Oral Contraceptives

Androgenic symptoms in patients with hyper-androgenism may also be minimized with OC combinations. OC may be indirectly antiandro-genic through the suppression of ovarian androgen production (antigonadotropic activity) and the oestrogen-induced elevation of SHBG. In fact, all synthetic progestins are either progesterone or testosterone derivatives. They not only bind the progesterone receptor but may also bind AR, with either agonistic or antagonistic activity. Therefore, most progestins have...

Conglobata Controversial Views

In the twentieth century the French School of Dermatology centered the nosological discussion on the connections between acne conglo-bata (described in 1901 by Spitzer under the name of dermatitis folliculitis et perifolliculitis conglobata) 24 and hidrosadenitis. In 1904, Audry (Toulouse), authoring the chapter on sweat glands in the prestigious Pratique Dermatologique, pointed out the fact that reading the authors who dealt with this question gave an impression of a complete darkness,...

Mabel

I live in South San Francisco, California and am a 40 year old female suffering from hidradenitis suppurativa since the onset of puberty. It has become progressively worse and in 1994 I had surgery to remove the abscesses from the groin area. I remained in the hospital for 3 months due to complications from the surgery (i.e. secondary infection). I had relief from the hidrad-enitis for only about 9 months after which time it returned to the areas under both arms. Since then I have been in...

The Use of Local Therapy

Initial treatment of early or minor lesions is usually local. Provided the local therapy is part of a therapeutic strategy for each patient, this is a rational approach, in terms of both traditional clinical assessments such as effect and cost-efficiency and also cost-benefit to the patient. Efficient forms of local treatment exist, although none is universally effective. They have a low potential for side-effects and many can be used for both prophylactic therapy and self-treatment of early...

Infection

There are no convincing data to suggest that HS is primarily an infectious disease (see Chap. 11). The polymicrobial infection (or colonization) of HS - Staphylococcus aureus, Gram-negative rods, anaerobic bacteria - is quite different from the usual colonization of acne by Propionibacte-rium acnes and coagulase-negative staphylococci. The role of bacteria in HS may therefore be either secondary to some as yet unknown mechanism, or purely secondary once anatomical disruptions are established....

Arnold

I understand your concern for keeping the group inclusive. Having suffered this disease for about 10 years now, it is a bit exciting - and a bit embarrassing - to share the experience with others. First, just to let you know who is writing, I am a 58 year-old white male that has the disease in the groin, upper thigh and buttock areas. There is no known similar disease anywhere in the family that is known to me. I am within 10 pounds of my correct weight for height and age and have never had a...

Possible Consequences for Bacterial Ecology due to Antibiotic Treatment in HS

The drawback to the usefulness of long-term antibiotic treatments is concern about the effect on microbial ecology (Table 11.5). The normal microflora act as a barrier against colonization by potentially pathogenic bacteria and the control of growth of opportunistic bacteria is called colonization resistance. The normal equilibrium between host and microorganisms may be disturbed by a number of factors, but commonly and essentially by antibiotic therapy. To what extent disturbances occur...

Hidradenitis Suppurativa as a Side Effect of Immunosuppressive Drugs

HS has also been described as a side-effect of immunosuppression associated with rapamycin (Sirolimus) treatment 13 . This drug has been reported to be associated with acne-like and other follicular inflammation in renal transplant patients. The study was carried out with stringent dermatological evaluation of patients, and an incorrect diagnosis of HS is therefore relatively unlikely. Specific immunosuppressive mechanisms may therefore play a role and not all immunosuppressants may be suitable...

Caroline

I began to develop what I initially called acne and now know to be HS quite a number of years ago, probably when I was in my late 20s (I'll be 35 tomorrow). It began as very small cysts or pimples in my groin area or on my buttocks that initially would go away in a few days. I mentioned it briefly to a few doctors and was simply told I had ingrown hairs or folliculitis or it was from wearing jeans most of the time. Over the years, the frequency, size, and duration of them increased to the point...

Cryosurgery

Cryosurgery has been used extensively in the treatment of both benign and malignant skin tumors. Usually liquid nitrogen is used as the coolant, and the effect of the cryotherapy is to induce necrosis based on hypothermic damage of both cell membrane and cytoplasmic organelles. A study reporting the follow-up of ten patients treated with cryotherapy has been published suggesting that cryotherapy is a feasible form of therapy in select HS cases 13 . The treatment is however not unproblematic,...

Inguinal Disease

Higher recurrence rates are seen in the inguino-perineal region 63 . Despite a high recurrence rate most patients are well satisfied after ingui-no-perineal excision because local areas of recurrence are trivial compared with the disease before surgery. Excision and healing by granulation can be applied 61 . All tissue is excised down to the fascia and the wound is allowed to granulate and epithelialize spontaneously. The excision must not go deeper than the fascia, to protect the vas deferens...

Key points

New drugs are being studied for the medical therapy of hidradenitis suppurativa (HS) Zinc salt may have an anti-inflammatory effect in HS Botulinum toxin may play a role in the treatment of HS tory lesions (superficial and deep lesions). Zinc gluconate was about 15 less effective than minocycline in a randomized trial 12 . In Verneuil's disease, the drugs usually prescribed in acne (cyclines, isotretinoin) are poorly effective, and a need for additional therapy exists. So, owing to the in...

Introduction

There has been significant debate around the pathological features of hidradenitis suppurati-va (HS) over the years. This debate focuses on whether the primary event relates to an inflammatory process of the apocrine duct or whether follicular occlusion is integral to the initiating process. HS was first described as a distinct clinical entity in 1839 by Velpeau 1 . In 1854 Verneuil suggested that the inflammatory changes which affected skin of the axillae, sub-mammary mammary regions (Fig....

TNF Signalling and HS

A number of other, smaller families and sporadic cases that were too small on which to perform a genome-wide screen did not show linkage to either of the candidate loci described here. Although HS is now known to be a heterogeneous disease it appears that it may be caused by mutations in genes at multiple locations making it a more complex disease than originally thought. The linkage to chromosome 6 was narrowed down by recombinants to a small region of 6q25.2 containing only six genes. The...

Case Series Reports

Fr hlich et al. 5 examined 231 patients and found complete relief of symptoms in 89 patients (38 ) in 92 patients (40 ) there was clear improvement and only 2 patients did not react to radiotherapy. No side-effects were noted. In the same year Seegenschmiedt et al. 6 examined patients treated with radiotherapy for benign diseases among them were 221 HS patients. They found significant geographic and institutional variations in the treatment schedules. The doses prescribed were mostly in the low...

Heredity

Is this an inheritable disease Partly. In about one-third of cases there is someone in the family who is affected by the disease. 2. What are the risks of transmitting the disease to my children There is little risk, but it is difficult to calculate. The risk is probably greater if there are other cases in the family, but the possible degree of severity cannot be predicted on the basis of your own experience. 4. Can you screen for the disease No.

Discussion

Physical therapies may be directed against either the secondary prevention of early lesions or the destruction of established lesions. For true primary prevention of early lesions the pathogenic process is insufficiently described to allow a causal therapy, although the use of laser depilation may yet prove to be fruitful in proper studies. The possible effect of PDT on the hair follicle is at present unclear. It is not convincingly proven that the penetration of ALA-PDT is sufficiently deep to...

Publication Bias

The paucity of controlled trials is apparent for local therapy of HS. There is therefore a strong bias towards anecdotal therapies. For anecdotal therapies there is a strong and well known bias towards false-positive reports. In this review of treatments the authors have attempted to assess the local therapies in view of personal experience with the treatment modalities, but this does obviously not substitute for controlled trials or careful follow-up studies of cohorts of patients. Just as in...

References

Brenner S, Matz H (1999) Improvement in androgenic alopecia in 53-76 year old men using oral finasteride. Int J Dermatol 38 928-30 2. Cunliffe WJ (1989) Non-acne disorders of the pilose-baceous unit, hidradenitis suppurativa. In Cunliffe W (ed) Acne. Martin Dunitz, London, pp 87-92 3. Drapier-Faure E (1998) Traitement hormonal des hy-perandrogenies. Reprod Hum Hormones 11 80-8 4. Farrell AM, Randall VA, Vafaee T, Dawber RPR (1999) Finasteride as a therapy for hidradenitis suppurativa. Br J...

Reporting the Viewpoint of Physicians Uniform Outcome Variables

The measures of the QoL must be correlated with the severity of the disease. Recently suggestions for uniform outcome variables for treatment effects in HS have been reported 7 1. Anatomical region involved (axilla, groin, gluteal or other region or inframammary region, left and or right 3 points per region involved) 2. Number and scores of lesions (abscesses, nodules, fistulas, scars points per lesion of all regions involved nodules abscesses 2 fistulas 4 scars 1 others 1) 3. The longest...

Defensins

The expression of human -defensin-1 (hBD-1), the first isolated human -defensin, is constitutive in epidermal keratinocytes and shows antimicrobial activity against predominantly Gramnegative bacteria such as Escherichia coli (E. coli) and Pseudomonas aeruginosa (P. aeruginosa) 3, 20 . The constitutive expression of hBD-1 in the suprabasal layers of the epidermis suggests that it contributes to the innate resistance of the skin to Gram-negative infections. The second human -defensin, hBD-2, was...

John

I've been having trouble with hidradenitis since my teens, I'm 44 now. Over the years it seems to flare up and give me trouble for extended periods and then subside, sometimes for a year or more with no trouble, except for mild acne mostly on my back. For the first 10 years or so I didn't know what the problem was except that I would occasionally have to have a flare-up lanced. I just assumed they were boils and just the nature of my skin. Nobody (doctors) said anything. Then one time when I...

Acknowledgements

Anders Johannesson for his valuable comments about previous versions of the manuscript, Dr. Karin Sartorius for useful advice, Dr. Zoe and Francis P. Walsh for linguistic revision and Gun-Britt Karlberg and Barbro Andersen for their help. This work was supported by grants from Karolinska Institutet, the Edvard Welander Foundation, Finsen Foundation and the Foundation for Cancer and Allergy Research. No exact estimates of the costs have been made but, but there are at least three...

Linkage of Hidradenitis Suppurativa to 6q252

The genome-wide linkage screen using 400 flu-orescently labelled primers on a number of families resulted in two candidate regions for HS susceptibility. These two regions were found on separate chromosomes indicating that HS is a genetically heterogeneous disease 64 . The first area of linkage was found to the long arm on chromosome 6 (band 6q25.2). Linkage was originally found in Family M with three markers after screening all affected and unaffected members. As HS can have a variable...

Practical Hints and Comments

22.6.3.1 The Carbon Dioxide Laser Technique The poor prognosis regarding complete healing in long-standing cases of HS and the need for prompt surgical intervention encouraged us to develop a fast and convenient surgical method suitable for an outpatient setting that was radical but at the same time tissue-sparing and that had minimally priced post-operative professional wound care. Carbon dioxide laser has previously been used in HS treatment 18, 24, 40 . Before there was access to scanner...

Crohns Disease and Hidradenitis Suppurativa CoOccurrence A Rationale for AntiTNF Therapy

With disruption of the immune system's ability to compartmentalize the bacilli in granulomas and inhibition of macrophage apoptosis. Thus the FDA requires that patients have a negative purified protein derivative (PPD) test prior to the onset of infliximab therapy. Infusion-associated anaphylactoid reactions, while rare, can be avoided by slowing the infusion rate and pre-treating the patients with antihistamines and steroids. Antibody formation can be seen with long-term use and is inversely...

Familyhistory negative or equivocal in previous study Family

11 4 111 1 111 2 111 3 111 4 111 5 Fig. 10.1a, b. b Pedigrees of families without evidence of family history in prevoius study The genetic basis of many common genodermatoses has now been identified. By 2003 over 350 single-gene skin disorders had been characterized at a molecular level 41 . These represent only a fraction of the well over 1000 single-gene disorders now known 1 . The X-linked Duch-enne muscular dystrophy locus was the first disease to be mapped. It was mapped to Xp21 in 1982...

Practical Questions

Can one use sanitary tampons Yes HS lesions do not penetrate the vagina. 2. Should linen be washed separately No this disease is not infective. 3. Is it possible to shave or remove hair by waxing There are no contraindications. However, some patients experience further inflammation following depilation others benefit. You just have to try. 4. Is it better to take a bath or a shower It makes no difference. 5. What soap should I use is an anti-septic soap advisable Any soap will do it is not...

Nassim

I really understand why you are asking for bios, I sometimes find it hard to share even with my family I am 18 years old female from Turkey (so excuse me if my English is sometimes not good enough) I have had 3 surgeries until now, the first one 5 years ago from both of my armpits. And the others at anal zone. But I've been informed that I have this illness only 6 months ago. I've used a lot of antibiotics, only Tetra-dox was a little bit useful for me. I am using Roaccutane since 2 months...

Conclusions

The histological spectrum of HS is broad. The disease appears to be predominantly follicular and apocrine glands appear to be primarily involved in only a minority of histological specimens. Although inflammation does not appear to originate within the apocrine glands, the exclusive finding of the disease within apocrine-gland-bearing skin indicates an apocrine effect. This diversity of pathological presentation may well explain the therapeutic challenge that this condition poses.

Incidence and Prevalence

It is very difficult to find reliable figures about the incidence or prevalence of squamous cell carcinoma in HS patients since there are very few publications describing follow-up of HS patients. Mostly isolated didactic cases are described, which increase clinical vigilance but do not provide predictive data. The risk of squa-mous cell carcinoma in HS has been estimated at between 1.7 and 3.2 52 . Case reports however also allow the reader to form a clinical picture of a given problem when...

Reporting the Viewpoint of Patients Methods of Measurement of Quality of Life

The techniques used to measure QoL are questionnaire based 4 . These measures either cover all ways in which patients' lives can be affected by any disease, or are more specific to diseases of systems or individual diseases. In the absence of questionnaires specific to HS, two kinds can be used general health questionnaires and skin-disease-specific questionnaires. The questionnaires allow the calculation of either global scores, or a profile with a score for each dimension. In our experience...

Excision Margins

The choice of the surgical borders for radical excision is important. Usually the excision reaches the deep subcutaneous fat or the fascia. Three types of excisional limits can be discussed (after Soldin et al. 63 ) 1. Limited local excision. Only the obviously diseased tissue is excised. Usually this is within the area of the hair-bearing skin. 2. Hair-bearing skin excision - the area of axillary tissue containing terminal hair is excised. The excision is performed down to the fascia. 3. Wide...

What Can We Achieve With Therapy

The obvious goal of therapy in HS is to heal existing lesions and prevent the development of new lesions. This is potentially possible in early lesions (Hurley stage I), where scarring does not dominate the clinical picture, although this form of treatment is more likely to alleviate symptoms than to cure patients. Therapy may furthermore not influence established fibrosis, although intralesional steroids, for example, are commonly used to treat hypertrophic scars. Immunosuppression however has...

Antimicrobial Peptides

Activation of TLRs, expressed by epidermal keratinocytes, is directly involved in the induction of antimicrobial peptides 21, 55 . This diverse family of small, mostly cationic polypeptides exerts a broad spectrum of cytotoxic activity against bacteria, fungi, parasites, and enveloped viruses. During the inflammatory processes of the skin, keratinocytes are the main cellular sources of antimicrobial peptides and their expression levels correlate with the susceptibility of the skin to...

Anatomical Regions Involved

The choice of surgical techniques varies according to region. Wide excision surgery gives good symptomatic control of severe HS of the axilla and perianal regions but higher recurrence rates in the inguino-perineal region and it is less satisfactory for submammary disease 63 . Despite a high recurrence rate most patients are well satisfied after inguino-perineal excision because local recurrences are trivial compared with the disease before surgery. Primary closure can be more effective with...

Kerrie

My first outbreak, if you can call it that, showed up right before a vacation to Florida, which is why I remember it so clearly. I wore shorts at the beach as opposed to a bathing suit. It started then with one swollen red painful lump on the left side of my genital region. Since then, it's spread a bit. They are on both sides now - about 6 or more each side with little tunnels running between them. Around the time of my period one side or the other, rarely both, will become severely inflamed...

Pilonidal Sinus

Many HS patients mention previous surgery for supposed pilonidal sinus, with a tendency to recur several times. Clinically it is very difficult to be sure that these pilonidal sinuses are not in fact localized HS. It is particularly difficult to distinguish the two if histopathology has not been performed and if the patient keeps having abscesses in the gluteal cleft. The differential diagnosis is further complicated by the fact that pilonidal sinus is most often treated by surgeons, whereas HS...

Prognosis

The absence of large, well-defined cohorts precludes precise assessment of survival figures. The reported follow-up varies between 18 days and 4 years. In the early publications, diagnosis was usually very late and a rate of 50 death was seen 82 . In more recent literature this appears to have improved significantly, most likely because of the general quality improvements in health care and education. Undoubtedly the dermatological community is now more aware of this potential complication, and...

Elizabeth

I'm 42 years old (43 next month) and have been getting lesions all my adult life. It's been much worse in the past 5 years - I always have 6-10 active abscesses. Areas affected - mostly in my groin and under my breasts. I hadn't had one in my armpits for years, but guess where I feel one starting now (We all know that old feeling, don't we ) My dermatologist and I are presently trying different antibiotics - so far erythromy-cin and minocycline have failed to make the slightest difference. I'm...

Pro Inflammatory Cytokines

Upon challenge with microbial compounds ke-ratinocytes express numerous cytokines acting as cytoprotective factors in the processes of the immune response. In primary keratinocytes, S. aureus-derived PGN induces the secretion of granulocyte-macrophage colony-stimulating factor (GM-CSF) 47 , a cytokine essential for the survival, differentiation, and maturation of dendritic and Langerhans cells. The effects of GM-CSF on the antigen-presenting cells shift the immune response to the Th2-type. Its...

Crohns Disease

Pilonidal sinus - CK17 was absent in type A epithelium (original magnification x100) hyperkeratosis of the infundibulum with plugging and dilatation of the follicle (Fig. 4.8). Superficially the sinus is often lined with stratified squamous epithelium but towards the deeper reaches the wall consists of granulation and scar tissue. The early inflammatory event is perifol-liculitis with neutrophils, lymphocytes and his-tiocytes leading to rupture of follicular epithelium. In a recent...

Cytokeratin Expression in HS

Cytokeratin (CK) is an important marker for evaluating the origin and state of differentiation of epithelial cells. CK17 (found in normal in-fundibulum, Fig. 4.6) has been shown to be absent from infundibular-like keratinized epithelium from HS lesions. This suggests fragility of the draining sinus epithelial, which may allow rupture to occur more easily, thus resulting in a subcutaneous abscess. Keratin expression in non-infundibular keratinized and non-keratin-ized epithelium of HS lesions...

Cyproterone Acetate

In 1986 Mortimer et al. 9 published the first and only study of the effects of CPA in women with HS. This was a double-blind controlled cross-over trial of EE 50 g CPA 50 mg compared to an ordinary OC in combination with EE 50 g norgestrel 500 g, in 24 female patients for 12 cycles. They reported substantial improvement in disease activity with both treatments. In fact, only 18 patients out of 24 completed the trial and only 12 patients improved, while 4 deteriorated. No clinically significant...

General Requirements for Treatment

The difficulties of treatment mean that there is a strong need for additional randomized controlled trials in HS therapy. Such trials however require data for planning, and any successful treatment regimens should therefore be reported. This requires individual physicians to keep a strict log of standardized outcomes, i.e. Sartori-us score, pain VAS, number of flares, etc. in order to enumerate the results obtained. With improved reporting sufficient data may become available for a better...

Gluteal PerianalPerineal Disease

Grafting is associated with high failure rates in the perineal and perianal areas and is not advised for closure of the anal canal either. Grafts in this area may contract, leading to anal stenosis 12 . Some authors argue that cases with extensive tissue resection over the buttocks and perianal region benefit from skin grafting 8, 71 , while others find that the overwhelming majority of perineal resections do well with closure by secondary intention 21 . Simple wide excision, early discharge,...

Psychological Aspects

Is stress a factor that may cause or aggravate the illness In many people stress may contribute to a worsening of the symptoms, but stress not a cause of the disease. 2. How do I break the news to my partner There is no recipe for this This question and the next two require careful consideration. 3. How do I tell the family See Question 2 above. The same answer. But in any case you should make it clear that there is no risk to anyone else. It is also important to have someone with whom you can...

Two Main Zones

The axillary and the inguino-crural zones are the two main areas involved in HS 1, 2, 7 . The diagnosis is often most obvious in the axillary region. Involvement of axillae may extend to the lateral part of the chest (see Fig. 3.14) and sometimes achieve connection with lesions on the breast, where they may be misdiagnosed as a breast abscess. Rope-like hypertrophic scarring is particularly prominent in advanced axillary lesions. Inguino-crural or groin involvement is more frequent than...

Keratolytics

HS is a disease of the hair follicle with histological signs of poral occlusion 9-11 . Although there are only a few publications about the use of keratolytics in HS 12, 13 , the follicular occlusion brought about by hyperkeratosis may be a therapeutic target. Therefore, by analogy to acne, this also appears to be a good target for keratolytics. In acne, another disease of the follicle with abnormal keratinization (microcom-edo), the mainstay in classical therapy of early lesions is topical...

And Innate Immune Responses of the Epidermis

Normal human skin supports the growth of resident microbiota and it is colonized with a wide variety of resident microorganisms. In addition to normal flora, the skin is constantly challenged by pathogens, most of which do not cause clinical symptoms. Besides microbial adherence and virulence, environmental and local factors as well as host immunity are important components of cutaneous infections. In particular, skin becomes more susceptible to infections when the epidermal barrier function is...

Androgenization

One of the less sex-specific effects of androgens is that on the skin and its appendages, and in particular their action on the pilosebaceous unit. Hirsutism is the major symptom of hyper-androgenism in women. Other dermatological conditions include acne and the chronic hair loss usually termed androgenic alopecia (AGA). Whereas acne, hirsutism and chronic hair loss may coexist in the same patient, it is not unusual to find only one of these androgenic manifestations 16 . Depending on the...

Deborah

I have suffered with hidradenitis since I was about 10. Many doctors told me over the years that I had boils. These were very deep, open holes, leaving extensive scaring. They put me on many different medications, none of them worked so I went the surgery route. I have had 4 surgeries with my first in 1993. In '94 I had my arms and legs done. My legs alone required 80 stitches My last surgery involved my groin area and they re-did my arms. Now I'm having...

Background

Similar to most chronic diseases in the current medical milieu, there is a constant interplay between therapeutics and pathophysiology. With the development of novel therapeutic agents with specific mechanisms of action and the knowledge of such mechanisms, new inroads have been made into our understanding of the pathophysiology of several diseases. This in turn has spawned further development of medical pharmacological therapies. The closer we get to understanding the molecular mechanisms of a...

Cathelicidins

LL-37 (CAP18), the only human antimicrobial peptide that has been identified in the cathelici-din gene family, is produced in neutrophils and also induced in keratinocytes during inflammatory skin disorders 17, 43 . In vivo, LL-37 provides protection against necrotic skin infection caused by group A streptococci and it also exerts antimicrobial activity against a wide variety of Gram-positive and Gram-negative bacteria 52 . Similar to defensins, LL-37 plays multiple roles in the fight against...

Differential Diagnoses

To the untrained eye the main impression of an acute lesion is the inflammatory, focal process. The most frequent mistake is therefore a failure to recognize what appears as a common abscess as a manifestation of HS. Carbuncles, furunculosis, lymphadenitis, infected Bartholin's gland, and the infected epidermal cyst are therefore to be differentiated from early lesions 3 . Carbuncles and furunculosis and other staphylococcal skin infections are often asymmetrical in distribution and by their...

Treatment Regimens

In general, the earlier the treatment begins, the better the outcome. Another aspect of the treatment regimen to consider is the dose, which ideally should be as small as possible 1, 2 for the acute stage of HS. Fischer 3 called this R ntgenkleinstdosen (very small R ntgen dose), namely a treatment schedule in which only 0.05- to 0.2-Gy single doses are used. The total doses of these series amount to between 1 and 4 Gy. If the HS is chronic and recurrent, the success rate is not as good, and...

Practical Guidelines for Testing New Physical Therapies

The option of randomized controlled trials is generally not available for surgical techniques. In lieu of the randomized controlled trial a lower degree of evidence is therefore acceptable. The key features of any investigations should remain the randomization, which has independent value even when assessed in an open or a single-blinded fashion. The central problem is one of control, but in an exploratory period abstention from active treatment is enough. In future cases sham procedures may be...

Axillary HS

Digital examination of the anus and rectum should be performed in perianal HS to diagnose anal fistula 16 . The diagnosis is based on the presence of an indurate fistula in the anal canal with the primary lesion of the anal gland palpated close to the dentate line. Fistulas connecting with deep organs such as the anal canal, rectum or urethra have been rarely mentioned in the literature. Regarding perianal surgery, some authors prefer the use of a colostomy to...

Rheumatological Disorders

Inflammatory dermatoses such as psoriasis have well-known rheumatological associations and often lead to collaboration between dermatologists and rheumatologists for the benefit of the patient. Similarly pustular diseases have well recognized rheumatological aspects, e.g. SAPHO syndrome, where SAPHO stands for the combination of synovitis, acne, pustulosis, hyperostosis and osteitis. The association between HS and rheumatological disorders has been less well studied, although a better...

Recurrent Disease After Surgery

Niently coagulated by the laser and little or no bleeding occurs during the surgical procedure. Bleeding from vessels larger than 0.5-1 mm in diameter is preferably stopped using electroco-agulation or ligation. The treatment is terminated by a final soak with 0.9 sodium chloride solution and examination for remaining diseased areas and bleeding. The wound, left to heal by secondary intention, is immediately covered after the surgical procedure with dry dressings or ointment-impregnated...

Treatment

Is there one particular treatment that is successful 2. Should I take anti-inflammatory drugs Anti-inflammatory drugs may reduce pain in the short term there are no indications to the contrary. Their only benefit is to relieve pain. They are sometimes suspected of encouraging the spread of, or aggravating, infections, but this has never been convincingly demonstrated. However, it is advisable to be careful. Isotretinoin (Roaccutane , Curacne) is almost never effective. One can try retinoids for...

General Factors About Bacterial Involvement in HS Pathogenicity

The series of events in HS pathogenesis are unclear and the exact role of bacteria in the etiology of the disease remains controversial. Shelley and Cahn 49 were able to reproduce HS lesions by applying atropine-impregnated adhesive tape to a manually depilated axillary region. They noticed subsequent dilatation, inflammation, and bacterial invasion of the apocrine duct and concluded that HS is a bacterial infection of an obstructed apocrine sweat gland with the causative bacteria deriving from...

BS2 Intermediate Course of the Disease

A large variation in clinical picture can be observed, depending on the several different In this stage of the disease long-lasting suppuration has previously led some patients to complications such as anaemia, hypoprotein-emia and amyloidosis. This kind of wasting syndrome is generally not seen today however, other complications may occur. Carcinoma may occur, particularly on the buttocks. These cancers appear to have a particularly bad prognosis, possibly because of the local immunodeficiency...

Photodynamic Therapy

Photodynamic therapy (PDT) provides semi-selective tissue destruction and a general antimicrobial effect. It is currently used extensively for the treatment of precancerous lesions or non-melanoma skin cancer 2 . The treatment is based on the activation of a photosensitizer applied to the tissue (usually absorbed through the skin surface), and subsequent tissue destruction. The photosensitizer most commonly used at the time of writing is either aminolevulinic acid (ALA, 20 cream) or esterified...

HS and Biological Hyperandrogenism

Furthermore, as far as serum levels of andro-gens and SHBG are concerned, there is no clear evidence for biochemical hyperandrogenism. On average, androgen levels (total plasma testosterone and free testosterone index due to a low SHBG) were increased, but were normal in many individual patients, and no significant decrease of SHBG could be detected 13 . In fact, SHBG is known to be regulated by factors that influence body weight and this study was not controlled for body weight, and neither...

James J Leyden MD

University Hospital of Pennsylvania Department of Dermatology Spruce Street 3400 19104 Philadelphia, USA Library of Congress Control Number 2006928941 ISBN-10 3-540-33100-X Springer Berlin Heidelberg New York ISBN-13 978-3-540-33100-1 Springer Berlin Heidelberg New York This work is subject to copyright. All rights are reserved, whether the whole or part of the material is concerned, specifically the rights of translation, reprinting, reuse of illustrations, recitation, broadcasting,...

Treatment of Hidradenitis Suppurativa Hurley Stage I

This most limited form of disease is the most amenable to medical therapy, and can often be held in check by prophylactic treatment. In Stage I disease the patients often have only a few flares per year. Treatment is therefore most often aimed at reducing the duration of the flares (Table 25.2), and may consist of Table 25.2. Treatment of HS Hurley Stage I disease Clindamycin 1 (lotion) Clindamycin 2 (cream) Resorcinol Short course systemic treatment Tetracycline Erythromycin or other...

Topography

Lateral thoracic extension of axillary involvement Fig. 3.14. Lateral thoracic extension of axillary involvement The typical distribution of HS lesions closely corresponds to the anatomical localization of apocrine sweat glands armpits and the groin. Lesions also occur in a line connecting these two regions passing by the breast and extending to the anal zone. Fig. 3.15. Pubic and inner thigh involvement Fig. 3.15. Pubic and inner thigh involvement In some patients the...

Altered Self Reported Health Qualitative Approach

Many dermatologists rate HS as a heart-sink condition and would agree that it ranks among the most unpleasant of skin diseases. When looking at patients' statements about their disease they emphasize that the soreness and pain are the cause of their disability. Patients suffer from embarrassment and self-consciousness caused by the frequent occurrence of boils with malodorous discharge. One study in Denmark has shown impairment of self-reported health in HS 6 the general self-reported level of...

Preface

This is the first and most comprehensive book dedicated to the understanding and treatment of hidradenitis suppurativa. The most recent monograph on this disease was Dr Benedek's supplement to Acta Dermatovenerologica in 1957 (1) The long interval has not been justified by a benign nature or quiet disposition of the disease. This is a common and debilitating disease that significantly reduces the quality of life for patients. In spite of this it has not achieved a deserved level notoriety and...

Severity Indexes

Due to the large spectrum of clinical severity, to the severe repercussions on quality of life and to the variety of treatments available, a reliable method for evaluating disease severity is needed. Ideally it should take into account the number, type and size of lesions, evolution, pain and repercussions for quality of life. Such a comprehensive instrument does not yet exist, but two attempts have been proposed to classify patients according to severity. This is historically the first...

Quality of Life in Hidradenitis Suppurativa

14.2.1 Why Try to Measure Quality 14.2.2 Reporting the Viewpoint of Patients Methods of Measurement of Physicians Uniform Outcome Variables 117 14.2.4 Practical Approach Reconciling the Viewpoints of Patients 14.3 Impact of HS upon Quality of Life 117 14.3.1 Altered Self-Reported Health Qualitative Approach 117 14.3.2 Measure of Quality of Life Quantitative Approach 118 14.4.1 Conclusion for Patients 118 14.4.2 Conclusion for Physicians 119 15.2 Clinical Experience 121 16.1 I ntroduction 124...

Side Effects

The side-effects of isotretinoin are very numerous 2, 30-32 . In the studies mentioned, no serious side-effects are reported. In patients who are treated with isotretinoin (mostly for 16 weeks), liver function tests and determination of lipid profiles have to be performed at baseline and on one occasion after 4 weeks 31 . Teratogenicity is by far the most serious of all the side-effects of retinoids and requires responsible prescribing by physicians and reliable patients 31, 32 . Women of...

Therapeutic Aspects and Prognosis

Flare-up episodes are usually treated with aminosalicylates or prednisolone, according to their clinical severity. When steroid therapy fails (10 -30 of patients), the anti-tumor necrosis factor-alpha antibody infliximab 5 mg kg is usually active. Maintenance treatment prescribed with the goal of preventing flare-ups uses aminosalicylates and, in more severe forms, immunosuppressive drugs (azathioprine or methotrexate). Immunosuppressants are very effective as maintenance treatment, achieving...

Clinical Presentation

Symptoms depend on disease location (colonic lesions being more expressive) and anatomical severity. Patients usually present with diarrhea, mucous and blood discharges, abdominal pain, and weight loss. Other features of flares are fatigue, fever, articular, cutaneous and ocular manifestations, and biological inflammatory response (elevated C-reactive protein and eryth-rocyte sedimentation rate, ESR). In some cases, the disease is latent until the development of a complication, a stricture...

Delay in Diagnosis

The varying sites involved and the non-specific nature of early lesions mean that patients with HS are referred to many different specialists, including general surgeons, gynaecologists, plastic surgeons, dermatologists, infectious medicine specialists, immunologists, gastroen-terologists, proctologists and urologists. This occurs mainly because lesions are most often considered to be common abscesses requiring treatment with short courses of antibiotics and lancing, which is an ineffectual...

Treatment of Hidradenitis Suppurativa Hurley Stage III

No curative effect can be expected with medical therapy in Stage III disease. All medical therapy therefore aims to be palliative and temporary, i.e., the disease recurs shortly after the treat ment is stopped. The antibiotic combination of clindamycin and rifampicin may also be helpful. An alternative viable strategy for gaining better control of the disease at this stage may be an immunosuppressive therapy. Traditionally corticosteroids and ciclosporin have been used. Recently the use of...

Members of the TLR Family Expressed by Keratinocytes

Since Gram-positive skin-infecting bacteria such as Staphylococcus aureus (S. aureus) or Borrelia burgdorferi (B. burgdorferi) are known to be rich sources of LTA and lipoproteins, which are well-known ligands of TLR2 homodi-mers (Table 13.1), the abundant and constitutive expression of TLR2 in the epidermis is not surprising 36, 49, 59 . TLR2 homodimers are also involved in the recognition of lipoarabino-mannan of mycobacteria and atypical lipopoly-saccharides of Leptospira and Porphyromonas...

Of the Skin Folds Dowling Degos and Kitamuras Diseases

Dowling-Degos disease (DDD) or dark dot disease and Kitamura's reticulate acropigmenta-tion are both rare pigmentation genodermatoses that have been described in association with HS. DDD was first described by Dowling and Freudenthal in 1938 35 , then by Degos and Os-sipowski in 1954 36 . It is characterized by a reticulate pigmentation of the flexures with prominent comedo-like lesions and pitted scars 37-56 . The disease runs in families, it has a genetic autosomal dominant transmission 42,...

Hidradenitis Suppurativa and Crohns Disease

Philippe Seksik, Jean-Fran ois Contou, Anne Cosnes and Jacques Cosnes 7.1.1.1 Genetic Factors 51 7.1.1.2 Enteric Microflora 51 7.1.1.3 Immune-Mediated Tissue Injury . . 51 7.2 Pathology 51 7.3 Clinical Aspects 52 7.3.1 Disease Location 52 7.3.2 Clinical Presentation 52 7.4 Therapeutic Aspects and Prognosis 52 7.5 Cutaneous Crohn's Disease 52 7.5.2 Therapeutic Aspects and Prognosis . 53 and Hidradenitis Suppurativa . . . .53 7.6.1 Differential Diagnosis 53 7.7 Co-Existence of HS and CD 55 7.8...

Other CoOccurrences

These other co-occurrences may be pure coincidences, but they may also give us clues to a better understanding of the mechanisms of this disease. HS is occasionally seen as a side-effect to other treatments (see Table 6.4). HS has been described in several cases in association with lithium therapy, where it appears to have similarities to other poral occlusive diseases, as well as to immunosuppressive therapy used following kidney transplantation. HS has also been described as a side-effect of...

Practical Use of Immunosuppressive Therapy

Immunosuppressive therapy can be used at all stages of the disease for the benefit of the patient. Essentially it may be directed at two different aspects of the disease disease progression and flares. When used to treat disease progression it is a long-term therapy aimed at controlling the inflammation-induced fibrosis of the tissue, and helping the patient to take more control of their disease. The treatment should be continued for at least 3 months with monitoring of the effect as well as...

Experimental Therapies

Often standard therapies fail, and both patient and physician are left with a therapeutic challenge. Most often one of two general approaches is used in off-label experimental therapy. One avenue of approach is to regard the disease as a predominantly inflammatory disease, and consequently treat with immunosuppressive drugs as in so many other dermatological diseases. The use of topical immunosuppressants is rarely sufficient, and systemic therapy has been...

Melony

Hello I am 38 years old and I have had Hidrad-enitis Suppurativa since puberty. I get these cysts on my buttocks, inside of my thighs, and the groin area. I am very discouraged with this disease, as you probably know, there is no cure. That is what the doctors have told me. I have been on every kind of medication even Accu-tane. I have had surgery on my buttocks, have had some of the glands taken out, however, the cysts will come back as they cannot take every gland out of your body. I have had...

Isotretinoin in Combination Treatment of HS

One author reported on a patient with Crohn's disease and HS who showed a satisfactory outcome following treatment with azathioprine (150 mg day) and methylprednisolone (16 mg day) combined with isotretinoin (0.7 mg kg per day) and periodic administration of antibiotics 28 . Another report details a patient with multiple pustular and cystic lesions located on swollen and red labia majora. She was successfully treated with prednisolone and erythromycin for months and then long-term isotretinoin...

Pregnancy

How does the illness develop during pregnancy There is often an improvement during pregnancy, but this is not always the case. 2. Is there a risk to the baby during birth The risk is practically nonexistent. There may be a risk of bacterial infection if there is serious and persistent suppuration at the time of delivery. But in this case a caesarean delivery is always an option, or otherwise it is possible to prevent the infection of the child with antibiotics. In any event there is no serious...

Keratinocyte Derived Effector Molecules in the Innate Immune System of the Skin

Sociates with tumor necrosis factor (TNF) receptor-associated factor 6 (TRAF6), leading to the activation of at least two distinct signaling pathways, JNK and NF-kB. TLR signaling through MyD88 leads to the phosphorylation and degradation of IkB, the regulator protein of NF-kB, an event allowing the nuclear translocation of NF-kB (Fig. 13.1). In the nucleus NF-kB binds to the promoter region of genes of proin-flammatory cytokines chemokines, antimicrobial peptides, inducible enzymes, and...

Complications

Complications include suture dehiscence, wound separation, postoperative bleeding, hematoma, and loss of flap or skin graft. Local wound and septic infections are rare and not prevented by preoperative antibiotics. The late sequelae involve different types of problems related to scar formation, especially when in the axilla, and on occasion require release of the contracture or even Z-plasty. In the perineum, scar contractures occur quite regularly but present little functional disability. When...

Depilation

Theoretically depilation could offer some relief to patients with HS. Previously X-ray therapy has been used to treat HS 7, 8 . The dosage varied, but often successful therapy induced depilation of the treated areas. Earlier papers on the topic have suggested that the positive effects were due as much to depilation as to the anti-inflammatory effects of the treatment (Chap. 23). Such an effect may be hypothesized from the histology of the early lesions. An element of fol-licular occlusion is...

Diagnosis

No diagnostic paraclinical tests are currently available and a biopsy is rarely required to exclude an alternative diagnosis. Frequently, and especially in benign cases, the diagnosis has to be established primarily on patient history, i.e. more on the questioning of the patient than on objective data gathered by examination. In atypical cases - e.g. buttock involvement alone - the chronicity of the inflammatory and suppurative process is a cornerstone of...

Clinical Course

Chronicity is one of the main features of HS. In a questionnaire survey of 110 patients Von der Werth and Williams reported that in their population - mean age 40 years - the average duration of the disease had been 18.8 years and 98 110 still had active disease 12 . This is indeed a heart sink condition for the patient. Furthermore, there is an extreme variation in the severity of HS. In a personal series of 164 patients 1 , 76 were in Hurley's stage I, 20 in stage II and 4 in stage III. Most...

Squamous Cell Carcinoma

Many chronic suppurative lesions such as chronic osteomyelitis and chronic leg ulcers are well known for their potential for malignant transformation into squamous cell carcinoma 69, 70 . The resultant tumours are known as Marjolin's ulcers, named after Marjolin's first description in 1826 87 . Such tumours have been described in HS. scribed in women 5, 53, 54, 74, 85, 88, 91, 92 . This may however partly be explained by reporting bias. For instance, vulval squamous cell carcinomas may well be...

Why Try to Measure Quality of Life

Quality of life is a concept incorporating all factors that impact upon an individual's life 4 . The concept has been divided into several components, including psychological, social, and physical domains. All clinicians use an intuitive view of how much the disease is affecting their patients when taking management decisions, but patients may assess QoL differently from their doctors. The use of simple QoL measures is usually welcomed by patients who wish to express their concerns. Methods of...

Healing

Zinc's efficacy in Verneuil's disease is probably due to its anti-inflammatory activity. It has activity against various targets implicated in the cutaneous inflammatory reaction and the mechanism of action is still only partially understood. As for non-specific immunity, 30 mg zinc metal 3 inhibits the chemotactic migration of granulocytes, both in vitro and in vivo. It activates natural killer cells and the phagocytic function of granulocytes 4 . Zinc also inhibits the expression of integrins...

Associated Diseases Causality or Complications

Jemec 6.2 Cutaneous Diseases (Excluding Squamous Cell Carcinomas) 39 6.2.1 Follicular Occlusion Diseases 39 6.2.1.1 Acne Conglobata 40 6.2.1.2 Dissecting Folliculitis of the Scalp .40 6.2.1.3 Pilonidal Sinus 41 of the Skin Folds Dowling-Degos and Kitamura's Diseases 41 6.3 Rheumatological Disorders 42 6.3.1 Clinical Picture 42 6.3.2 Radiographic Features 42 6.3.3 Treatment 42 6.4 Associated Cancers 43 6.4.1 Non-Skin Cancers 43 6.4.2 Squamous Cell Carcinoma 44...

Relations To Other Factors

Is articular (arthritic) pain related to the illness Articular pain can be related to the disease, but this is not true for all articular pains. It is known that some rheumatic inflammations may accompany diseases such as suppurating hidra-denitis and that these joint symptoms may be either peripheral (such as arms, legs, fingers) or in some cases central (such as pelvis or back). 2. Does tobacco provoke or aggravate the disease It has been demonstrated that HS is more prevalent among smokers...

Topical Anti Inflammatory Therapy

No formal studies have been published on the use of topical anti-inflammatory treatment of HS. Experience nevertheless suggests that some preparations may prove useful to selected patients. The preparations tried are azelaic and fusidic acid, the latter both alone and in combination with topical corticosteroids (personal observation, G.B.E. Jemec). Azelaic acid occurs naturally on the skin. It is a straight-chained nine-carbon-atom dicarbox-ylic acid, which is non-toxic, non-teratogenic, and...

Info

The elementary lesions of perianal Crohn's disease (CD, posterior fissure, skin tags, and fistulas) Fig. 7.1. The elementary lesions of perianal Crohn's disease (CD, posterior fissure, skin tags, and fistulas) Fig. 7.2. A CD fissure merging on the perianal skin Fig. 7.4. HS lesions in a patient with operated perianal CD Fig. 7.3. A suppurative form of perianal CD with multiple fistulas Fig. 7.4. HS lesions in a patient with operated perianal CD anal lesions may be absent or have...

S4 XRay Examination

The use of imaging techniques is not widely developed in dermatology, most likely because the skin is immediately available for inspection and clinical examination. Imaging techniques, however, do have a role to play in the examination of skin diseases, in particular in diseases that may spread to deeper tissues. HS is an excellent example of such a disease. Different techniques are available (see Table 5.1) and each method has different advantages, although traditional x-ray examination...

And Antiandrogen Therapies

Antiandrogens are molecules that bind the androgen receptor (AR) and act as androgen antagonists at the target cell level. Cyproterone acetate (CPA) and spironolactone are the most commonly used androgenic antagonists 3, 12 . Additional hormonal treatment of hyperan-drogenism includes (1) inhibition of the conversion of testosterone (T) into its active metabolite dihydrotestosterone (DHT) through 5a-reductase inhibition (2) suppression of ovarian androgen production with oral contraceptives...