Журило Олександр Анатолiйович, доктор медичних наук, лаборатор1я мшробюлоги Державно! установи, Нацюнальний шститут фтиз1атрИ i пульмонологи ím. Ф. Г. Яновського НАМН Укра!ни, вул. Амосова Ми-коли, 10, м. Ки!в, Укра!на, 03680 E-mail: microbio@ifp.kiev.ua
Трофимова Полша CTamcraBiBHa, кандидат медичних наук, науковий ствробгтник, лабораторiя мжробь ологii Державно! установи, Нацюнальний шститут фтизiатрi! i пульмонологи ím. Ф. Г. Яновського НАМН Укра!ни, вул. Амосова Миколи, 10, м. Ки!в, Укра!на, 03680 E-mail: trofimova@ifp.kiev.ua
Алieва Наталiя МиколаТвна, лшар-бактерюлог першо! категори, заввдувачка кл1н1ко-д1агностичною ла-боратор1ею, клшшэ^агностична лаборатор1я, Полтавський обласний протитуберкульозний диспансер, вул. Шиловська, 51-А, м. Полтава, Укра!на, 36028 E-mail: microbio@ifp.kiev.ua
UDC 616.5
DOI: 10.15587/2313-8416.2015.51530
HIDRADENITIS SUPPURATIVA SUCCESSFULLY TREATED WITH ADALIMUMAB © I. Bukhari, A. Al Zahrani
Гнтний г1драден1т (ГГ) - це захворювання шк1ри, що характеризуется формуванням численних к1ст, аб-сцес1в та свщевих тракт1в в зон апокринних залоз. Етюлогя i патогенез ГГ нев1дом1.1снуюч1 медичт та х1рург1чн1 терапевтичнi практики мають мiнiмальний ефект лкування цього захворювання. Бiологiчний зааб Адалмумаб - це новий перспективний зааб лiкування ГГ Ключовi слова: Гнтний гiдраденiт, бюпрепарати, апокринш залози, адалiмумаб
Aim: To discuss the new beneficial effect of adalimumab in the management of hidradenitis suppurativa(HS). Case: We report a 25-year-old arabic female with a 14-year history with long-standing poorly controlled active hidradenitis suppurativa who was successfully treated with adalimumab.
Discussion: Hidradenitis suppurativa is a skin disorder characterized by the formation of multiple cysts, abscesses and sinus tracts in apocrine gland-bearing areas. The aetiology and pathogenesis of HS are unknown. Current medical and surgical therapies are only minimally effective at treating the disease. The biologic agent Adalimumab is a new promising agent for the treatment of HS.
Conclusion: The biologic agent adalimumab is an effective treatment for HS Keywords: Hidradenitis suppurativa, biologics, apocrine glands, adalimumab
1. Introduction
Hidradenitis suppurativa (HS) is a chronic inflammatory disease of the skin characterized by recurrent nodules, abscesses, sinus tract formation and scarring affecting mainly areas rich with apocrine glands including the axillae, groin, buttocks, perianal and submammary regions. The disease initially presents during puberty and is more common in females. Treatment options include oral antibiotics, isotretinoin, finasteride, prednisone, cyclospo-rine, surgery, carbon dioxide laser therapy and radiotherapy. Recently, the efficacy of different biologics has been demonstrated. We report a patients with long-standing active hidradenitis suppurativa who was successfully treated with adalimumab.
2. The case
A 25-year-old arabic female presented with a 14-year history of painful discharging nodules, sinuses and cysts involving axillae (Fig. 1, a), submammary region and groin. Her past medical history was negative ex-
cept for mild acne vulgaris affecting the face. There was no history of inflammatory bowel disease or any other medical illnesses. Physical examination of the axillary areas, submammary and the groins revealed multiple er-ythematous tender nodules with discharging sinuses and abcesses. Some areas were macerated due to the conti-neous severe inflammation. Other areas showed healed hypertrophic scars. Her baseline investigations including complete blood count, liver function tests, renal function tests, lipid profile, anitnuclear antibodies, antidouble stranded DNA, complement 3 and 4 and c reactive protiens were within normal limits. Culture of the discharge from the abcesses was negative. So the patient was diagnosed as a case of HS. Initially, she was treated with oral isotretinoin 1 mg per kg for 5 months with no improvement then a course of oral doxycycline 100 mg once daily for 12 weeks failed to improve the condition. A single course of Infliximab (three intravenous infusions at weeks 0, 2 and 6) was started for 10 weeks with minimal improvement. Over a period of few months
the condition worsened so she was admitted to the surgical ward for wound care and debridement. After a period of one month the patient was started on Adalimumab 80 mg subcutaneous as a loading dose followed by 40 mg every one week for 16 weeks. The condition improved immediately with regard to the lesions size and the discharge with no new lesions appearing. She maintained her response for almost one year but due to unavailability of adalimumab in the hospital the patient discontinued taking it for three months. This time, she presented to the clinic with severe relapse. Adalimumab was restarted with a dose of 160 mg subcutaneous injection as a loading dose followed by 80 mg in the following week then mainatianed on 40 mg weekly with an excellent response (Fig. 1, b). She is being followed up every 8 weeks in the clinic with good control of the lesions.
3. Discussion
Hidradenitis suppurativa (HS) was first described in 1839 by Velpeau [1]. It was classified as a member of the follicular occlusion triad, along with Acne conglobata and dissecting cellulitis of the scalp [2, 3]. In 1975, pilon-idal cyst was added as a member to this triad, forming the follicular occlusion tetrad [4]. The global HS prevalence was reported as 1 % in a review of several studies with female predominance [5-7]. The mode of inheritance of HS remains unclear. Genetic studies have indicated several gene loci on chromosomes 6q25.1-25.2 and 9p12-p13.11 but no causative genes have yet been identified [8]. The disease is essentially limited to areas of the skin that are rich in terminal hair follicles and apocrine glands, such as the axillae, anogenital area, submammary areas and the buttocks [7, 9]. Clinically, it is characterized by recurring pustules, inflammatory nodules, abscesses, draining sinus formation, fibrosis, secondary lymphedema and dou-
ble-ended pseudocomedones. In its severe form it can be a devastating disease with a great impact on the quality of life [10]. HS transformation into SCC has been documented in 41 reports [11-20] with 61 % present at the perineal or buttock area. It is considered as the most severe complication of HS. The diagnosis of HS is primarily made on the basis of its characteristic clinical presentation. A consistent finding in histological studies of HS is follic-ular occlusion due to hyperkeratosis leading to occlusion of the apocrine gland with subsequent follicular rupture, inflammation and possible secondary infection. A reduction in the percentage of NK cells over time and a lower monocyte response to bacterial components was observed in patients with HS [21]. Current treatment of hidradenitis suppurativa consists of topical antibacterial or antiseptic solutions, systemic antimicrobials such as tetracyclines (doxycyclme, minocycline) [22], retinoids, systemic and mtralesional steroids, hormonal therapy, and a wide range of surgical interventions [23]. The beneficial effect of anti-TNF therapies such as infliximab in hidradenitis suppurativa was first reported [24-28]. Subsequently reports with etanercept (Enbrel) and adalimumab (Humira) in hidradenitis suppurativa have been published [29-31 ]. The clinical improvement of HS with an-ti-TNF therapies supports the hypothesis for an altered immune response m these patients [32]. Besides, a reduction in the percentage of natural killer cells over time and a lower monocyte response to triggering by bacterial components is observed m patients with H S [21].
4. Conclusions
This is a case of chronic persistent HS that failed all classical therapies for HS but has shown superior response with adalimumab. Hopefully, future reports will reveal the pathogenesis of HS through the action of these drugs which might lead to discovery of specific new innovative therapies to this debilitating condition.
Acknowledgement
I would like to acknowledge Prof. Christos C. Zou-boulis, Städtisches Klinikum Dessau, Germany for his valuable recommendations.
References
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Fig. 1. hidradenitis suppurativa involving the left axillary area: a - Left axilla with severe inflammation and draining sinuses and hypertrophic scars; b - healing of the lesions after adalimumab treatment
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References
1. Velpeau, A. A.; Bechet J. Z. (Ed.) (1839). Dictionnaire de médecine, un repertoire générale des Sciences Medicals sous le rapport theorique et pratique. Paris, 2, 1839-1891.
2. Brunsting, H. A. (1939). Hidradenitis suppurativa; abscess of apocrine sweat glands a study of the clinical and pathologic features with a report of twenty-two cases and a review of the literature. Archives of Dermatology and Syphilology, 39 (1), 108-120. doi: 10.1001/archderm.1939.01480190111010
3. Pillsbury, D. M., Shelley, W. B., Kligman, A. M.; Pill-sbury, D. M. (Ed.) (1956). Bacterial infections of the skin. Dermatology. Philadelphia: Saunders WB, 482-484.
4. Plewig, G., Kligman, A. M. (1975). Acne: Morphogenesis and Treatment. Berlin: Springer-Verlag, 192-193. doi: 10.1007/978-3-642-96246-2
5. Naldi, L.; Jemec, G., Revuz, J., Leyden, J. (Eds.) (2006). Hidradenitis suppurativa. Springer Berlin Heidelberg, 58-64. doi: 10.1007/978-3-540-33101-8
6. Jemec, G. B. E., Heidenheim, M., Nielsen, N. H. (1996). The prevalence of hidradenitis suppurativa and its potential precursor lesions. Journal of the American Academy of Dermatology, 35 (2), 191-194. doi: 10.1016/s0190-9622(96)90321-7
7. Revuz, J. (2009). Hidradenitis suppurativa. Journal of the European Academy of Dermatology and Venereology, 23 (9), 985-998. doi: 10.1111/j.1468-3083.2009.03356.x
8. Von der Werth, J., Wood, P., Irvine, A. D., McLean, W. H. I. (2006). Genetics of Hidradenitis Suppurativa. Hi-dradenitis Suppurativa, Berlin Heidelberg: Springer, 70-85. doi: 10.1007/978-3-540-33101-8_10
9. Alikhan, A., Lynch, P. J., Eisen, D. B. (2009). Hidradenitis suppurativa: A comprehensive review. Journal of the American Academy of Dermatology, 60 (4), 539-561. doi: 10.1016/ j.jaad.2008.11.911
10. Mekkes, J. R., Bos, J. D. (2007). Long-term efficacy of a single course of infliximab in hidradenitis suppurativa. British Journal of Dermatology, 158 (2), 370-374. doi: 10.1111/j.1365-2133.2007.08332.x
11. Wolkenstein, P., Loundou, A., Barrau, K., Auqui-er, P., Revuz, J. (2007). Quality of life impairment in hidrad-
enitis suppurativa: A study of 61 cases. Journal of the American Academy of Dermatology, 56 (4), 621-623. doi: 10.1016/ j.jaad.2006.08.061
12. Short, K. A., Kalu, G., Mortimer, P. S., Higgins, E. M. (2005). Vulval squamous cell carcinoma arising in chronic hi-dradenitis suppurativa. Clinical and Experimental Dermatology, 30 (5), 481-483. doi: 10.1111/j.1365-2230.2005.01875.x
13. Weber, L. A., Kantor, G. R., Bergfeld, W. F. (1990). Reticulate pigmented anomaly of the flexures (Dowling-Degos disease): a case report associated with hidradenitis suppurativa and squamous cell carcinoma. Cutis, 45, 446-450.
14. Li, M., Hunt, M. J., Commens, C. A. (1997). Hi-dradenitis suppurativa, Dowling Degos disease and perianal squamous cell carcinoma. Australasian Journal of Dermatology, 38 (4), 209-211. doi: 10.1111/j.1440-0960.1997.tb01700.x
15. Talmant, J.-C., Bruant-Rodier, C., Nunziata, A. C., Rodier, J. F., Wilk, A. (2006). Dégénérescence de maladie de Verneuil en carcinome épidermoïde: à propos de 2 cas et revue de la littérature. Annales de Chirurgie Plastique Esthétique, 51 (1), 82-86. doi: 10.1016/j.anplas.2005.11.002
16. Maclean, G. M., Coleman, D. J. (2007). Three Fatal Cases of Squamous Cell Carcinoma Arising in Chronic Perineal Hidradenitis Suppurativa. The Annals of The Royal College of Surgeons of England, 89 (7), 709-712. doi: 10.1308/003588407x209392
17. Kurokawa, I., Nishimura, K., Yamanaka, K., Mizuta-ni, H., Tsubura, A., Revuz, J. (2007). Cytokeratin expression in squamous cell carcinoma arising from hidradenitis suppurativa (acne inversa). Journal of Cutaneous Pathology, 34 (9), 675-678. doi: 10.1111/j.1600-0560.2006.00680.x
18. Barresi, V., Vitarelli, E., Barresi, G. (2008). Acne inversa complicated by squamous cell carcinoma in association with diffuse malignant peritoneal mesothelioma arising in the absence of predisposing factors: a case report. Journal of Cutaneous Pathology, 35 (1), 70-73. doi: 10.1111/j.1600-0560.2007.00766.x
19. Constantinou, C., Widom, K., Desantis, J., Obmann, M. (2008). Hidradenitis suppurativa complicated by squamous cell carcinoma. Am. Surg., 74, 1177-1181.
20. Katz, R. D., Goldberg, N. H. (2009). Marjolin Ulcer Arising Within Hidradenitis. Annals of Plastic Surgery, 62 (2), 173-174. doi: 10.1097/sap.0b013e31817d87b3
21. Giamarellos-Bourboulis, E. J., Antonopoulou, A., Petropoulou, C., Mouktaroudi, M., Spyridaki, E., Baziaka, F. et. al (2007). Altered innate and adaptive immune responses in patients with hidradenitis suppurativa. British Journal of Dermatology, 156 (1), 51-56. doi: 10.1111/j.1365-2133.2006.07556.x
22. Jemec, G. B. E., Wendelboe, P. (1998). Topical clindamycin versus systemic tetracycline in the treatment of hidradenitis suppurativa. Journal of the American Academy of Dermatology, 39 (6), 971-974. doi: 10.1016/s0190-9622(98)70272-5
23. Slade, D. E. M., Powell, B.W., Mortimer, P. S. (2003). Hidradenitis suppurativa: pathogenesis and management. British Journal of Plastic Surgery, 56 (5), 451-461. doi: 10.1016/s0007-1226(03)00177-2
24. Lebwohl, B., Sapadin, A. N. (2003). Infliximab for the treatment of hidradenitis suppurativa. Journal of the American Academy of Dermatology, 49 (5), 275-276. doi: 10.1016/ s0190-9622(03)01132-0
25. Sullivan, T. P., Welsh, E., Kerdel, F. A., Burdick, A. E., Kirsner, R. S. (2003). Infliximab for hidradenitis suppura-tiva. British Journal of Dermatology, 149 (5), 1046-1049. doi: 10.1111/j.1365-2133.2003.05663.x
26. Adams, R., Gordon, K. B., Devenyi, A. G., Ioffre-da, M. D. (2003). Severe hidradenitis suppurativa treated with infliximab infusion. Archives of Dermatology, 139 (12), 15401542. doi: 10.1001/archderm.139.12.1540
27. Mekkes, J. R., Hommes, D. W. (2004). Treatment of suppurative hidradenitis with surgical deroofing and infliximab. Ned. Tijdschr. Dermatol. Venereol., 14, 196-197.
28. Thielen, A.-M., Barde, C., Saurat, J.-H. (2006). Long-term infliximab for severe hidradenitis suppurativa. British Journal of Dermatology, 155 (5), 1105-1107. doi: 10.1111/j.1365-2133.2006.07528.x
29. Cusack, C., Buckley, C. (2006). Etanercept: effective in the management of hidradenitis suppurativa. British Journal of Dermatology, 154 (4), 726-729. doi: 10.1111/j.1365-2133.2005.07067.x
30. Campione, E., Mazzotta, A., Bianchi, L., Chimenti, S. (2006). Severe Acne Successfully Treated With Etanercept. Acta Dermato-Venereologica, 86 (3), 256-257. doi: 10.2340/ 00015555-004
31. Moul, D. K., Korman, N. J. (2006). Severe hidrade-nitis suppurativa treated with adalimumab. Archives of Dermatology, 142 (9), 1110-1112. doi: 10.1001/archderm.142.9.1110
32. Zouboulis, C. C.; Wolff, K., Goldsmith, L. A., Katz, S. I., Gilchrest, B. A., Paller, A. S., Leffell, D. J. (Eds.) (2009). Update on hidradenitis suppurativa/acne inversa. Fitzpatrick's Dermatology in General Medicine. New York Chicago.
Дата надходженнярукопису 18.09.2015
Iqbal Bukhari, Doctor of Medical Sciences, Dermatology Department, College of Medicine, University of Dam-mam and King Fahd Hospital of the University, King Fahd Hospital of the University, 40189 str., Alkhobar, Saudi Arabia, 31952
Abdulaziz Al Zahrani, Doctor of Medical Sciences, Internal Medicine Department, King Abdulaziz Hospital, Al Ahsa, 2477 str., Al Ahsa, Kingdom of Saudi Arabia, 31982
УДК: 616.12-073.432.19:616.12-008.46-036.1]-055.2-056.253/.257 DOI: 10.15587/2313-8416.2015.51545
ЕХОКАРДЮГРАФ1ЧШ ЗМ1НИ У Ж1НОК З ХРОН1ЧНОЮ СЕРЦЕВОЮ НЕДОСТАТН1СТЮ ЗАЛЕЖНО В1Д 1НДЕКСУ МАСИ Т1ЛА
© П. П. Бщзшя
До^джено ехокардiографiчнi змти у жток з хротчною серцевою недостаттстю залежно eid тдексу маси тта. Встановлено, що 3i збтьшенням тдексу маси тта вiдбуваeться наростання ступеня дилатацИ аорти, лiвого шлуночка та правого передсердя на фот поглиблення концентричной гтертрофп мюкар-да, кальцинозу клапанiв серця та дiастолiчноi дисфункцп I типу. Спостер^аеться переважання чисто'1' систолiчноi дисфункцП серед жток з ХСН та нормальною масою тта, та зменшення ii поширеностi зi збтьшенням 1МТ
Ключовi слова: ехокардiографiчнi змiни, жтки, хронiчна серцева недостаттсть, тдекс маси тша
Chronic heart failure (CHF) is a widespread disease of cardiovascular system its prevalence is caused with improvement of diagnostic methods simultaneously with an aging of general population. Women are the half of all patients with CHF most of them has preserved fraction of output from the left ventricle with associated diastolic dysfunction. The aim of research was the study of echocardiography changes in women with chronic heart failure depending on body mass index.
Methods: There were examined 237 women with different body mass index (BMI), in 222 was diagnosed CHF of 1-111 functional class (FC). All patients underwent calculation of BMI using Ketle formula and Doppler-echocardiographic examination. The calculations of results were carried out using the methods ofparametric and nonparametric statistics depending on size and distribution of samples.
Results: In women with CHF the growth of dilatation of aorta, right atrium and left ventricle take place at an increase of BMI. The maximal size of the left atrium and right ventricle were registered in the group of examined person with normal body weight. The hypertrophy of left ventricle myocardium in women with CHF progresses proportionally to an increase of BMI. In patients with CHF on the background of obesity the deeper processes of valvular calcination take place. There is a predominance of pure systolic dysfunction in women with CHF and normal body weight, and reduction in its prevalence with BMI increasing. In women with CHF with an increase of BMI takes place the deepening of diastolic dysfunction (by the type of relaxation disorder) and an increase of its prevalence. The remodeling of myocardium in women with CHF in most cases was presented by concentric hypertrophy of the left ventricle and its frequency is directly proportional to an increase of BMI.