Acne inversa

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Classification according to ICD-10
L73.2 Hidradenitis suppurativa
ICD-10 online (WHO version 2019)

Acne inversa is inflammation of the sebum gland and outer root sheath of the terminal hair follicle . The area under the armpits, the perianal and perigenital regions and the groin area are mainly affected .

history

The disease was first described by Velpeau in 1839 . In 1854 Verneuil published that the disease was inflammation of the sweat glands. 70 years later, the apocrine sweat glands located in the armpit and perianal region were suspected to be the cause.

As a result, the term hidradenitis suppurativa was introduced. This term is also used synonymously for the disease in many medical books and can still be found today under the ICD-10 designation, another synonym is sweat gland abscess . It was later proven that this disease does not originate from the sweat gland, but from the sebum gland and the outer root sheath of the terminal hair follicles. The inflammation of the sweat glands is secondary. Acne inversa is therefore not an inflammation of the sweat glands and the term hidradenitis is misleading ( Greek ἱδρώς hidrós sweat, adenitis glandular inflammation).

Based on these findings, the term acne inversa was introduced in 1989 by Gerd Plewig and Michael Steger , but this has not yet been included in the ICD-10.

Epidemiology

Acne inversa is common worldwide. It is estimated that around 70 million sufferers worldwide. The estimates in Germany amount to between 225,000 and 3.1 million people affected, as the number of unreported cases is very high due to an incorrect diagnosis. It affects both sexes, although it is more common perianal in men. The first manifestation can take place from puberty to old age.

Pathogenetic factors

The causes of the disease are the subject of research and have not yet been clarified. There are a number of factors that favor the course of the disease or even lead to the onset of the disease:

  • Smoking: Statistical studies clearly show that around 80% to 95% of acne inversa patients smoke. Smoking seems to play a big role in the development of the disease. It appears that nicotine promotes the multiplication of Staphylococcus aureus and acts as a trigger factor .
  • Obesity: Leads to increased moisture, a warm environment and softening of the skin due to layers of skin lying on top of one another.
  • Tight clothing: Superficial damage to skin cells due to mechanical irritation.
  • Diabetes : weakened immune system.
  • Male hormones: increased sebum production and thus faster clogging of the sebum ducts.
  • Genetic factors: Usually several people in the family have acne inversa .
  • Bacteria: Are not causally involved, but have a significant influence on the severity of the course of the disease.

Pathogenesis

A keratinization disorder of the sebum leads to an obstruction of the excretory canal. The hair continues to grow without reaching the surface. The hair roots and the sebum glands fill up more and more with horn material, so that at the beginning of the disease a palpable lump forms under the skin. This becomes increasingly larger and creates a cavity that can ignite. Bacteria (usually Staphylococcus aureus ) become infected .

The sebum becomes inflamed and pus ( leukocytes , proteins , tissue debris, etc.) accumulates in the sebum. As the disease progresses, the sebum cyst tears at some point . The inflammation spreads in the tissue and the sweat glands become involved in the inflammation. Painful ulcers , abscesses and later fistulas develop .

In the armpit region and in the perianal area, Staphylococcus aureus is often found on the skin, which otherwise only occurs in a few areas of the body. This gram-positive bacterium can often be found in the pus of acne inversa abscesses. Apocrine sweat glands are so-called scent glands, which are also located in the skin areas in which acne inversa occurs. The secretions produced by these glands do not have an acidic, but an alkaline pH . Together with the moisture itself and the skin temperature around 37 ° C, this particularly promotes the multiplication of bacteria. The natural protective function of the skin is at an average pH of 5.5.

Consequences and complications

Inflammatory efflorescences that occur as part of acne inversa are usually painful. In the case of major inflammation, there is also the risk of lymph node swelling , headache and fever. After inflammation-related destruction of blood vessels, bleeding can occur. If bacteria get into the blood vessels of the subcutaneous tissue, there is a risk of sepsis with high lethality .

As a chronic , recurrent (long-term and recurring) illness, acne inversa can also represent a considerable psychological burden for those affected. The inflammatory skin changes and scarring are perceived as stigmatizing , surgical rehabilitation (see therapy) is associated with absenteeism at work. As a result, for example, the loss of a job with subsequent unemployment , social withdrawal and depression can occur.

Division into stages according to Hurley

Historically, this is the first division. It is currently still in use (e.g. for psoriasis , acne inversa, acne ). Hurley divides patients into three different stages. This division has also been used in clinical studies in the past and is still helpful in treating the disease.

stage Characteristics
I. Single or multiple demarcated abscesses . No fistulas .
II One or more widely spaced abscesses with fistulas and scarring
III Extensive infestation with abscesses, fistulas and scarring

therapy

Treatment of acne inversa should be based on appropriate guidelines. There is currently both a German and a European guideline. The therapy takes place depending on the degree of severity by means of local measures, medication or surgical intervention.

Mild forms of acne inversa can be treated with creams or solutions containing diclofenac or clindamycin .

For moderate to severe forms, drug therapies are possible. In addition to long-term therapy with clindamycin, rifampicin or the tetracycline doxycycline , which in this case are not used as antibiotics but because of their anti-inflammatory effects, a good response of the disease to therapy with TNF-α inhibitors has been described. Of the drug therapies, only the effectiveness of the TNF-α inhibitor adalimumab has been proven in controlled randomized clinical studies. Adalimumab is currently the only systemic medication that has European approval for the drug therapy of acne inversa and, according to specialist information, is indicated for the therapy of moderate to severe acne inversa in adults. The long-term effectiveness (longer than 12 weeks), which is particularly important for chronic patients with acne inversa, has not yet been clinically tested. Adalimumab had clear side effects in the overall assessment of the EMA (European Medicines Agency) 2003., Since the beginning of 2017, a physical combination therapy of light and radio frequency (" LAight ") for the treatment of all degrees of severity has been approved in the EU, which is also supported in the Innovation Fund Project EsmAiL (evaluation of a structured and guideline-based multimodal care concept for people with acne inversa) is used.

In the second or third line, corticosteroids, retinoids such as isotretinoin , colchicine or dapsone are also possible , although the literature on these therapies only allows a weak recommendation.

If the response to drug therapy is insufficient and the findings are pronounced, extensive surgical treatment with partial closure of the wounds or open, secondary wound healing is the gold standard of therapy. Smaller findings can be treated by superficial covering of inflammatory nodules, known as deroofing. In addition, successes with ablative and non-ablative laser methods have been reported in smaller clinical studies .

Assessment under social law

Acne inversa can be recognized as a disability under social law in Germany . The classification takes place on application by the pension office according to the following expert advice:

Differential diagnosis

Acne inversa can be mistaken for swelling of the lymph nodes . This can also indicate other infections.

literature

Web links

Individual evidence

  1. ↑ a b charité: Acne inversa.
  2. ^ A b H. Breuninger, V. Wienert : Acne inversa. In: Dt. Doctor bl. 2001 ; 98: A 2889-2892 (issue 44) full text
  3. ^ A. König, C. Lehmann, R. Rompel, R. Happle: Cigarette smoking as a triggering factor of hidradenitis suppurativa. In: Dermatology. Volume 198, Number 3, 1999, pp. 261-264, ISSN  1018-8665 . PMID 10393449 .
  4. ↑ A. Hana, G. Frongia, A. Gratchev, H. Kurzen: New findings on the pathogenesis of acne inversa (PDF; 163 kB)
  5. ↑ Alice Martin: Skin dictionary: Hidradenitis suppurativa (acne inversa) - symptoms. Dermanostic, accessed May 19, 2020 .
  6. ↑ internet agency bonn cologne sunzinet typo3 and reddot programming: AWMF: Detail. In: www.awmf.org. Retrieved August 12, 2016 .
  7. ↑ Christos C. Zouboulis, N. Desai, L. Emtestam, RE Hunger, D. Ioannides: European S1 guideline for the treatment of hidradenitis suppurativa / acne inversa . In: Journal of the European Academy of Dermatology and Venereology . tape 29 , no. 4 , April 1, 2015, ISSN  1468-3083 , p. 619-644 , doi : 10.1111 / jdv.12966 ( wiley.com [accessed August 12, 2016]).
  8. ↑ Gregor BE Jemec, University of Copenhagen, Roskilde, Denmark; Alice Gottlieb, Tufts Medical Center, Boston, MA, United States; Seth Forman, Forward Clinical Trials, Tampa, FL, United States; Evangelos Giamarellos-Bourboulis, 4th Department of Internal Medicine, Athens, Greece; Ziad Reguiai, CHU de Reims, Hôpital Robert Debré, Service de Dermatologie, Reims Cedex, France; Yihua Gu, AbbVie Inc, North Chicago, IL, United States; Martin Okun, AbbVie Inc, North Chicago, IL, United States: Efficacy and safety of adalimumab in patients with moderate to severe hidradenitis suppurativa: Results from PIONEER II, a phase 3, randomized, placebo-controlled trial . Ed .: Journal of the American Academy of Dermatology, Volume 72, Issue 5, AB45.
  9. ^ Technical information on Humora. (No longer available online.) Archived from the original on August 12, 2016 ; accessed on August 8, 2016 .
  10. ↑ “The most common side effects […] (seen in more than 1 patient in 10) are infections (including in the nose, throat and sinuses), injection site reactions (redness, itching, bleeding, pain or swelling), headache as well as muscle and bone pain. Adamimulab and other drugs in its class may also affect the immune system's ability to fight infections and cancer, and some cases of serious infections and blood cancers have occurred in patients taking Humira [Abbvie]. ”Cited in European Public Assessment Report (EPAR) summary , Retrieved November 9, 2017
  11. ↑ Uwe Kirschner: Suitability of a non-invasive combination therapy of intensely pulsed light and radio frequency (lAight® therapy) for the disease control of acne inversa . In: WUNDmanagement 2018 . tape 2018 , no. 12 , ISSN  2570-1207 , p. 42 .
  12. ↑ Program of the European Hidradenitis Suppurativa Foundation (EHSF) Congress 2018. Accessed June 27, 2017 .
  13. ↑ Newsdetail University Medical Center Mainz. Retrieved June 27, 2019 .
  14. ↑ a b Wayne Gulliver, Christos C. Zouboulis, Errol Prens, Gregor BE Jemec, Thrasivoulos Tzellos: Evidence-based approach to the treatment of hidradenitis suppurativa / acne inversa, based on the European guidelines for hidradenitis suppurativa . In: Reviews in Endocrine and Metabolic Disorders . February 1, 2016, ISSN  1389-9155 , p. 1-9 , doi : 10.1007 / s11154-016-9328-5 ( springer.com [accessed August 12, 2016]).