What is Hidradenitis Suppurativa?
Hidradenitis suppurativa is an extremely painful, severe and incurable skin ailment. This is, immediately, a contrast to nearly every other acneiform condition, most of which are completely curable, and furthermore, most of which are merely inconveniencing or uncomfortable, rather than out-and-out painful. Hidradenitis suppurativa is only an acneiform condition in the academic sense. It is undoubtedly the result of sebaceous hyperplasia, like all acne conditions, but this is effectively where the similarities end.
Hidradenitis suppurativa is known by many other names, including acne inversa. However, this name has not gotten much traction in the medical community. Hidradenitis suppurativa is characterized by its cystic nature and location. It almost never afflicts the face or back, as is most common with acne. Rather, tends to assault other areas of the skin that are prone to sebaceous over-activity: the thighs, buttocks, breasts, underarms and groin. The cystic nodules formed by hidradenitis suppurativa may grow from the size of a pea up to the size of a tennis ball, or even bigger. These cysts are almost invariably a source of extreme pressure pain as they put strain on the local nerves and stretch the skin.
Hidradenitis suppurativa originates primarily from an excess of androgens and subsequent hyperplasia of the sebaceous glands. Hyperplasia of the apocrine (sweat) glands also plays a role, although these are not sensitive to the workings of the endocrine system in the way that the sebaceous glands are. Overactive apocrine glands are not frequently the cause of hidradenitis suppurativa, but dysfunctional apocrine glands are.
Who gets Hidradenitis Suppurativa?
Females are far more likely to suffer from hidradenitis suppurativa than males. However, the strongest root cause of hidradenitis suppurativa is the presence of excessive androgens. It is not entirely clear as to why this does not increase the susceptibility of males to hidradenitis suppurativa, but this is likely because men are not as likely to have the same estrogen levels as women.
Because hidradenitis suppurativa has not been thoroughly researched, many details related to its origin, including any predisposing traits that people may possess, are not well understood. It is largely agreed that there are a number of genetic factors that may contribute to hidradenitis suppurativa. Most of these are secondary; genetic predisposition to hidradenitis suppurativa almost always implies a genetic inclination toward sebaceous or apocrine malfunction, however, it is entirely possible that hidradenitis suppurativa itself is the product of genetic abnormality.
Statistics indicate a certain racial bias. Individuals of Middle Eastern, North African and Mediterranean origin are far more likely to suffer from hidradenitis suppurativa than individuals of other origins. This correlation is likely genetic.
Individuals that develop hidradenitis suppurativa are almost always post-pubescent. This is a factor of endocrine function. Pre-pubescent individuals have far fewer androgens in their system and are possessed of far greater endocrine stability prior to puberty than post-pubescent individuals. This is especially true with females.
It should be noted that hidradenitis suppurativa’s predisposing factors are very, very different from its direct causal factors. Hidradenitis suppurativa recurs and remits for years and years at a time at the beck and effect of a number of possible stimuli. These do not relate to its actual development. These simply are factors that may or may not cause an outbreak, and have absolutely no impact on the presence or absence of the disease itself.
What causes Hidradenitis Suppurativa?
Hidradenitis suppurativa itself is caused primarily by a culmination of predisposing factors, including, but not limited to endocrine dysfunction, especially androgen overload, as well as apocrine dysfunctionality. Genetic factors almost certainly come into play as well. However, hidradenitis suppurativa is not very well understood at all. Some call it an ‘orphaned disease’, as very little active research has been conducted on it, and very little is currently going on. It was first identified as a distinct condition around the beginning of the 20th century, but it is only scarcely understood better now, and much of that understanding is peripheral, coming from a better comprehension of the endocrine system, the apocrine glands and the sebaceous glands.
Apocrine dysfunction and apocrine blockages are thought to be a major culprit. However, apocrine blockages do not function in quite the same way as the blockages of sebaceous glands; apocrine glands are significantly smaller, as are the relevant pores. It should be pointed out that sebaceous blockages result in acne vulgaris, the most basic form of acne associated with adolescents on prom night. Apocrine blockage is another beast entirely that may contribute largely to hidradenitis suppurativa, but they cannot be dealt with in the same way that blackheads can.
However, while the direct causes of hidradenitis suppurativa are not all well-comprehended, the causes of hidradenitis suppurativa outbreaks are very well understood and very well documented. While on the surface it would seem this would make evading hidradenitis suppurativa outbreaks a matter of following a checklist of things to avoid, the unfortunate truth is that there are a very great number of things that can cause outbreaks of hidradenitis suppurativa, and any outbreak can be quite severe.
At the fore are any and all endocrine changes that might in any way affect the sebaceous glands. ‘Endocrine changes’ can be things incidental and controllable, such as the use of certain oral contraceptives, but they can also include a woman’s monthly cycle, or the use of antibiotics, or even a change in diet to include more or less soy. Any of these factors can cause the sebaceous glands to activate, activate more regularly or activate incorrectly, causing a hidradenitis suppurativa outbreak. Hormonal factors can be very difficult to control or predict, and the lack of understanding with respect to the nature of hidradenitis suppurativa means that many doctors will not be aware of the ailment and related considerations. For this reason it is highly recommended that diagnosed patients advocate for themselves or have their doctors correspond.
Environmental changes play a major role in the cause of hidradenitis suppurativa outbreaks, as well. There is a very strong correlation between hot, damp, humid conditions and hidradenitis suppurativa outbreaks. Exposure to humid, swamp-like or tropical conditions for very long is almost certain to cause an outbreak of considerable severity. Warmer climes in general are worse with respect to hidradenitis suppurativa outbreaks, but properly arid desert climates are a documented bastion against them.
Tight clothing can frequently irritate the skin, especially at the sites of the apocrine glands. This can prompt outbreaks over a remarkably short period of time. Likewise, any other mechanical irritation of the skin may prompt a hidradenitis suppurativa outbreak. Tight clothing is most likely to be persistent and thus the factor most worth mentioning, but any repeated offender may also be a candidate.
Various drugs unrelated to the endocrine and apocrine systems may also provoke an outbreak of hidradenitis suppurativa. Psychotropics are at the top of this list, especially the stronger drugs. Lithium in particular has been responsible for many, many outbreaks of hidradenitis suppurativa.
Various actions related to hair in the area may have a significant impact on hidradenitis suppurativa outbreaks. Shaving may irritate the skin and cause inflammation that can contribute to an outbreak, as can the use of chemical depilatories. Epilation, waxing and other forms of hair removal may similarly contribute, although they are more likely to cause issues related to sebaceous activity than hidradenitis suppurativa. The full extent of the contribution of these activities is not fully understood and is a point of contention in the dermatological community.
Smoking may trigger outbreaks of hidradenitis suppurativa, although the mechanism by which this occurs is unclear. There is a statistical correlation and a number of equally valid possibilities by which this may occur, and smokers have a much higher rate of hidradenitis suppurativa outbreaks than non-smokers. It should also be noted that smoking may contribute to further issues with hidradenitis suppurativa in its more advanced stages, although these effects are likely complementary rather than directly causal.
Embarrassment is the primary factor that holds patients back from seeking treatment for hidradenitis suppurativa. Thus, it is equally important to understand what factors do not trigger hidradenitis suppurativa outbreaks. Treatment grows progressively more difficult the longer the ailment is allowed to go without intervention, and even though most treatment methods are anything but guarantees, it is still better to engage in them sooner rather than later. Even if they can’t kick hidradenitis suppurativa outright, they can certainly lessen the suffering.
First and foremost, hygiene issues are never at the root of hidradenitis suppurativa. The first thing most sufferers will seek to blame and the first thing the unsympathetic will question is often whether or not the individual in question has been washing themselves properly. Hygiene issues can worsen a pre-existing case of hidradenitis suppurativa by irritating the skin, but they cannot directly cause it. Just as blackheads are not actually the result of dirt blocking the sebaceous glands (as is commonly believed), hidradenitis suppurativa is not an ailment caused by foreign matter blocking the glands.
Obesity is not a cause of hidradenitis suppurativa. Because hidradenitis suppurativa may lead individuals to avoid going out, and the pain may make maintaining fitness difficult, individuals suffering from hidradenitis suppurativa are statistically more prone to obesity. Obesity can exacerbate hidradenitis suppurativa by keeping the body sweating, making proper hygiene more difficult and irritating the skin with skin-on-skin contact. Through this, it may contribute to an outbreak. However, it does not directly cause hidradenitis suppurativa, nor can it directly cause an outbreak.
Bacterial elements do not come into play with respect to hidradenitis suppurativa’s cause or outbreak triggers. Bacterial infections may certainly result from hidradenitis suppurativa, as the disease frequently leaves behind open wounds, but the cysts caused by hidradenitis suppurativa have no bacterial origins and should not be thought of as such. This point must be stressed as outdated literature still circulates with respect to the origins of hidradenitis suppurativa, which are only now coming under true scrutiny—however limited that scrutiny may be.
What does Hidradenitis Suppurativa cause?
Hidradenitis suppurativa causes a host of severe symptoms, which make it a horrific struggle of an ailment to weather. The symptoms present in degrees of worsening severity. There are multiple scales against which this can be charted for the purposes of diagnosis, which will not be used here. All symptoms, however, appear in the same chronological order. The primary symptom of hidradenitis suppurativa are its large, fluid-filled lesions. These are uncomfortable at best and ultimately result in severe pressure pain. They vary in magnitude from between the size of a pea or a pencil eraser and the size of a tennis ball or an average man’s fist. The size correlates somewhat to location, with larger lesions forming on the legs and under the arms, and smaller lesions forming around the buttocks and, in women, under the breasts.
When these lesions reach a particular size without being drained, they will rupture. Whether they are drained or rupture on their own as a result of their own fluid pressure or mechanical exacerbation, they leave behind an open wound that will not heal. Eventually the lesion will reform and the cycle will repeat. Buildup of scar tissue is possible over the course of years, causing pressure to persist through the life of the cyst.
These lesions almost always result in severe scarring. The inability of the open wounds to heal fully only worsens this, causing the skin to mottle and darken, often appearing necrotic (though it is not). This is most notable when the ailment is in its most advanced stage, at which point surgical intervention becomes the only option.
The list of complications related to hidradenitis suppurativa is far longer than its list of symptoms. Most all of them arise when the disease is in its most advanced stage and left untreated. With proper treatment, most hidradenitis suppurativa complications can be circumvented. Potential complications include a myriad of infections. In the skin, these are primarily bacterial as a result of exposure due to open sores. However, in its most advanced stages, hidradenitis suppurativa can leave the body susceptible to systemic infections, including but not limited to pneumonia and bronchitis in the lungs. Anemia is commonly associated with hidradenitis suppurativa, as is renal failure. At its worst, persistent and untreated fistulas can result in squamous cell carcinoma. Squamous cell carcinoma is very rare, although not unheard of, but it is an extremely aggressive form of cancer. Its tumors spread very quickly, often afflicting the sinuses and rectum, and frequently metastasize, especially to the lungs. It must be stressed that squamous cell carcinoma is very rare even with chronic hidradenitis suppurativa as a catalyst and that this will only introduce itself as a factor after a decade or more of untreated, mistreated or undertreated hidradenitis suppurativa affliction.
The psychological effects of hidradenitis suppurativa may seem to pale in comparison to the physical complications, but in truth they are equally as profound. Depression as a result of hidradenitis suppurativa is very common. Hidradenitis suppurativa can limit motion by placing pressure on joints and making many activities very painful. Even with appropriate intervention to relieve some of the pain and discomfort, the frequently chronic nature of hidradenitis suppurativa means that it is very likely to return. This alone can be very discouraging, but hidradenitis suppurativa runs even deeper. Because it is not widely understood, and full understanding is incomplete at best, individuals suffering from hidradenitis suppurativa may not understand what they are fighting and, simultaneously, may be met with unsympathetic and judgmental treatment. Hidradenitis suppurativa is frequently (although usually erroneously) blamed on hygiene, obesity and a number of other factors, and many tend to blame the victim without fully comprehending the nature of the ailment. It is this lack of understanding and fear of judgment that prevents so many individuals from pursuing treatment in its early stages, when hidradenitis suppurativa remains relatively manageable. It is this psychological impact that tends to let the disease run its full course to its chronic, most extreme stage.
Various forms of acne are frequently related to hidradenitis suppurativa, and some think of it as a sort of acne condition. While it may seem easy to connect the dots between hidradenitis suppurativa and other forms of acne, such as acne conglobata or acne vulgaris, one does not lead to another. Many acne conditions are rooted in some of the same founding conditions as hidradenitis suppurativa, however, most notably endocrine imbalance. The mutual causation of hidradenitis suppurativa and various forms of acne can lead to the misdiagnosis of hidradenitis suppurativa in many instances, further contributing to its elusive nature.
How serious is Hidradenitis Suppurativa?
Hidradenitis suppurativa is a very serious medical condition. While hidradenitis suppurativa itself does not carry a mortality rate, its complications very much do. The number of conditions co-morbid to hidradenitis suppurativa far exceed that of other acneiform conditions.
The impact of hidradenitis suppurativa is threefold. The primary symptom, coming in the form of painful cysts and lesions, can be quite debilitating on its own even with treatment. The swelling and inflammation of these lesions and cysts can make various motor functions painful, even excruciatingly so, and even physically limit motion to the point of impossibility. The lesions alone can render an individual unable to walk if they are allowed to progress to their ultimate stage without intervention.
The secondary effects and first wave of complications grows even more troublesome. The scarring and bacterial infection of the lesions and cysts provide a constant threat. Furthermore, the systemic infections that can result from prolonged affliction can cause difficulty breathing, further compounding the physical debilitation suffered. Drawn to their ultimate conclusion, the physical complications of hidradenitis suppurativa may be fatal, coming in the form of squamous cell carcinoma, a rare and aggressive form of cancer that can rapidly spread to the lungs.
Hidradenitis suppurativa should be handled aggressively by a well-informed dermatologist as soon as it rears its head.
What does Hidradenitis Suppurativa treatment look like?
Treatment options for hidradenitis suppurativa are regrettably not as extensive as they perhaps should be to guarantee recovery. Hidradenitis suppurativa treatment outlook varies depending on the progression of the affliction, with the simplest, most affordable and relieving treatments being effective only in the early stages.
Warm compresses can help greatly to reduce inflammation in affected areas. This can also soften any sebaceous buildup present in the cysts, relieving a great deal of pressure. This is a topical treatment that is only effective after a fistula has presented itself.
A change of diet is frequently called for when a confirmed diagnosis of hidradenitis suppurativa has been acquired. This is to help the patient avoid foods that can contribute to inflammation and hormonal imbalance that might prompt an outbreak of hidradenitis suppurativa lesions.
If at all possible, climate management should come into play. A persistently warm and humid environment can keep outbreaks frequent and severe, whereas an arid environment, whether hot or cold, can cause outbreaks to seize and put hidradenitis suppurativa into remission. This may require relocation, which is not an option for the majority of patients, but even frequent trips to locales of favorable climate can be sufficient to abate the invasive spread of hidradenitis suppurativa.
In its more advanced stages, local treatments and simple abatement of the spread of hidradenitis suppurativa ceases to be an effective treatment. Late-stage hidradenitis suppurativa treatment almost always requires surgery. This usually is to relieve pressure in the lesions and cysts, and is relatively minor, but it can be necessary to perform more invasive surgeries if the lesions have made themselves severe enough. More extreme options generally require excision of the affected skin, followed by grafting. The various complications intrinsic to skin grafts render this an unfavorable option under the best of circumstances, but at the disease’s full progression it is frequently the only option of any effectiveness. More contemporary treatments apply carbon lasers to the affected area. This can contribute to the effectiveness of the treatment, but even then, it is still an imperfect gambit.
How do I know if I have Hidradenitis Suppurativa?
Hidradenitis suppurativa frequently resembles more mundane forms of skin ailments, particularly acneiform conditions. There is no effective means by which to diagnose oneself with hidradenitis suppurativa. However, there are a number of warning signs to look out for that, themselves, are worthy of bringing up with a dermatologist.
Remember, above all else: if you believe you have any skin condition, always address this with a medical professional as soon as possible. Do not rely on self-diagnosis and self-treatment. This is doubly true with hidradenitis suppurativa.
The initial symptoms of hidradenitis suppurativa are tight lesions in which fluid pressure builds. While initially these lesions will be relatively indistinguishable from acne, the difference will very quickly become apparent.