Little known, debilitating and surprisingly common

As a GP, I have at times felt confronted by how severe and complex hidradenitis suppurativa can be.
 
For such a chronic and debilitating condition affecting approximately 1% of the population, hidradenitis suppurativa tends to have low practitioner awareness.
 
It is a chronic autoinflammatory disease characterised by the development of abscesses, nodules, comedones and tunnels, with a predilection for intertriginous areas such as the axilla, groin and buttocks. Classic hidradenitis suppurativa lesions are deep-seated erythematous, painful nodules that can form rope-like scarring over time.
 
As these initial lesions are often mistaken for boils, patients can be made to feel unclean and become increasingly despondent with consecutive recurrences. The tunnels that form frequently cause malodorous discharge and progressive scarring that can lead to pain and a restricted range of motion.
 
Early recognition through increasing awareness and knowledge are important factors in instituting early management to help prevent tunnel formation, which can often only be treated by surgical intervention.
 
Take the example of Fatima (not her real name), a 36-year-old woman who developed acne at the age of 14. Soon after she noticed the development of recurrent boils under her arms and in her groin. She was seen by several GPs and emergency departments where incision and drainages were performed and short courses of antibiotics were prescribed.
 
Nothing seemed to help the situation. Eight years later, Fatima heard the name of the disease for the first time in a consultation with her new GP.
 
Such a timeframe is not uncommon, with seven years the average time to diagnosis.  
 
What are the reasons for this common delay?
 
In part, the answer is the nature of the disease itself, with its insidious onset and ability to be misinterpreted as recurrent boils and folliculitis.
 
Awareness among GPs is crucial, as we play a vital role in early recognition and management of hidradenitis suppurativa, which can help mitigate its significant burden of disease.
 
And it can indeed be a heavy burden.
 
Fatima was very self-conscious through her adolescent years. Her skin condition, she feels, is one of the main factors leading to her depression and ongoing self-esteem issues.
 
The disease was at its most emotionally painful when she started forming relationships. Acutely aware of the visibility of the disease, she experienced significant distress and had difficulties with intimacy.
 
Fatima is not alone.
 
Mental health issues are common in those who experience hidradenitis suppurativa, with double the rates of depression, anxiety and substance abuse, as well as significantly higher levels of sexual distress and dysfunction.
 
This can be addressed, as Fatima has done, through a mental health care plan. With this in place, she was able to link in with a psychologist and receive the support she needed.
 
The disease can have other consequences.
 
Although Fatima had always been slightly overweight, she found it increasingly difficult to maintain a healthy weight during puberty. She gained more weight as she got older and found that her periods became increasingly irregular and painful.
 
She sought help from her GP and, after bloodwork and an ultrasound, was diagnosed with polycystic ovarian disease (PCOS). Her PCOS made it difficult for her to conceive, and Fatima had to enlist the help of a fertility specialist to fall pregnant.
 
This, too, is not uncommon. Hidradenitis suppurativa is a complex disease that has strong associations with metabolic diseases, including obesity, acne, dyslipidaemia, diabetes and PCOS. As a result, caring for patients with this condition often requires a holistic approach, with GPs playing a key role in coordinating care and enlisting allied health professionals and specialists.
 
Over her life to date, Fatima has tried many different treatments. She had multiple incisions and drainages, as well as courses of antibiotics, and was put on the acne medication isotretinoin for a time. She has also tried many over-the-counter products, but has been largely disappointed with the results.
 
Though long-term courses of antibiotics remain first-line therapy for hidradenitis suppurativa patients, the disease is often refractory.
 
One newer approach is the use of biologics in conjunction with surgery to maintain adequate disease control.
 
These treatment options at present are limited due to Pharmaceutical Benefits Scheme (PBS) restrictions on biologic agents. Promising new trial medications may be accessible at tertiary care centres, though access to these services is often difficult. The lack of public hidradenitis suppurativa clinics has resulted in long wait times and many surgeons and dermatologists unable to offer de-roofing.
 
While Fatima realises there is no cure, she has found that a multidisciplinary approach offers the greatest level of disease control and improvement in her quality of life.
 
What does that look like? She sees a psychologist to support her mental health, and her GP to help with the management of her metabolic disease, smoking cessation and weight loss. Fatima’s dermatologist prescribes a biological agent and performs de-roofing surgery to remove any persistent tunnels.
 
This article was aided by Liverpool Hospital dermatologists Dr Jane Woods and Dr John Frew.
 
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dermatology hidradenitis suppurativa skin disease