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Clinical Presentation of Pili Torti - Case Report*
CASE REPORT 29 ▲ Clinical presentation of pili torti - Case report* Jeane Jeong Hoon Yang1 Karine Valentim Cade1 Flavia Cury Rezende1 José Marcos Pereira (In memoriam)1 José Roberto Pereira Pegas1 DOI: http://dx.doi.org/10.1590/abd1806-4841.20153540 Abstract: Pili torti also known as ‘twisted hairs’ (Latin: pili=hair; torti=twisted) is a rare, congenital or acquired clinical presentation, in which the hair shaft is fl attened at irregular intervals and twisted 180º along its axis. It is clinically characterized by fragile, brittle, coarse and lusterless hairs, due to uneven light refl ection on the twisted hair surface. Pili torti may be associated with neurological abnormalities and ectodermal dysplasias. There is no specifi c treatment for this condition, but it may improve spontaneously after puberty. We report a case of pili torti in a child who presented fragile, brittle, diffi cult to comb hair. The patient had no comorbidities. Keywords: Capillary fragility; Hair diseases; Rotation INTRODUCTION Pili torti is a congenital or acquired, dominant tion, audiometry and measurement of serum zinc were autosomal disease in which the hair shaft is fl attened performed. All results were within the normal range. at irregular intervals and twisted along its axis. It most The diagnostic hypothesis of pili torti was made. The commonly occurs in fair-haired girls, beginning in the patient remains in outpatient follow-up and shows second or third year of life. The hairs become dry, lus- age-appropriate neuropsychomotor development. terless, fragile and brittle. The condition may be asso- ciated with neurosensorial deafness1 and is probably caused by changes in the internal hair sheath. -
Acne Keloidalis Nuchae
Dermatologic Therapy, Vol. 20, 2007, 128–132 Copyright © Blackwell Publishing, Inc., 2007 Printed in the United States · All rights reserved DERMATOLOGIC THERAPY ISSN 1396-0296 Blackwell Publishing Inc Acne keloidalis nuchae Acne keloidalis nuchae, also known as folliculitis its antimicrobial and antiinflammatory effect), nuchae, is a chronic scarring folliculitis charac- and a series of intralesional steroids (40 mg/cc of terized by fibrotic papules and nodules of the existing keloids). Education is the key to preven- nape of the neck and the occiput. It particularly tion. I discourage high-collared shirts, short hair- affects young men of African descent and rarely cuts, and close shaving or cutting the hair along occurs in women; in either case its occurrence the posterior hairline. In the long-term, patients has a significant impact on the patient’s quality benefit from laser hair removal using diode or of life. We’ve asked our experts to share their expe- Nd:YAG, which helps avoid disease progression. rience in helping patients with this cosmetically Early treatment decreases the morbidity that can disfiguring disorder. be associated with late-stage disease. Question Dr. Vause: I treat early acne keloidalis nuchae by instructing patients to wash the skin frequently Please describe your approach to the treatment of with a mild keratolytic like tar or an alpha hydroxy patients with early (less than 20 papules, pustules acid cleanser. Patients are instructed to apply and 1–2 < 2 cm nuchae keloids) acne keloidalis topical clindamycin with steroid in the morning nuchae. (1–3) and retinoid at bedtime. Dr. Brauner: An option is to treat all patients with Response chlorhexadine cleanser as a daily shampoo and minocycline 100 mg daily b.i.d. -
Integrative Approach to a Difficult Trichology Patient Natalie Barunova* International Scientific-Practical Centre “Trichology”, Moscow, Russia
Global Dermatology Clinical Case ISSN: 2056-7863 Integrative approach to a difficult trichology patient Natalie Barunova* International Scientific-Practical Centre “Trichology”, Moscow, Russia Abstract Folliculitis decalvans belongs to a group of primary cicatricial alopecias with neutrophilic inflammation of the scalp. It is characterized by recurrent purulent follicular exudation with inevitable destruction of pilosebaceous unit as an outcome of the disease. Staphylococcus aureus is supposed to play an important role in the pathogenesis of the disease. The treatment is usually focused on the eradication of S. aureus. A clinical case of effective adjuvant treatment of folliculitis decalvans patient is presented in this manuscript. The previous traditional treatment with antibiotics, topical glucocorticosteroids, oral prednisone and retinoid treatment had minor efficacy and subsequent recurrence. Integrative approach to this difficult case brought the patient into remission and improve the patient’s condition. Introduction alopecias, such as dissecting cellulites, lichen planopilaris, discoid lupus erythematosus, central centrifugal cicatricial alopecia and acne Scarring alopecias relate to a group of relatively rare diseases with keloidalis nuchae [2,7-9]. one common feature - inevitable destruction of pilosebaceous unit due to replacement of hair follicles by fibrous tissue. Differential diagnosis is performed with the following conditions [2,10]: FD is classified as a primary neutrophilic scarring alopecia according to the classification from the 2001 Workshop on cicatricial • Dissecting folliculitis – occurs almost exclusively in males. alopecias at Duke University Medical Center [1]. Clinical features include boggy scalp, deep inflammatory nodes, interconnected sinus tracts with purulent material; It is characterized by recurrent purulent follicular exudation with patches of scarring (cicatricial) alopecia as an outcome of the condition • Acne keloidalis nuchae – also occurs mainly in males and low efficacy of the treatment. -
An Open Label Study of Clobetasol Propionate 0.05% and Betamethasone Valerate 0.12% Foams in the Treatment of Mild to Moderate Acne Keloidalis
HIGHLIGHTING SKIN OF COLOR An Open Label Study of Clobetasol Propionate 0.05% and Betamethasone Valerate 0.12% Foams in the Treatment of Mild to Moderate Acne Keloidalis Valerie D. Callender, MD; Cherie M. Young, MD; Christina L. Haverstock, MD; Christie L. Carroll, MD; Steven R. Feldman, MD, PhD Acne keloidalis (AK) is a disease affecting pri- 1942.2,3 It is predominantly a condition of African marily African American men. Topical steroids American men4; however, it also occurs in African are a widely accepted treatment of AK; however, American women5 and other ethnic groups. The no studies have been published investigating true incidence of AK is varied, and studies suggest their effectiveness. The purpose of this open- a range of 0.45% to 13.7% in blacks.6-8 Studies per- label study was to assess the efficacy and toler- formed by Halder et al9 and Kenny10 did not find ability of clobetasol propionate 0.05% and AK to be in the 12 most common diagnoses in betamethasone valerate 0.12% foams in the African Americans. treatment of AK in 20 African American patients. AK begins as papules and pustules on the occip- These patients were treated for 8 to 12 weeks ital scalp and posterior neck that may develop into using a pulsed-dose regimen. We found topical nodules or coalesce into plaques. In some cases, clobetasol propionate foam to be effective in other areas of the scalp may be involved, including improving AK, and our patients found the foam the vertex. Initially, hair shafts can be seen exiting vehicle to be cosmetically acceptable. -
Hair Loss in Infancy
SCIENCE CITATIONINDEXINDEXED MEDICUS INDEX BY (MEDLINE) EXPANDED (ISI) OFFICIAL JOURNAL OF THE SOCIETÀ ITALIANA DI DERMATOLOGIA MEDICA, CHIRURGICA, ESTETICA E DELLE MALATTIE SESSUALMENTE TRASMESSE (SIDeMaST) VOLUME 149 - No. 1 - FEBRUARY 2014 Anno: 2014 Lavoro: 4731-MD Mese: Febraury titolo breve: Hair loss in infancy Volume: 149 primo autore: MORENO-ROMERO No: 1 pagine: 55-78 Rivista: GIORNALE ITALIANO DI DERMATOLOGIA E VENEREOLOGIA Cod Rivista: G ITAL DERMATOL VENEREOL G ITAL DERMATOL VENEREOL 2014;149:55-78 Hair loss in infancy J. A. MORENO-ROMERO 1, R. GRIMALT 2 Hair diseases represent a signifcant portion of cases seen 1Department of Dermatology by pediatric dermatologists although hair has always been Hospital General de Catalunya, Barcelona, Spain a secondary aspect in pediatricians and dermatologists 2Universitat de Barcelona training, on the erroneous basis that there is not much in- Universitat Internacional de Catalunya, Barcelona, Spain formation extractable from it. Dermatologists are in the enviable situation of being able to study many disorders with simple diagnostic techniques. The hair is easily ac- cessible to examination but, paradoxically, this approach is often disregarded by non-dermatologist. This paper has Embryology and normal hair development been written on the purpose of trying to serve in the diag- nostic process of daily practice, and trying to help, for ex- ample, to distinguish between certain acquired and some The full complement of hair follicles is present genetically determined hair diseases. We will focus on all at birth and no new hair follicles develop thereafter. the data that can be obtained from our patients’ hair and Each follicle is capable of producing three different try to help on using the messages given by hair for each types of hair: lanugo, vellus and terminal. -
Keratitis-Ichthyosis-Deafness Syndrome in Association With
Genes and skin Eur J Dermatol 2005; 15 (5): 347-52 Laura MAINTZ1 Regina C. BETZ2 Keratitis-ichthyosis-deafness syndrome Jean-Pierre ALLAM1 in association with follicular occlusion triad Jörg WENZEL1 Axel JAKSCHE3 Nicolaus FRIEDRICHS4 Thomas BIEBER1 Keratitis-Ichthyosis-Deafness syndrome is a rare congenital disorder of Natalija NOVAK1 the ectoderm caused by mutations in the connexin-26 gene (GJB2) on 1 chromosome 13q11-q12, giving rise to keratitis, erythrokeratoderma Department of Dermatology, University of and neurosensory deafness. We report the case of a 31-year-old black Bonn, Sigmund-Freud-Str. 25, 53105 Bonn, Germany male diagnosed as having KID syndrome. Sequencing analysis showed 2 Institute of Human Genetics, University of a heterozygous missense mutation D50N (148G > A) in the GJB2 gene. Bonn, Wilhelmstr. 31, 53115 Bonn, In addition to the classical features of vascularizing keratitis, erythro- Germany 3 Department of Ophthalmology, University keratoderma and congenital deafness, our patient presented a follicular of Bonn, Sigmund-Freud-Str. 25, 53105 occlusion triad with hidradenitis suppurativa (HS, alias acne inversa), Bonn, Germany acne conglobata and dissecting cellulitis of the scalp, leading to cicatri- 4 Institute of Pathology, University of Bonn, Sigmund-Freud-Str. 25, 53105 Bonn, cial alopecia and disfiguring, inflammatory vegetations of his scalp. Germany Conservative therapy such as a keratolytic, rehydrating and antiseptic external therapy, antibiotic, antimycotic and retinoids were only of Reprints: N. Novak moderate benefit, so we finally chose the curative possibility of surgery Fax: (+49) 228 287 4883 <[email protected]> therapy of the axillar papillomas and of the scalp. The inflammatory papillomatous regions of the axillae and of the scalp were radically debrided. -
Pili Torti: a Feature of Numerous Congenital and Acquired Conditions
Journal of Clinical Medicine Review Pili Torti: A Feature of Numerous Congenital and Acquired Conditions Aleksandra Hoffmann 1 , Anna Wa´skiel-Burnat 1,*, Jakub Z˙ ółkiewicz 1 , Leszek Blicharz 1, Adriana Rakowska 1, Mohamad Goldust 2 , Małgorzata Olszewska 1 and Lidia Rudnicka 1 1 Department of Dermatology, Medical University of Warsaw, Koszykowa 82A, 02-008 Warsaw, Poland; [email protected] (A.H.); [email protected] (J.Z.);˙ [email protected] (L.B.); [email protected] (A.R.); [email protected] (M.O.); [email protected] (L.R.) 2 Department of Dermatology, University Medical Center of the Johannes Gutenberg University, 55122 Mainz, Germany; [email protected] * Correspondence: [email protected]; Tel.: +48-22-5021-324; Fax: +48-22-824-2200 Abstract: Pili torti is a rare condition characterized by the presence of the hair shaft, which is flattened at irregular intervals and twisted 180◦ along its long axis. It is a form of hair shaft disorder with increased fragility. The condition is classified into inherited and acquired. Inherited forms may be either isolated or associated with numerous genetic diseases or syndromes (e.g., Menkes disease, Björnstad syndrome, Netherton syndrome, and Bazex-Dupré-Christol syndrome). Moreover, pili torti may be a feature of various ectodermal dysplasias (such as Rapp-Hodgkin syndrome and Ankyloblepharon-ectodermal defects-cleft lip/palate syndrome). Acquired pili torti was described in numerous forms of alopecia (e.g., lichen planopilaris, discoid lupus erythematosus, dissecting Citation: Hoffmann, A.; cellulitis, folliculitis decalvans, alopecia areata) as well as neoplastic and systemic diseases (such Wa´skiel-Burnat,A.; Zółkiewicz,˙ J.; as cutaneous T-cell lymphoma, scalp metastasis of breast cancer, anorexia nervosa, malnutrition, Blicharz, L.; Rakowska, A.; Goldust, M.; Olszewska, M.; Rudnicka, L. -
Comfort with Care: Dermatology for Ethnic Skin
Comfort with Care: Dermatology for Ethnic Skin Nkanyezi Ferguson, MD Dermatology Department University of Iowa Hospital and Clinics — 1 — Objectives • Define ethnic skin/skin of color • Discuss skin conditions affecting ethnic skin • Discuss hair conditions affecting ethnic skin • Discuss cosmetic considerations in ethnic skin — 2 — Defining Skin Color • Ethnic skin or skin of color – Broad range of skin types and complexions that characterize individuals with darker pigmented skin – Includes African, Asian, Latino, Native American, and Middle Eastern decent – Encompasses skin types IV - VI — 3 — COMMON SKIN CONDITIONS IN ETHNIC SKIN Skin Cancer • Skin cancer – Non-melanoma skin cancer and melanoma • 4‐5% of all cancers in Hispanics • 1‐4% of all cancers in Asians, Asian Indians and African‐Americans – Less common in dark‐skin however has greater morbidity and mortality – Risk factors • Ultraviolet (UV) radiation from sunlight • Scarring processes/chronic injury (e.g. burns, non-healing leg ulcers, skin lupus) • Depressed immune system — 5 — Skin Cancer • Non-melanoma skin cancer: – Flat or raised – Shiny, red, pink or brown – Asymptomatic or painful – Bleeding, scabbing – Growing, changing – Can occur anywhere on the body — 6 — Skin Cancer • Melanoma: – Dark brown to black – Flat, raised or ulcerated lesions – Asymptomatic or painful – Feet, palms, fingernails, toenails, and inside of the mouth – Can travel to other parts of the body (metastasize) — 7 — Skin cancer Melanoma: • Asymmetry • Border irregularity • Color variation • Diameter -
Pili Torti: Clinical Findings, Associated Disorders, and New Insights Into Mechanisms of Hair Twisting
CONTINUING MEDICAL EDUCATION Pili Torti: Clinical Findings, Associated Disorders, and New Insights Into Mechanisms of Hair Twisting Paradi Mirmirani, MD; Sara S. Samimi, MD; Eliot Mostow, MD, MPH RELEASERELEASE DATE:DATE: AugustSeptember 2009 2009 TERMINATIONTERMINATION DATE:DATE: AugustSeptember 2010 2010 TheThe estimatedestimated timetime toto completecomplete thisthis activityactivity isis 11 hour.hour. GGOALOAL ToTo understandunderstand primarypili torti tocutaneous better manage nodular patients amyloidosis with the(PCNA) condition to better manage patients with the condition LLEARNINGEARNING OBJOBJECTIECTIVVESES UponUpon completioncompletion ofof thisthis activity,activity, youyou willwill bebe ableable to:to: 1.1. RecognizeDistinguish thepili clinicaltorti from presentation other hair shaftof PCNA. disorders. 2.2. DiscussList conditions the pathophysiology frequently associated of PCNA. with pili torti. 3.3. DistinguishExplain the primarypathophysiologic systemic amyloidosismechanisms from that PCNAcan lead based to pili on torti. clinical and laboratory findings. IINTENDEDNTENDED AAUDIENCEUDIENCE ThisThis CMECME activityactivity isis designeddesigned forfor dermatologistsdermatologists andand generalgeneral practitioners.practitioners. CMECME TestTest andand InstructionsInstructions onon pagepage 107.148. ThisThis articlearticle hashas beenbeen peerpeer reviewedreviewed andand approvedapproved byby CollegeCollege ofof MedicineMedicine isis accreditedaccredited byby thethe ACCMEACCME toto provideprovide MichaelMichael Fisher,Fisher, -
Acne Keloidalis Nuchae in the Armed Forces
MILITARY DERMATOLOGY MILITARY DERMATOLOGY IN PARTNERSHIP WITH THE ASSOCIATION OF MILITARY DERMATOLOGISTS Acne Keloidalis Nuchae in the Armed Forces Catherine Brahe, MD; Kristopher Peters, DO; Nicole Meunier, MD cne keloidalis nuchae (AKN) is a chronic inflam- PRACTICE POINTS matory disorder most commonly involving the • Acne keloidalis nuchae (AKN) is a chronic inflamma- A occipital scalpcopy and posterior neck characterized tory disorder of the occipital scalp and posterior neck by the development of keloidlike papules, pustules, and characterized by keloidlike papules, pustules, and plaques. If left untreated, this condition may progress plaques that develop following mechanical irritation. to scarring alopecia. It primarily affects males of African • Military members are required to maintain short hair- descent, but it also may occur in females and in other cuts and may be disproportionately affected by AKN. ethnic groups. Although the exact underlying patho- • In the military population, early identification and treat- not genesis is unclear, close haircuts and chronic mechani- ment, which includes topical steroids, oral antibiotics, cal irritation to the posterior neck and scalp are known UV light therapy, lasers, and surgical excision, can inciting factors. For this reason, AKN disproportionately prevent further scarring, permanent hair loss, and dis- affects active-duty military servicemembers who are held figurement from AKN. Doto strict grooming standards. The US Military maintains these grooming standards to ensure uniformity, self- discipline, and serviceability in operational settings.1 Acne keloidalis nuchae (AKN) is a chronic inflammatory skin disease Regulations dictate short tapered hair, particularly on the characterized by the development of keloidlike papules, pustules, back of the neck, which can require weekly to biweekly and plaques on the occipital scalp and posterior neck following haircuts to maintain.1-5 mechanical trauma and irritation. -
Keratosis Follicularis Spinulosa Decalvans Associated with Acne Keloidalis Nuchae
Case Report DOI: 10.7241/ourd.20142.36 KERATOSIS FOLLICULARIS SPINULOSA DECALVANS ASSOCIATED WITH ACNE KELOIDALIS NUCHAE Ilteris Oguz Topal1, Ilknur Mansuroglu Sahin2, Betül Berberoglu3, Mehmet Ozer2 1Department of Dermatology, Okmeydani Research and Training Hospital, Istanbul, Turkey 2Department of Pathology, Okmeydani Research and Training Hospital, Istanbul, Turkey 3 Source of Support: Department of Pathology, Batman State Hospital, Istanbul, Turkey Nil Competing Interests: Corresponding author: Dr Ilteris Oguz Topal [email protected] None Our Dermatol Online. 2014; 5(2): 151-154 Date of submission: 02.01.2014 / acceptance: 11.02.2014 Abstract Keratosis follicularis spinulosa decalvans (KFSD) is a keratinization disorder characterized by diffuse follicular hyperkeratosis, progressive cicatricial alopecia, corneal dystrophy, and photophobia. Acne keloidalis nuchae (AKN) is a syndrome of chronic folliculitis that manifests as follicular-based pustules and papules on the occipital region of the scalp, which may eventually lead to cicatricial alopecia. Various diseases such as cutis laksa, deafness, aminoaciduria, mental retardation, and atopy have been reported to be associated with KFSD, but AKN is a rare cutaneous manifestation. Herein, we report the case of a patient with KFSD associated with AKN. He was presented to our clinic with follicular-based pustules and papules that had been progressively advancing for five years that were now manifesting as cicatricial alopecia. Key words: Keratosis follicularis; cicatricial alopecia; acne keloidalis nuchae Cite this article: Topal IO, Sahin IM, Berberoglu B, Ozer M. Keratosıs follıcularıs spınulosa decalvans assocıated wıth acne keloıdalıs nuchae. Our Dermatol Online. 2014; 5(2): 151-154. Introduction A dermatological examination revealed a cicatricial plaque In 1905, Lamaris first described keratosis follicularis with a diameter of 7x8 cm, follicular tufting, especially on the spinulosa decalvans (KFSD) as ichthyosis follicularis [1]. -
Alopecia with Perifollicular Papules and Pustules
Tyler A. Moss, DO; Thomas M. Alopecia with perifollicular Beachkofsky, MD; Samuel F. Almquist, MD; Oliver J. Wisco, DO; papules and pustules Michael R. Murchland, MD A.T. Still University, Our 23-year-old patient thought his hair loss was Kirksville College of Osteopathic Medicine, probably “genetic.” But that didn’t explain the painful Kirksville, Mo (Dr. Moss); Kunsan AB, Republic of pustules. Korea (Dr. Beachkofsky); Wilford Hall Medical Center, Lackland AFB, Tex (Drs. Almquist, Wisco, and Murchland) A 23-year-old African American man Physical examination revealed multiple sought care at our medical center because perifollicular papules and pustules on the [email protected] he had been losing hair over the vertex of his vertex of his scalp with interspersed patches DEPARTMENT EDITOR scalp for the past several years. He indicated of alopecia (FIGURE 1). Th ere were no lesions Richard P. Usatine, MD that his father had early-onset male patterned elsewhere on his body and his past medical University of Texas Health alopecia. As a result, he considered his hair history was otherwise unremarkable. Science Center at San Antonio loss “genetic.” However, he described waxing and waning fl ares of painful pustules associ- ● The authors reported no WHAT IS YOUR DIAGNOSIS? potential confl ict of interest ated with occasional spontaneous bleeding relevant to this article. and discharge of purulent material that oc- ● HOW WOULD YOU MANAGE curred in the same area as the hair loss. THIS PATIENT? FIGURE 1 Alopecia with a painful twist A B PHOTOS COURTESY OF: OLIVER J. WISCO, DO PHOTOS COURTESY This 23-year-old patient said that he had spontaneous bleeding and discharge of purulent material in the area of his hair loss.