DOCSLIB.ORG

Three Cases of Androgen-Dependent Disease Associated with Myotonic

Total Page：16

File Type：pdf, Size：1020Kb

Download full-text PDF Read full-text
Three Cases of Androgen-Dependent Disease Associated with Myotonic JEADV (2003) 17, 56–58 CASE REPORT ThreeBlackwell Science, Ltd cases of androgen-dependent disease associated with myotonic dystrophy SM Cooper,†* RPR Dawber,† D Hilton-Jones‡ †Department of Dermatology, Oxford Radcliffe Hospitals, Oxford, OX3 7LJ, UK, ‡Muscle and Nerve Centre, Radcliffe Infirmary, Oxford, OX2 6HE, UK. *Corresponding author, Department of Dermatology, Churchill Hospital, Headington, Oxford, OX3 7LJ, UK, tel. +01865 228232; fax +1865 228240 ABSTRACT Three cases of androgen-dependent disease in females with myotonic dystrophy are described. Serum androgens in individuals affected by myotonic dystrophy are known to be lower on average than in normal controls. Despite this these three females developed diseases that are androgen dependent, including acne, hidradenitis suppurativa, androgenetic alopecia and keratosis pilaris. These cases support the hypothesis that the peripheral response to androgens rather than absolute circulating levels of androgens is important in androgen-dependent conditions. Key words: androgenetic alopecia, androgens, hidradenitis suppurativa, keratosis pilaris, myotonic dystrophy Received: 29 December 2000, accepted 5 February 2002 Myotonic dystrophy (Steinert’s disease) is the commonest form of muscular dystrophy in adults, with an estimated incidence of Case reports 1 in 8000. It is inherited as an autosomal dominant trait and Case 1 caused by an unstable expansion of a trinucleotide (CTG) repeat in the untranslated region of the dystrophia myotonica A 43-year-old woman with myotonic dystrophy developed protein kinase gene on chromosome 19q13.3.1 Age of onset and slowly progressive thinning of hair over the vertex, in an severity correlate with the size of the expansion.2 The expansion androgenetic distribution, in her third decade. The hair was is unstable and tends to increase in size on transmission, giving diffusely thin (Ludwig pattern type II) with retention of the rise to the phenomenon of anticipation, in which disease frontal hairline (fig. 2). The scalp was normal with no evidence severity increases in successive generations. of scarring and the hair shafts were normal. She had no other Classical onset myotonic dystrophy is a multisystem disease.3 virilizing features and was otherwise well with no previous In addition to skeletal muscle features (facial and anterior neck endocrine problems. She was diagnosed with myotonic muscle wasting and weakness, ptosis, early distal limb weakness dystrophy at the age of 34, the diagnosis only being suspected and myotonia) endocrine features include premature balding, when she presented to an ophthalmologist with a presenile testicular atrophy and insulin resistance. Systemic features cataract. The diagnosis of myotonic dystrophy was confirmed by include presenile cataracts, cardiac conduction defects, exces- the presence of an expansion of approximately 633 repeats. She sive daytime sleepiness and reduced IQ. The combination of is mildly affected by myotonic dystrophy. Endocrine investigations, facial muscle involvement and premature balding gives rise to including sex hormone binding globulin (SHBG), follicle- the characteristic facial appearance (fig. 1). stimulating hormone (FSH), luteinizing hormone (LH), We report three cases of females affected by myotonic dystro- prolactin, ferritin and thyroid function were normal and serum phy in whom androgen-dependent diseases were diagnosed. testosterone was at the lower end of the normal range. Circulating androgens are decreased in myotonic dystrophy compared with normal controls.4 These cases provide import- Case 2 ant clinical evidence for abnormalities of end-organ sensitivity to androgens in myotonic dystrophy and also provide insight A 37-year-old woman with myotonic dystrophy had a 20-year into the mechanism of other androgen-dependent diseases. history of recurrent, suppurating boils in the groins and 56 © 2003 European Academy of Dermatology and Venereology Androgen-dependent disease associated with myotonic dystrophy 57 fig. 2 Vertical thinning with preservation of the frontal margin. fig. 1 Typical myotonic facies. Note the frontal recession. thighs. Horny follicular papules with variable perifollicular erythema were observed consistent with keratosis pilaris. axillae. Examination findings included grouped comedones, Her myotonic dystrophy was severe and she had learning scarring and boils consistent with a diagnosis of hidradenitis difficulties. suppurativa. She had been treated with long courses of antibiotics, topical antiseptics and Dianette® (cyproterone acetate 2 mg, ethinyloestradiol 35 µg) with limited benefit only. Discussion Frontal balding was observed, but hair was normal over the We present three women with known androgen-dependent vertex. She had extensive horny follicular papules on the upper conditions in the setting of a disease associated with low arms, typical of keratosis pilaris, but there were no other circulating androgens. Carter and Steinbeck found that serum virilizing features. She had severe myotonia, marked facial DHEA and dehydroepiandosterone sulphate (DHEAS), the wasting and weakness, and limited mobility. Genetic studies most abundant circulating androgens, were significantly lower confirmed she carried an expansion of approximately 1400 than controls in 19 affected male and female patients.5 repeats. Endocrine investigations, including SHBG, FSH, LH, Johansson et al.6 confirmed significantly lower DHEA, DHEAS prolactin, androstenedione, ferritin and thyroid function were and 17α-hydroxyprogesterone in a further 15 males. normal but serum dehydroepiandosterone (DHEA) and serum Our three cases all demonstrate androgen-dependent testosterone were below the normal range. diseases: male-pattern alopecia, hidradenitis suppurativa, keratosis pilaris and acne. The evidence for androgen dependence of acne and male pattern baldness comes from the response to Case 3 treatment with antiandrogens and the development of acne A 37-year-old female, severely affected by congenital myotonic after puberty. In addition, androgenetic alopecia in males does dystrophy, was noted to have papules, pustules and comedones not occur in eunuchs and hirsutism does not occur in males or over the trunk and face typical of acne vulgaris. She had females with absent androgens. Keratosis pilaris was first extensive rough areas on the upper outer arms and upper proposed as an androgen-dependent disorder by Barth et al.7 © 2003 European Academy of Dermatology and Venereology JEADV (2003) 17, 56–58 58 Cooper et al. who found an increased incidence and severity of keratosis pilaris disruption of intranuclear pathways.1 It is possible that intra- in hyperandrogenized, obese subjects. They proposed the under- nuclear pathways for androgen binding may be affected. lying cause to be hyperkeratinization of the pilosebaceous unit of terminal hairs in response to androgens, akin to the response of the infundibulum of the pilosebaceous unit in acne. Sawers References et al.8 demonstrated that an antiandrogen, cyproterone acetate, 1 Brook JD, McCurrach ME, Harley HG et al. Molecular basis of effectively inhibited hidradenitis. Finasteride, a selective inhibitor myotonic dystrophy: expansion of a trinucleotide (CTG) repeat at of the 5-α reductase type II isoenzyme, has been effective in treating the 3′ end of a transcript encoding a protein kinase family member. two females with severe, long-standing hidradenitis.9 Cell 1992; 68: 799–808. The presence of androgen-dependent disease in subjects with 2 Harley HG, Brook JD, Rundle SA et al. Expansion of an unstable myotonic dystrophy in whom androgens tend to be lower than DNA region and phenotypic variation in myotonic dystrophy. normal is evidence that the peripheral response to androgens is Nature 1992; 355: 545–546. more important than absolute circulating levels. In many of the 3 Harper PS. Myotonic dystrophy, 2nd edn. Saunders, London, 1989. androgen-dependent diseases circulating androgens are often 4 Harper PS. Endocrine abnormalities in myotonic dystrophy. In: normal or only modestly elevated. Barth et al.10 found no evidence Myotonic dystrophy, 2nd edn. Saunders, London, 1989: 121–148. for biochemical hyperandrogenism in 66 women with hidradenitis 5 Carter JN, Steinbeck KS. Reduced adrenal androgens in patients suppurativa when compared with age- and weight-matched with myotonic dystrophy. J Clin Endocrinol Metab 1985; 60: controls. If absolute levels of androgens were important, one 611–614. would expect a much higher incidence of acne and hidradenitis 6 Johansson A, Henriksson A, Olofsson BO et al. Adrenal steroid in males in whom circulating levels are higher. In fact, in dysregulation in dystrophia myotonica. J Intern Med 1999; 245: hidradenitis, there is marked female excess. 345–351. An alternative explanation is that androgens are irrelevant in 7 Barth JH, Wojnarowska F, Dawber RPR. Is keratosis pilaris another the so-called androgen-mediated diseases. The response to androgen-dependent dermatosis? Clin Exper Derm 1988; 13: treatment of these conditions with antiandrogens suggests 240–241. otherwise. It is therefore likely that there is a difference in the 8 Sawers RS, Randall VA, Ebling FJG. Control of hidradenitis peripheral response to androgens between individuals, mediated suppurativa. Br J Dermatol 1986; 141: 1138–1139. by peripheral androgen receptors, and that absolute levels of 9 Farrell AM, Randall VM, Vafaee T et al. Finasteride as a therapy for circulating androgens are of limited importance. We speculate hidradenitis suppurativa. Br J Dermatol 1999; 141: 1138–1139. that there is a functional difference in receptors accounting for 10 Barth JH, Layton AM, Cunliffe WJ. Endocrine factors in pre- and the frontal balding in myotonic dystrophy. The molecular basis postmenopausal women with hidradenitis suppurativa. Br J of myotonic dystrophy remains uncertain, but may involve Dermatol 1996; 134: 1057–1059. Visit the EADV website at: www.eadv.org © 2003 European Academy of Dermatology and Venereology JEADV (2003) 17, 56–58.
Load more

Recommended publications
  • Endocrinology 12 Michel Faure, Evelyne Drapier-Faure
    Chapter 12 Endocrinology 12 Michel Faure, Evelyne Drapier-Faure Key points 12.1 Introduction Q HS does not generally appear to be In 1986 Mortimer et al. [14] reported that hi- associated with signs of hyperan- dradenitis suppurativa (HS) responded to treat- drogenism ment with the potent antiandrogen cyproterone acetate. They suggested that the disease could Q Sex hormones may affect the course of be androgen-dependent [8]. This hypothesis HS indirectly through, for example, was also upheld by occasional reports of women their effects on inflammation with HS under antiandrogen therapy [18]. Actu- ally, the androgen dependence of HS (similarly Q The role of end-organ sensitivity to acne) is only poorly substantiated. cannot be excluded at the time of writing 12.2 Hyperandrogenism and the Skin Q The prevalence of polycystic ovary syndrome in HS has not been system- Androgen-dependent disorders encompass a atically investigated broad spectrum of overlapping entities that may be related in women to the clinical consequenc- es of the effects of androgens on target tissues and of associated endocrine and metabolic dys- functions, when present. #ONTENTS 12.1 Introduction ...........................95 12.2.1 Androgenization 12.2 Hyperandrogenism and the Skin .........95 12.2.1 Androgenization .......................95 One of the less sex-specific effects of androgens 12.2.2 Androgen Metabolism ..................96 12.2.3 Causes of Hyperandrogenism ...........96 is that on the skin and its appendages, and in particular their action on the pilosebaceous 12.3 Lack of Association between HS unit. Hirsutism is the major symptom of hyper- and Endocrinopathies ..................97 androgenism in women.
    [Show full text]
  • Back to Basics: Understanding Hidradenitis Suppurativa
    PRACTICE DEVELOPMENT Back to basics: understanding hidradenitis suppurativa KEY WORDS Hidradenitis Suppurativa (HS) is a chronic recurrent debilitating skin disease of the Dermatology hair follicle. It is a condition that has been overlooked in wound care publications, Fistulae with most articles found in dermatological journals. However, the condition affects Hidradenitis Suppurativa 1% of the population in Europe and produces painful nodules in one or more of Scarring Sinus tracts the apocrine-gland bearing aspects of the skin that can ulcerate and produce pain and a foul odour and can multiply and eventually develop sinus tracts and fistulae. HS is often misdiagnosed as alternative skin ulcerating conditions, leaving the individuals with many years of suffering from the physical symptoms and their psychological consequences. The disease often begins in puberty and burns out by middle age, leaving the individual with unsightly scarring. This article examines the pathophysiology, clinical presentations and comorbidities associated with the disease. The treatment options focus on controlling the comorbidities, moderating life-style behaviours and arresting the disease. The medical and surgical options are discussed along with their limitations. idrarenitis Suppurativa (HS) was first selection bias, however is thought to be 1% in described by the French surgeon Velpeau Europe (Gulliver et al, 2016; WUWHS, 2016). in 1839. The origin of the term HS comes Prevalence is rare in children and when HS does Hfrom the Greek hidros meaning sweat and aden occur in this population it is often associated with denoting glands (Ather et al, 2006). Initially it was hormonal disorders such as metabolic syndrome, thought to be due to infection of the sweat glands precocious puberty, adrenal hyperplasia and however it is now recognized as an acneform premature adrenarche (Vivar and Kruse, 2017).
    [Show full text]
  • Metformin for the Treatment of Hidradenitis Suppurativa: a Little Help Along the Way
    DOI: 10.1111/j.1468-3083.2012.04668.x JEADV ORIGINAL ARTICLE Metformin for the treatment of hidradenitis suppurativa: a little help along the way R. Verdolini,† N. Clayton,‡,* A. Smith,‡ N. Alwash,† B. Mannello§ †Department of Dermatology, Princess Alexandra Hospital NHS trust, Harlow, Essex, and ‡Department of Dermatology, The Royal London Hospital, London, UK §Mannello Statistics, Via Rodi, Ancona, Italy *Correspondence: N. Clayton. E-mail: [email protected]; [email protected] Abstract Background Despite recent insights into its aetiology, hidradenitis suppurativa (HS) remains an intractable and debilitating condition for its sufferers, affecting an estimated 2% of the population. It is characterized by chronic, relapsing abscesses, with accompanying fistula formation within the apocrine glandbearing skin, such as the axillae, ano-genital areas and breasts. Standard treatments remain ineffectual and the disease often runs a chronic relapsing course associated with significant psychosocial trauma for its sufferers. Objective To evaluate the clinical efficacy of Metformin in treating cases of HS which have not responded to standard therapies. Methods Twenty-five patients were treated with Metformin over a period of 24 weeks. Clinical severity of the disease was assessed at time 0, then after 12 weeks and finally after 24 weeks. Results were evaluated using Sartorius and DLQI scores. Results Eighteen patients clinically improved with a significant average reduction in their Sartorius score of 12.7 and number of monthly work days lost reduced from 1.5 to 0.4. Dermatology life quality index (DLQI) also showed a significant improvement in 16 cases, with a drop in DLQI score of 7.6.
    [Show full text]
  • Hidradenitis Suppurativa FOUR CORNERS of CARE
    THE www.thedermdigest.com Vol. 2, No. 3 | March 2021 Hidradenitis Suppurativa FOUR CORNERS OF CARE Topical CBD: Hope or Hype? The Dermatologist as PCP Ask the Experts Permit No. 129 No. Permit Your Most High-Impact Columbus, WI Columbus, PAID Cosmetic Innovations US Postage US Prsrt Mkt Prsrt EDUCATIONAL • INTERACTIVE • AUTHORITATIVE Say Hello again to Not Actual Size Most eligible commercially $ per prescription insured patients pay as little as 20at any pharmacy* * Certain restrictions apply. The LEO Pharma CONNECT program may reduce out-of-pocket expenses. Must be 12 years of age or older to be eligible, and a legal guardian over 18 years of age must redeem the card for patients aged 12 to 17. You are not eligible if you are enrolled or you participate in any state or federally funded health care program (eg, Medicare, Medicaid, etc). Full details of the LEO Pharma CONNECT program are available at www.FinaceaFoam.com/hcp or may be obtained by calling 1-877-678-7494 between 8:30 AM and 8:30 PM (Eastern), Monday through Friday. The LEO Pharma logo, LEO Pharma, and Finacea are registered trademarks of LEO Pharma A/S. www.FinaceaFoam.com ©2020 LEO Pharma Inc. All rights reserved. March 2020 MAT-32481 THE Contents www.thedermdigest.com Volume 2, Number 3 | March 2021 14 Cover Article You can help hidradenitis suppurativa patients achieve long-term control and avoid flares 2 20 Ted Talks Pediatrics Do you talk politics in your office? Combination therapy can produce good clearance of pediatric warts 6 Literature Lessons Research updates in rheumatologic diseases, psoriasis, hair and nails, contact dermatitis, acne, rosacea, and more continued on page 3 March 2021 | 1 Ted Talks “ In politics, stupidity is not a handicap.” —Attributed to Napoleon Bonaparte he year was 1960, in the middle of a highly conten- T tious presidential contest between John F.
    [Show full text]
  • Folliculitis Highlights Author: Indira Gowda (EM Resident Physician, Mount Sinai Hospital) • Definition: O Inflammation Of
    Folliculitis Highlights Author: Indira Gowda (EM Resident Physician, Mount Sinai Hospital) Definition: o Inflammation of the hair follicle caused by either chemical/physical irritation or viral/bacterial infection o Folliculitis is the smallest and most minor o Furuncles are larger, carbuncles larger than that, and boils even larger (3) Taken From: http://www.shorthillsderm .com/blog/wp- Common associations: content/uploads/2014/11/ o Diabetes, immunosuppression, shaving What-is-Folliculitis.jpg Diagnosis: o Based on appearance and history o Usually seen on scalp, face, legs, back, chest, axilla o Folliculitis is usually not tender compared to carbuncles and furuncles o If someone has had recent contact w/ hot tub or DM, consider pseudomonas as potential bacteria (3) o May see hyper- or hypopigmentation post infection Tx: o Warm compresses 3 times/day o Antibiotics as treatment of recurrent infections is controversial as increased antibiotic use may lead to increased resistance . Topical mupirocin for moderate folliculitis, more severe infections may require antibiotics . For tx of strep: dicloxacillin or cefadroxil; tx of strep infections helps to prevent PSGN but no effect on rheumatic fever (3) . For tx of pseudomonas: fluoroquinolones or carbapenems or other anti-pseudomonal options . For tx staph (particularly MRSA): 7-10 day course of Bactrim, clindamycin, doxycycline, vancomycin, linezolid,… other antibiotics reserved for more severe infections. (5) o Many people carry MRSA in their nares. Between 10-35% are persistent carriers and 20-75% intermittent carriers. (4) . Should we treat carriers? (Mashhood AA, 2006) recommends nasal Taken from http://riversideonline.com swab testing in all patients with recurrent “furunculosis” aka boils /source/images/image_po aka abscesses.
    [Show full text]
  • Understanding Hidradenitis Suppurativa (Hs)
    UNDERSTANDING HIDRADENITIS SUPPURATIVA (HS) Information to help you work with your doctor so you can better manage your HS WHAT’S INSIDE Hidradenitis suppurativa (HS) in brief . 4 HS – the facts . 5 Living with HS . 9 Coping with HS . 11 Tips and tricks for living with HS . 12 Addressing your thoughts and feelings . 14 Coping with stress . 16 Working with your health care team . 17 Symptom self-assessment questionnaire . 19 Useful online resources . 23 3 HIDRADENITIS SUPPURATIVA (HS) IN BRIEF You have been diagnosed with HS, a disease which is: PAINFUL PRORESSIVE INFLAMMATORY GETTING WORSE CAUSING OVER TIME SWELLING CHRONIC SYSTEMIC LASTING AFFECTING A LONG TIME THE WHOLE BODY HS is known to produce lesions in the skin that are inflamed (swollen), recurrent (reappearing after lesions disappear), and chronic (lasting a long period of time). HS can have both a physical and psychological impact. The resulting pain and affected areas of your skin can restrict movement and your ability to do day-to-day tasks. Because of the areas of the body affected and how unsightly, smelly, and uncomfortable or painful HS may be, it can also lead to negative feelings such as embarrassment, stress and low mood. While we understand this is how some people with HS may feel, it’s important to remember that HS is a medical condition — its development is not your fault. 4 It’s important to be able to manage the physical side of the condition while also trying to reduce any feelings of embarrassment you may feel. There is more information about addressing your thoughts and feelings on page 14.
    [Show full text]
  • RIPE for the PICKING Experts Profile the Future of Biologic Treatments
    RIPE FOR THE PICKING Experts profile the future of biologic treatments 22 DERMATOLOGY WORLD // September 2015 www.aad.org/dw BY VICTORIA HOUGHTON, ASSISTANT MANAGING EDITOR John Harris, MD, PhD, assistant professor of medicine at the University of Massachusetts in the division of dermatology — like many dermatologists — has watched the impressive evolution of treatments for psoriasis over the last decade with anticipation. “We initially had very broad immunosuppressants that were somewhat effective in some patients, but they also had significant side effects,” Dr. Harris said. However, “The onset of biologics and other targeted therapies has been incredible. They’ve revolutionized treatment for psoriasis.” However, while physicians are enthusiastic about the progress of these treatments for psoriasis, there is also hope that interest in developing these innovative therapies is increasingly shifting to other skin conditions. “Pharmaceutical companies have to start looking elsewhere, given how good current psoriasis therapies are,” Dr. Harris said. “The real room for growth is in other diseases.” As psoriasis has paved the way for an interest in developing biologic and other targeted treatments in skin conditions, physicians are anticipating a promising future for these treatments in the following conditions: Atopic dermatitis Hidradenitis suppurativa Chronic urticaria Vitiligo Dermatomyositis >> Alopecia areata DERMATOLOGY WORLD // September 2015 23 RIPE FOR THE PICKING Atopic dermatitis 133; 6:1626-34). The study showed that by blocking the According to Lawrence Eichenfield, MD, professor of immune pathways with CsA, the molecular abnormalities dermatology and pediatrics at the University of California, with AD skin barrier genes, such as filaggrin and loricrin, San Diego and chief of pediatric and adolescent dermatology normalized.
    [Show full text]
  • Common Infections of the Skin Candida of Nails How to Diagnose
    Candida of Nails • Occurs in persons who have hands in water • Green nails represent the co‐pathogen which is pseudomonas Common Infections of the Skin TREATMENT: • Fluconazole 150 mg qd x1 month PLUS Ciprofloxacin 500 bid x 2 weeks Toby Maurer, MD OR University of California, San Francisco Thymol 2‐4% soak 20 mins bid x 3 months and tobramycin or gentamycin ophthalmologic drops How to diagnose Onychomycosis • Not all dystrophic nails= onychomycosis • Topical treatment –use for the right type of • KOH‐difficult to do and operator dependent lesions • CULTURE is gold standard but takes 3 weeks to grow out. • Naftin gel for small superficial lesions • Now PCR‐used in Europe with high sensitivity and • Penlac (Ciclopirox 8%) reported to work 35‐ specificity 52% of the time • Cost effective and results in 24‐72 hours – cost: expensive 1 Right type of lesions for topicals • Lunula not affected • Griseofulvin‐least hepatotoxic but lower • Less than 5 nails affected efficacy‐ 250 mg bid x 12‐18 months • No thickening of nails • Fluconazole‐ 150 mg qweek for more than 6 • No separation of nail plate on sides months –July 2012 Dermat Tx Gupta AK et al • Itraconazole‐ can pulse it‐ 400 mg qd x 7 days q month x 4 months Terbinafine (Lamisil) • Still the leader of the pack‐most effective in BASELINE 1 YR 5 YR terms of INITIAL and LONG‐TERM cure rate. • DOSE: 250 mg qd Continuously x 3 months Terbinafine 77% 75% 50% for fingernails and x4 months for toenails Itraconazole 70% 50% 13% (July 2012) i.e. no pulsing Grispeg 41% Fluconazole ? ? ? 2 Liver toxicity What about laser? • Transaminase elevation 0.4% to 1% with • Photo‐ inactivation laser and destructive terbinafine and intraconazole laser • Transaminase elevation does not predict liver • 4 studies‐2 – no results; 2 show results but failure with recurrence.
    [Show full text]
  • Finasteride for the Treatment of Hidradenitis Suppurativa in Children and Adolescents
    OBSERVATION Finasteride for the Treatment of Hidradenitis Suppurativa in Children and Adolescents Harkamal Kaur Randhawa, MD; Jill Hamilton, MD; Elena Pope, MD Importance: Hidradenitis suppurativa (HS) is a chronic ity of disease flares with no significant adverse effects. debilitating cutaneous disease for which there is no uni- versally effective treatment. Patients typically present at Conclusions and Relevance: Finasteride is a thera- puberty with tender subcutaneous nodules that can prog- peutic option that provides benefit for pediatric patients ress to dermal abscess formation. Antiandrogens have been with HS. Further prospective data and randomized con- used in the treatment of HS, and studies have primarily trolled studies will provide helpful information in the man- focused on adult patients. agement of this disease. Observations: We present a case series of 3 pediatric JAMA Dermatol. 2013;149(6):732-735. patients with HS who were successfully treated with oral Published online March 20, 2013. finasteride, resulting in decreased frequency and sever- doi:10.1001/jamadermatol.2013.2874 IDRADENITIS SUPPURA- from that of cyproterone acetate, a previ- tiva (HS) is a chronic in- ously studied antiandrogen in patients flammatory disease that with HS. We outline findings in 3 cases primarily involves skin in presenting during childhood, all in pa- the axillae, groin, and tients who showed significant improve- Hanogenital regions, although disease may ment in disease activity with finasteride extend to the buttocks, chest, scalp, eye- treatment. lids, and retroauricular areas.1 Patients with HS develop exquisitely painful erythem- REPORT OF CASES atous subcutaneous nodules that may heal spontaneously or suppurate and coalesce to form dermal abscesses.
    [Show full text]
  • Daniel Zelac, MD Scripps Clinic  Conflicts of Interest – None
    Daniel Zelac, MD Scripps Clinic Conflicts of Interest – None Many of the photographs and diagrams contained in this talk can be referenced in Clinical Dermatology, 5th Edition By Thomas P. Habif, MD Please do not reproduce or distribute further (referenced in talk as “Habif 5th”) Atopic Dermatitis Perleche Allergic Dermatitis Lip Licker’s Dermatitis Herpes Impetigo Aphthous Ulcerative Disease Actinic Chelitis Squamous Cell Carcinoma Skinsight.com AKA ( cheilosis, angular stomatitis) noted for fissures and inflammation of the labial commisures. Primary causes – chemical irritant, nutritional deficiencies(B2, Fe, Zinc) Can be associated with Plummer-Vinson syndrome (esophogeal web, iron deficiency anemia, glossitis, cheilitis) TreatmentSecondary –causes – Overlying infection, Candida or Bacterial correction of underlying medical or physical problem Treatment of any secondary bacterial or fungal infection Allergic Contact Dermatitis – Scaling eczematous inflammatory reaction affecting areas in direct contact or neighboring regions to agents typically used in or around the oral cavity EastMaui.com Mango- has within it’s tree sap a compound called ‘Urushiol’ Contact with the sap externally can Principles of Pediatric Dermatology cause the same reaction in people as poison ivy. Toothpastes and Mouthwashes • Flavorings Mints( Spearmint, Peppermint, Menthol, Carvone) Cinnamal (cinnamon) Anethole (star anise) • Antiseptic Propolis • Plaque control Hexylresorcinol • Anti-Inflammatory Azulene • Florides • Preservatives Parabens Dental Floss • Colophony Rosin- from pine and spruce trees (colophonium, colophony, resinterethinae, tall oil, abietic acid, methyl abietate alcohol, abietic alcohol, abietyl alcohol) Dermnet nz.com Lip Licker’s Dermatitis- Herpes Simplex- Candidiasis Inverse Psoriasis Hailey-Hailey Disease Erythrasma (bacterial infection (C. minutissimum)) Tinea Corporis Hidradenitis Suppurativa Tinea Corporis Hidradenitis Suppurativa • Cause - bacterial infection (C.
    [Show full text]
  • Hormonal Therapies for Hidradenitis Suppurativa: Review
    Volume 23 Number 10 | October 2017 Dermatology Online Journal || Review DOJ 23 (10): 1 Hormonal therapies for hidradenitis suppurativa: Review Ashley K Clark1 BS, Rebecca L Quinonez2 BS, Suzana Saric1 BA, Raja K Sivamani3,4 MD MS CAT Affiliations:1 School of Medicine, University of California, Davis, Sacramento, California, 2School of Medicine, Universidad Autonoma de Guadalajara - San Antonio, Texas, 3Department of Dermatology, University of California, Davis, Sacramento, California, 4Department of Biological Sciences, California State University-Sacramento, Sacramento, California Corresponding Author: Raja Sivamani MD, MS, CAT, Department of Dermatology, University of California, Davis, 3301 C Street, Suite 1400, Sacramento, CA 95816, Tel: 916-703-5145, Fax: 916-734-7183, Email: [email protected] Abstract of the surrounding tissue [2]. Abscess formation is commonly seen and it can extend deep into the Hidradenitis suppurativa is a recurrent inflammatory subcutaneous tissue, further promoting deep sinus skin condition characterized by abscesses and boils, tract formation. Hypertrophic scarring is a common predominantly in the groin, armpit, and buttocks sequela of the condition, primarily affecting those areas. HS is not a life-threatening condition, but with darker skin tones. severely impairs quality of life in those affected. Finding a successful treatment approach for HS has Clinically HS presents as painful papules and nodules, been challenging, in part because of the lack of a gold- eventually progressing to abscess formation and standard treatment method, limited research-based severe scarring. It may also be characterized by information, and the nature of clinical variation in the having a malodorous sterile discharge. The severity of disease. Treatment commonly consists of antibiotics, the disease is described by the Hurley stages.
    [Show full text]
  • Hidradenitis Suppurativa with Focus on Rare Facial Involvement
    ACNE, ROSACEA, AND RELATED DISORDERS (INCLUDING HIDRADENITIS SUPPURATIVA) HIDRADENITIS SUPPURATIVA WITH FOCUS ON RARE FACIAL INVOLVEMENT I Kovacevic (1) - I Sjerobabski Masnec (2) Polyclinic Poliderma, Dermatology, Zagreb, Croatia (1) - University Clinical Hospital Center "sestre Milosrdnice", Dermatology, Zagreb, Croatia (2) Background: Hidradenitis suppurativa (HS) is a chronic inflammatory skin disease that appears as a deep painful dermal nodules, fistulas, sinus tract formations and hypertrophic scars in the apocrine gland-bearing areas. The primary sites of involvation are the intertriginous skin areas of the groin, axillary, perineal, perianal and inframammary regions. However, any area of skin that contains hair follicles and apocrine glands may be involved. There are only 21 reports in the literature about HS of face and other ectopic sites. Observationa: We would like to distinguish and describe face involvement in hydradenitis suppurativa with case report of a 33 year old male patient , smoker with a normal body mass index, who first developed facial unilateral HS in his early 20s. During the past 10 years, the disease progressed gradually, and now involve axillae, ingvinum and perianal region. The stage of illness is now Hurley II. In this patient there has been no history of facial acne vulgaris, but two years before the first HS lesion had dissecting cellulitis of the scalp. During the last 10 years, his dermatologist and surgeon had clinically diagnosed facial lesions as acne conglobata and menaged them with systemic antibiotics, isotretinoin and several surgical incisions and drainages. Key message : HS patients suffer from typical hidradenitis lesions, which are not present in the case of acne . It is important to distinguish facial HS from acne conglobata and with legitimate therapy prevent new boils and lesions from forming as well as severe scarring of the face.
    [Show full text]