Rare Complication of Sweet’s Syndrome

This is the first reported case of Fournier’s gangrene (FG) as a rare complication of Sweet’s syndrome (SS) (Choi et al, 2017).

Case-study.

A 31-year-old woman was admitted to hospital with a 7 day history of itchy, red skin lesions of various sizes all over her body. She also had a fever, and blood tests showed a raised erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP), indicating inflammation in the body. A procalcitonin test was given to determine whether or not she had developed sepsis – a rare, life-threatening complication of infection, that can occur when chemicals that the immune system releases into the bloodstream to fight infection end up causing inflammation throughout the entire body. In this case, the procalcitonin test result was normal. A skin biopsy of a lesion showed white blood cells called neutrophils in the tissues and damage to the vessels caused by neutrophils releasing noxious substances. The former was indicative of SS. The patient was treated with high dose steroids for 5 days, which she responded well to.

Three days after being discharged, the patient returned to hospital, having developed a painful 2 cm-sized lesion and swelling on her buttock. The drainage from this area had the odour of rotten fish and looked like greenish pus. An X-ray of the area did not show anything unusual, but computed tomography (CT) scan showed that gas had entered the gluteus maximus muscle layer. This is because infection can result in gas in the soft tissues. Bacterial culture showed the presence of several bacteria: Streptococcus anginosus, Pseudomonas, and Clostridium. The patient was eventually diagnosed with FG, and treated with wound debridement (surgical removal of dead tissue) and antibiotics. Two months later, the buttock lesion was completely healed.

What is Fournier’s gangrene?

FG is a rapidly progressive necrotizing fasciitis (NF) that affects the perineal, genital and perianal regions. NF is a rare and serious bacterial infection that affects the superficial fascia – a layer of connective tissue that lies beneath the skin and between the muscles and organs in the body. FG usually develops secondary to other perirectal or periurethral infections, and the main form of treatment is wound debridement and broad-spectrum intravenous antibiotics (Bracho-Riquelme, 2017; Choi et al, 2017).

Some additional & key points.

  • FG most commonly affects those with a weakened immune system (Choi et al, 2017). This includes those with a weakened immune system as a result of taking medications that suppress the immune system (immunosuppressants), e.g. the steroid, prednisone.
  • FG can be confused with the conditions erythema multiforme, erythema nodosum, leukocytoclastic vasculitis, or cellulitis.
  • CT scan is an important diagnostic tool in FG, and can detect the presence of soft tissue air.
  • Early aggressive wound debridement and antibiotics are vital in the treatment of FG.
  • Doctors should consider the possibility of FG when a SS patient who takes immunosuppressants, develops painful lesions on the perianal and perineal area (Ibid).
  • FG or NF should not be confused with the rare SS variant, necrotizing Sweet’s syndrome (NSS). This variant mimics NF, and the main form of treatment is high-dose steroids. Antibiotics do not work in the treatment of NSS, and wound debridement must be avoided as it can lead to the development of new lesions.

References.

Bracho-Riquelme, R. (2017) Fournier Gangrene. NORD: National Organization for Rare Disorders (online). Rodolfo L. Bracho-Riquelme, MD, is a general and colorectal surgeon at Hospital General de Durango, Mexico. Accessed 6/06/17.

Choi, H., Kim, Y., Na, C. and Shin, B. (2017) Fournier’s Gangrene: A Rare Complication of Sweet’s Syndrome. Annals of Dermatology, Jun;29(3):387-389 (PubMed).

© 2012-2017 Sweet’s Syndrome UK

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Challenges of Living with Sweet’s Syndrome

Sweet’s syndrome (SS) is a rare autoinflammatory condition and neutrophilic dermatosis. The most common symptom is skin lesions, but what some people don’t know is that it can cause lots of other symptoms too. On rare occasions, it can even be life-threatening, and approximately 15-20% of patients will develop SS secondary to some form of cancer, most commonly, a group of blood disorders called myelodysplastic syndromes (MDS).

Amongst the SS community, those with SS are typically referred to as ‘Sweeties’, and for them, living with SS can be incredibly difficult. This is not only due to the symptoms of SS, but to the many obstacles and unexpected challenges that they can face along the way. For example, problems associated with a change in appearance, depression, debilitating fatigue, isolation, pain, mobility issues, job loss and financial difficulties, to name but a few.

Living with Sweet’s syndrome. How can it affect a Sweetie?

Change in appearance.

  • Visible skin lesions.
  • Permanent skin discoloration or scarring.
  • Weight gain due to reduced mobility or side-effects of medication.
  • Rarely, hair loss due to the side-effects of medication.

Change in mood, depression or other changes in mental health.

  • Increased risk of anxiety or depression due to having to live or deal with certain difficulties or symptoms, e.g. not well enough to socialise, debilitating fatigue, or frequent or chronic pain.
  • Changes in mood due to the side-effects of medication.
  • Mental changes associated with the rare neurological variant, neuro-Sweet’s disease.

Loss of confidence.

  • Due to change in appearance.
  • Due to being insulted or verbally abused because of a change in appearance.

Isolation.

  • Not wanting to go out due to change in appearance.
  • Being too ill or in too much pain to work or socialise.
  • Symptoms of SS or the side-effects of medication making it difficult or impossible to do certain activities.
  • Others not wanting to associate with Sweeties because they think that their skin lesions can be ‘caught’.

Pain.

  • Painful skin lesions.
  • Headaches, less commonly, migraine.
  • Muscle pain.
  • Joint pain.
  • Eye pain associated with certain eye symptoms.
  • Occasionally, painful mouth ulcers or other types of oral lesion.
  • Rarely, pain as a result of other parts of the body being affected, including internal organs.

Mobility issues.

  • Difficulty walking and moving about because of joint pain and swelling.
  • Painful skin lesions affecting or restricting movement.
  • Neuro-Sweet’s disease causing limb weakness, or problems with co-ordination and balance.
  • Fatigue causing limb heaviness.

Lack of sleep.

  • Pain or other symptoms of SS making it difficult to sleep.
  • Side-effects of medication negatively affecting sleep, e.g. insomnia or nightmares.

Difficulty accessing information and treatment.

  • Struggling to find a doctor who has any understanding of SS.
  • Struggling to access patient information, on or off-line.
  • Health care staff unable to answer any questions that a Sweetie might have, and not able to provide information when requested.
  • Health care staff not taking SS seriously enough, or insulting a Sweetie because they don’t understand SS.
  • Having to wait a long time for the right treatment, or being denied access to certain treatments.

Difficulties associated with work.

  • Sweeties being bullied by work colleagues or customers because of their appearance.
  • Being bullied by an employer because they have to take time off, and are therefore viewed as a malingerer or a costly drain on resources. For example, having to take sick-leave when ill, being admitted to hospital, or having to attend doctor or hospital appointments on a regular basis.
  • Sweeties having to reduce their hours, even if they can’t afford to.
  • Difficultly gaining promotion, demotion or job loss.

Financial difficulties.

  • Struggling to afford to pay for care if a Sweetie lives in a country where health care is privatised.
  • Financial difficulties due to having to reduce hours at work, leave a job, being bullied out of a job or being sacked.
  • Struggling to access benefits due to the assessors or doctors having a poor understanding of SS and it not being recognised by the benefits system. This can happen even when a Sweetie is seriously ill.

Unsupportive family and friends.

  • Family and friends refusing to take SS seriously enough, and telling Sweeties to ‘just get on with it!’
  • Family and friends giving unhelpful advice and telling Sweeties that they are choosing to be ill (‘patient blaming’). For example, ‘ You wouldn’t be ill if you just ate more healthily, cut out gluten, didn’t get stressed, tried this herbal supplement, spent time in the sun, put essential oils on your lesions, or tried homeopathy.’ Some of these things can even make SS worse.
  • Getting annoyed and angry with a Sweetie because they can’t do what they once did, or are no longer in a position to help out others in the way that they did before.

There are many more challenges, obstacles and difficulties that could be added to this list, so please feel free to mention your own in the comments section below this post.

Today is Sweet’s Syndrome UK Day! Please share this post to help raise awareness of SS and the difficulties that those with this condition have to deal with, often on a daily basis. Also, a big thank you to all the health care staff, employers and work colleagues, family members and friends who DO support Sweeties across the globe. You do an amazing job, and frequently have to face your own challenges when it comes to helping and supporting those with SS. 💛

© 2012-2017 Sweet’s Syndrome UK