Atypical hidradenitis suppurativa

Hidradenitis suppurativa (HS) is a chronic relapsing inflammatory disease resulting in subcutaneous abscesses, sinus tracts and scarring, arising predominantly in apocrine gland‐bearing skin. The sites affected, in order of frequency, are the axillae, groins, perianal and perineal region, mammary and submammary skin, buttocks, and pubic region. Other sites that may be affected more rarely include the chest, eyelids, scalp, retroauricular and preauricular skin, thighs, and abdomen. Research has suggested that mechanical friction and follicular occlusion is the primary cause of HS. HS is one of the conditions of the ‘follicular occlusion tetrad’, which share a similar pathogenesis. HS at atypical sites may mimic other conditions both clinically and pathologically, so misdiagnosis is possible. We present a case series that illustrates a range of atypical sites affected. Our patients all had severe disease. Satisfactory control has not been achieved despite years of medical and surgical interventions.     

Complement in hidradenitis suppurativa

This is a study on the pathogenesis (causes) of hidradenitis suppurativa (HS). HS is a common skin disorder that affects almost 1% of people in Europe. It manifests with boils in areas of the skin rich in sweat glands like the axilla (arm pits), the breast, the groins and the buttocks. These boils progressively enlarge and rupture with the production of pus and blood. Stress usually proceeds this process. This study was undertaken at the University Hospital of Athens in Greece. The authors measured the concentrations of C5a and C5b‐9 in the plasma (blood) of patients with HS. The rationale for their study was that C5a causes the influx of neutrophils and the formation of pus. It was found that C5a and C5b‐9 are largely elevated in HS and that this is associated with HS severity, so higher levels of C5a and C5b‐9 are linked with worse HS symptoms. Interestingly, the authors describe that C5a stimulates the over‐activation of monocytes, which are cells causing inflammation. Based on this finding, the authors suggest that over‐production of C5a is a newly discovered pathway for the pathogenesis of HS. The authors also show how the drug IFX‐1, that selectively blocks C5a function, decreases the activation of monocytes and may become a promising new medicine for the treatment of HS.     

化脓性汗腺炎的治疗

化脓性汗腺炎虽少见,但病程慢性、治疗困难,往往给患者造成较大的痛苦。本文对本病的传统和最新的各种治疗方法,如长期应用抗菌药物、维甲酸、抗雄性激素药物、物理治疗、外科手术和生物制剂等作了介绍,以备临床医师参考。     

Infliximab for hidradenitis suppurativa

BACKGROUND: Hidradenitis suppurativa (HS) is a chronic disease characterized by significant morbidity. Current medical therapies are only minimally effective at treating the disease. Infliximab is a chimeric monoclonal antibody with high affinity for tumour necrosis factor (TNF)-alpha. TNF-alpha is known to induce proinflammatory cytokines and may play an important role in the therapy of a number of disparate inflammatory disorders. Infliximab has shown promise for the therapy of rheumatoid arthritis and psoriasis. OBJECTIVES: Retrospectively to evaluate the effectiveness of infliximab for the treatment of HS. METHODS: A retrospective chart review was performed for patients who received infliximab at the University of Miami Department of Dermatology. Patients were contacted and asked retrospectively to rate their disease activity immediately prior to and after therapy. RESULTS: Patients' self-reported disease activity scores were significantly decreased (P = 0.0001, paired t-test) following infliximab infusion. This correlated with physician-observed clinical improvement. CONCLUSIONS: Infliximab is a promising agent for the treatment of HS. These initial results suggest that infliximab is associated with objective and subjective improvement in HS. Further controlled studies of the efficacy of infliximab and its effect on the course of the disease are warranted.     

Acne and hidradenitis suppurativa

Acne and hidradenitis suppurativa (HS) both centre on hair follicles. They often occur together as part of the acne tetrad, but are found in distinct localizations. Acne is primarily defined by the presence of comedones and inflammatory lesions. However, in HS the intertriginous localization and chronicity play equally important roles for the diagnosis to the inflammatory lesions. Genetics, bacteria, environmental factors and innate inflammation have all been found to play a role in acne and/or HS. Surprisingly, there is little overlap between the findings so far. The genetics of acne and HS are distinct, bacteria have not been shown convincingly to play a role in HS, and the important risk factors obesity and smoking in HS cannot be easily translated to acne. The one driving factor central to both diseases is innate inflammation, most strikingly involving interleukin‐1. Hence the interleukin‐1 family, as already shown in autoinflammatory conditions associated with acne, could represent attractive treatment targets.     

Antibiotic treatment of hidradenitis suppurativa

Bacterial infection plays an important role in hidradenitis suppurativa (HS). This infection has a number of unique features: chronicity, absence of lymph node involvement, and absence in most cases of acute super-infection by Staphylococcus aureus and/or streptococci. Treatment is based mainly on antibiotics. Various bacteria are involved; they are often part of the resident flora and may combine in polymicrobial infections, and they consist mainly of two families: coagulase-negative staphylococci (CNS) and anaerobes. Numerous antibiotics are active against CNS: betalactamins, lincosamides, macrolides, rifampicin, tetracyclines and fluoroquinolones. Antibiotics active against anaerobes include metronidazole, betalactamins, lincosamides and one fluoroquinolone (moxifloxacine). Antibiotics are given per os and in combination. Treatment is usually long-term, with a frequent need for maintenance therapy. It has to be tailored to various clinical situations: intermittent development, in which "abortive" emergency treatment is used; major or major continuous forms, where combined antibiotics are used, most frequently rifampicin and clindamycin. The global treatment strategy involves a surgical approach, which can be aided but not replaced by antibiotics. While the risks of long-term antibiotic use are reduced in this specific population of "healthy" young adults, they are not absent.     

Practical management of hidradenitis suppurativa

Hidradenitis suppurativa is a chronic inflammatory disease that usually affects areas of the skin with a high density of apocrine glands (axillae, groin, perianal and perineal regions, submammary area, etc). It usually appears in women during puberty. The etiology of the disease is unknown, although it is thought to be a primary defect of the hair follicle. When diagnosing hidradenitis suppurativa, a number of genetic and hormonal predisposing factors should be considered alongside other putative triggers, such as obesity, smoking, and tight clothing. Diagnosis is essentially clinical and it is important to bear in mind the possible complications associated with the disease, such as the development of tumors. Management of the disease includes general measures (antiseptic soaps, warm baths, etc), pharmacological therapy (topical, intralesional, and systemic), surgery (direct closure, second-intention healing, grafts, flaps, etc), and other measures (carbon dioxide laser, radiation therapy, etc). It is important that the disease be diagnosed and treated as soon as possible given the potential physical and psychological problems that it can generate.     

Depression in patients with hidradenitis suppurativa

Background  Hidradenitis suppurativa (HS) is a chronic recurrent inflammatory skin disease with abscess formation and scarring predominantly in the inverse areas. The disease is often difficult to treat and patients experience a decreased quality of life (QoL). It is hypothesized that depression is more common in HS patients than among other dermatological patients. Objectives  To evaluate the prevalence of depression in patients with HS. Methods  In total 211 HS patients were included in the study and 233 were dermatological control patients. Their QoL and depression scores were assessed using the Dermatology Life Quality Index (DLQI) and the Major Depression Inventory (MDI) questionnaires. HS severity was recorded with a questionnaire and Hurley stages were extracted from the case records. Results  The DLQI was significantly higher for HS patients than for the control patients, 8.4 ± 7.5 vs. 4.3 ± 5.6 (P < 0.0001) and correlated with Hurley stage severity scores. Mean MDI scores were significantly higher for HS patients, 11.0 vs. 7.2 (P < 0.0001). However, clinically defined depression rates according to the International Classification of Diseases, 10th edition (ICD‐10) criteria were not significantly higher in HS patients compared to controls (9% vs. 6%). Conclusions  HS is a chronic skin disease with major impact on QoL even when compared to other dermatological diseases. MDI scores in HS patients correlate with disease severity. This correlation could indicate that the MDI represents a valid measure of disease related morbidity that may serve as an outcome measure in future studies and a relevant point of intervention for individual patients.     

Ustekinumab treatment for hidradenitis suppurativa

Hidradenitis suppurativa (HS) is a follicular occlusive inflammatory skin disease that occurs in the axilla, groin, buttocks and vulval region. Control of the intractable inflammation is a primary goal of HS treatments. Benefit of anti‐tumor necrosis factor (TNF) antibodies against HS have been reported, and adalimumab has been approved for HS in Europe, the USA and Japan. However, the alternative therapies for anti‐TNF antibodies have not been established yet. We experienced a case of HS which developed during the infliximab treatment for Crohn’s disease (CD) and was well managed by ustekinumab (UST). We reviewed the articles relating to ustekinumab treatments for HS. Twenty‐four HS patients, 16 women and eight men, have been treated with ustekinumab. The average age was 35.7 ± 10.8 years (mean ± SD). All were of Hurley stage II or III. Ten (10/24, 41.6%) had received anti‐TNF drugs including infliximab, adalimumab and etanercept prior to UST treatment for HS. Although the initial doses varied from 45 mg s.c. to 390 mg i.v., all cases were treated with 45 or 90 mg s.c. every 8 or 12 weeks at the regular dose, by following the regimen for psoriasis or CD. HS in most of the cases started to improve after 3–5 months of UST initiation, and some achieved complete remission. To our knowledge, our case is the first Asian HS patient improved by UST. Overall, UST is useful for HS and could be an alternative treatment if HS patients do not respond to other medications including anti‐TNF drugs.     

Morbidity in patients with hidradenitis suppurativa

BACKGROUND: Although skin diseases are often immediately visible to both patients and society, the morbidity they cause is only poorly defined. It has been suggested that quality-of-life measures may be a relevant surrogate measure of skin disease. Hidradenitis suppurativa (HS) leads to painful eruptions and malodorous discharge and is assumed to cause a significant degree of morbidity. The resulting impairment of life quality has not previously been quantitatively assessed, although such an assessment may form a pertinent measure of disease severity in HS. OBJECTIVES: To measure the impairment of life quality in patients with HS. METHODS: In total, 160 patients suffering from HS were approached. The following data were gathered: quality-of-life data (Dermatology Life Quality Index, DLQI questionnaire), basic demographic data, age at onset of the condition and the average number of painful lesions per month. RESULTS: One hundred and fourteen patients participated in the study. The mean +/- SD age of the patients was 40.9 +/- 11.7 years, the mean +/- SD age at onset 21.8 +/- 9.9 years and the mean +/- SD duration of the disease 18.8 +/- 11.4 years. Patients had a mean +/- SD DLQI score of 8.9 +/- 8.3 points. The highest mean score out of the 10 DLQI questions was recorded for question 1, which measures the level of pain, soreness, stinging or itching (mean 1.55 points, median 2 points). Patients experienced a mean of 5.1 lesions per month. CONCLUSIONS: HS causes a high degree of morbidity, with the highest scores obtained for the level of pain caused by the disease. The mean DLQI score for HS was higher than for previously studied skin diseases, and correlated with disease intensity as expressed by lesions per month. This suggests that the DLQI may be a relevant outcome measure in future therapeutic trials in HS.     

Evidence of genetic factors in hidradenitis suppurativa

Twenty-six probands suffering from hidradenitis suppurativa were identified from Hospital Activity Analysis (H.A.A.) records for the period 1980-83 and by direct referral from hospital specialists over a 6-month period in 1983-84. Investigation of their families eventually confirmed a total of 62 affected individuals from 23 families. In II families there was evidence in favour of single gene or Mendelian inheritance; in another three there was familial occurrence; in nine families there was a negative family history at the time of enquiry. The disease appears to be commoner than reports suggest and it is probable that there is an underestimation of affected individuals in our community. Problems of identification including variable age of onset and psycho-social factors were found and may be responsible for false-negative family histories.