Especially the diagnosis for Karl Marx took very long – until 2007. Although this disease – formerly called hidradenitis suppurativa – is anything but rare. An estimated 70 million people worldwide are suffering from it, in Germany alone between 200,000 and over 3 million people. Mainly women are affected by this chronic inflammatory skin disease which comes along with nodular inflammations, fistulas in the armpits, groins, groin creases and underneath the female breast. In men the inflammation coming and going in intermittent phases mainly shows perianal. Acne inversa occurs most frequently during the age between 20 and 30, but also during puberty or in old age.
Even danger of cancer
During the early stage of the disease, gigantic black heads and palpable lumps develop, in the late stage abscesses, fistulous tracts and large, confluent nodes dispensing pus, sebum or a secretion when put under pressure. All the way to the rectum intestinum and the ureter those fistulous tracts can spread. If the center of inflammation is not active any more, usually scars remain. In cases of long-term affection, bacterial super-infections come time and again wit the result of chronic local swellings. There are even infrequent reports about the development of skin cancer in acne inversa areas, the risk for a squamous epithelium carcinoma in the perianal area is estimated at around 2 to 3 percent (Volker Wienert: Coloproctology 2009, 31, 175-179).
Prejudice lack of body hygiene
Similar to other skin diseases, the life quality of patients with acne inversa is considerably limited, not only due to the symptoms but also to its successions such as depressions and social isolation. Again and again, patients have to face the accusation that they don’t take care of their body hygiene which causes their symptoms. But – despite intensive research – the exact cause for this disease is not known yet. One is for sure though: Genetic predisposition. In nearly one third of the patients the disease is considered genetically determined. Today it is considered certain that acne inversa is a disorder of the terminal hair follicle and not of the apocrine sweat glands. The inflammation of the apocrine sweat glands is a secondary phenomenon thus the old term hidradenitis suppurativa incorrect. In addition to smoking, over weight, increased sweating, tight clothing, diabetes mellitus as well as a weakened immune defence are being discussed as predisposing factors.
Mostly the diagnosis is made clinically. It is problematical that the disease often is not being recognized. The changes on the skin are often misinterpreted as sweat gland abscess. The diagnosis gets even harder due to additional disorders similar to acne inversa and only a histological examination clears matters. Examples here are furuncles caused by staphylococci or streptococci respectively carbuncles, pathogen caused diseases like deep trichophytia, actinomycosis, sporotrichosis and – mainly in the groins and genitals – lymphogranuloma inguinale. Additional differencial diagnoses are granulomatous diseases like tuberculosis cutis colliquativa or cutaneous fistulas in cases of Morbus Crohn.
Surgery is the standard therapy
Spontaneous healings are very rare. In the long run, conservative treatment methods, incisions, scissions of abscess or fistula don’t get anywhere. The therapy of choice is the radical surgical removal of the affected skin areas. But given the size of the surgery, the professionals kept looking for other conservative methods. There are experiences with X-rays from back in the fifties. But given the potential long-term damages on the skin, this procedure is rarely used today. Ablative laser procedures were applied as well. But here you have to expect increased scarring. Since high values of male hormones can promote acne inversa, physicians used anti-androgens like for example cyproterone acetate. But only women have this option and it is only effective if prior to the therapy the women showed an increased level of androgens. 5-alpha reductase inhibitors applied on men work similarly. Bacterial infection plays an outstanding role in the severity of the course of the disease. By giving specific antibiotics like for example topic Clindamycin, a radical surgery cannot be avoided, but the pain and secretion can be reduced. The therapy with retinoids like Acitretin and Etretinat appears to be useful for relapse prevention and symptom relief as well. Oral Isotretinoin however shows only little effect. An additional option being researched is the topic photodynamic therapy. Even Botulinum Toxin A was used for treatment already.
The multiplicity of therapy approaches leads to the assumption that there is no therapeutic-conservative royal road to success. So many dermatologists build their hopes on the relatively new substance group TNF-alpha-antagonists which has been used on patients with Morbus Crohn, psoriasis and rheumatoid arthritis successfully for several years. The first case reports and studies on TNF-alpha-antagonists used against acne inverse like for example Etanercept, Infliximab and Adalimumab are quite promising. Worldwide several studies are in process, also German clinics are participating, some studies have been concluded but not published yet. But even if the TNF-alpha-inhibitors should meet all expectations for a very effective and at the same time very well tolerated therapy, it still applies that the best of all therapies loses its value if the diagnosis is not made at all or way too late. Let’s exaggerate a bit: Just don’t let it take as long as it took with Karl Marx.