loose anagen hair syndrome: an unusual cause of alopecia in a child

1
P2102 Profile of alopecia areata in 655 Chinese patients SiSi Qi, MD, Department of Dermatology, Huashan Hospital, Fudan University, Shanghai, China; Feng Xu, MD, Department of Dermatology, Huashan Hospital, Fudan University, Shanghai, China; QinPing Yang, MD, Department of Dermatology, Huashan Hospital, Fudan University, Shanghai, China; WeiWei Dai, DO, Texas College of Osteopathic Medicine, University of North Texas Health Science Center, Fort Worth, TX, United States Background: Alopecia areata (AA) is an autoimmune disorder requiring both genetic and environmental factors for manifestation. The native Chinese population may differ from other ethnicities in both factors, contributing to clinical variability. The clinical profile of AA in the Chinese population has not been adequately studied. Objective: To investigate the demographic features, clinical characteristics, and quality of life (QoL) of AA in Chinese patients. Methods: Six hundred fifty-five patients (320 men and 335 women) newly diagnosed with AA were recruited as part of a prospective study between January 2006 and May 2008 at Huashan Hospital, Shanghai, China. Demographic data, medical history, and family history were collected. Patients were categorized by disease subtype. Each patient was evaluated for disease onset, duration, severity, and relapse. Psychological impact of AA was assessed by a validated Chinese version of Dermatology Life Quality Index (DLQI) questionnaires. Results: Three clinical subtypes of alopecia were observed in this study: patchy alopecia, alopecia totalis (AT), and alopecia universalis (AU). Overall mean age of onset was 35.2 years with a mean duration of 14.79 months. Patchy alopecia was observed in the majority (86.6%) of patients while a minority (13.4%) exhibited alopecia totalis or universalis (AT/AU). However, AT/AU patients exhibited earlier age of onset (29.4 vs 36.1 years; P ¼.001) and longer disease duration (43.1 vs 10.3 months; P \.001) than patchy AA. Disease relapse was present in 29.8% of patients. Patients with recurrent versus primary disease also exhibited an earlier age of onset (28.6 vs 38.0 years; P \.001) and longer disease duration (32.5 vs 7.8 months; P \ .001). Females demonstrated a higher rate of relapse (P ¼ .004). Positive family history was observed in 12.6% of patients, with a higher frequency in females (P ¼ .007). A medical history of at least one atopic disorder was observed in 9.9% of patients. Atopy was found to be associated with an earlier age of onset (P ¼.026) and more extensive disease (P ¼.022). QoL, demonstrated by DLQI scores, ranged from 0 to 29 with a mean 6 SD of 5.4 6 5.5. Mean scores of patients with AT/AU were higher compared to patchy AA (8.0 vs 5.1; P \.001). Patients with recurrent versus primary disease also exhibited higher scores (6.8 vs 4.9; P \.001). Conclusion: In this study, patients with AT/AU had earlier age of onset, longer disease duration, and quality of life was more severely impacted than patients with patchy AA. Similar observations were made for patients with recurrent disease. In addition, history of atopy was associated with greater disease severity. Commercial support: None identified. P2103 Loose anagen hair syndrome: An unusual cause of alopecia in a child Felicidade Santiago, MD, Dermatology Department, Coimbra, Portugal; Ame ´rico Figueiredo, MD, PhD, Dermatology Department, Coimbra, Portugal; Ricardo Vieira, MD, Dermatology Department, Coimbra, Portugal Introduction: Loose anagen hair syndrome (LAHS) is characterized by the ability to easily and painlessly extract unsheathed anagen hairs from the scalp with only a gentle hair pull. This is an unusual benign hair disorder of childhood that is considered to be underdiagnosed, with no more than 100 patients previously reported in the literature. Case report: A 5 year-old girl was referred to our clinic because of a 5-month history of diffuse hair loss. Her hair was blond and dull with evident patches of alopecia. There were no signs of scalp inflammation or scarring. The condition did not affect the eyebrows or eyelashes. The girl was in good health and had no abnormalities of her nails, teeth, skin, or eyes. A hair pull test revealed multiple hairs, easily and painlessly extracted. Light microscopic examination and trichogram were consis- tent with loose anagen hair syndrome. Discussion: The diagnosis of LAHS is essentially clinical and is confirmed by firmly pulling locks of hair and microscopically examination of typical loose anagen hairs. With the present case report, the authors highlight that even though hair loss in children raises a considerable concern, LAHS is of cosmetic importance only and improvement occurs with aging. Diagnostic tests, other than trichogram and hair microscopic examination, and unnecessary treatments should be avoided. Commercial support: None identified. P2104 Alopecia areata and primary sclerosing cholangitis Ana Moreira, MD, CHVNGaia/Espinho, Vila Nova de Gaia, Portugal; Armando Baptista, MD, CHVNGaia/Espinho, Vila Nova de Gaia, Portugal; Paulo Varela, MD, CHVNGaia/Espinho, Vila Nova de Gaia, Portugal; Rita Guedes, MD, CHVNGaia/Espinho, Vila Nova de Gaia, Portugal; Rolando Pinho, MD, CHVNGaia/Espinho, Vila Nova de Gaia, Portugal We present a 33-year-old man with a 2-year history of extensive alopecia areata with patchy, confluent hair loss of the scalp. The remaining dermatologic examination was normal. He was referred by the gastroenterology consultation with the diagnosis of small duct primary sclerosing cholangitis. He presented chronic cholestasis with persistent high levels of alkaline phosphatase (AlkP), g-glutamyl transpeptidase (GGT), alanine aminotranferase (ALT), and bilirubin discrete eleva- tion. The patient had no symptoms and these alterations were noticed in a routine serum analyses. The peripheral antineutrophil cytoplasmic antibody (P-ANCA) was positive (1/160). The hepatic histology was consistent with primary sclerosing cholangitis showing intrahepatic bile duct inflammation and fibrosis but with normal findings on magnetic resonance cholangiography. A normal colonoscopy and upper endoscopy excluded the hypothesis of inflammatory bowel disease. Drug history, viral serologies, a1 antitrypsin, and ceruloplasmin were negative. The renal and thyroid functions were normal as well as the remaining immunologic study. Improvement of alopecia areata has been difficult despite treatment with oral cyclosporine (4mg/kg/day), topical, intralesional, and systemic glucocorticoids, and PUVA. Control of hepatic disease parallels alopecia areata although ingestion of ursodeoxycholic acid (12mg/kg/day). There is an increased incidence of autoim- mune diseases in patients with alopecia areata, particularly thyroid-related disease. Its pathogenesis is still obscure, although most authors tend to classify alopecia areata as an autoimmune disease. To our knowledge, this is the third case that reports the association between alopecia areata and primary sclerosing cholangitis. Commercial support: None identified. P2105 Incidence and risk factors for neonatal occipital alopecia: A retrospective study Bong Seok Shin, MD, Department of Dermatology, School of Medicine, Chosun University, Gwangju, South Korea; Chan Ho Na, MD, Department of Dermatology, School of Medicine, Chosun University, Gwangju, South Korea Background: For many years, the etiology of neonatal occipital alopecia (NOA) ocurring on the occiput during the early months of life has been thought to be friction caused by the neonate’s sleeping position. It is recently clear that NOA has no relationship with the sleep position but with the physiologic shedding of hair in early neonatal life. However, very few studies have focused on epidemiology and etiology of NOA. Objective: We sought to evaluate the incidence and risk factors for NOA. Methods: Medical records of 240 postpartum patients delivered in the obstetrics department at Chosun University Hospital between January 2006 and June 2007 were reviewed to obtain data on gravid females and their neonatal baseline characteristics. Telephone interviews with 193 respondents among them were conducted to investigate the actual conditions of babies’ NOA. Along with record reviews, the related clinical data including the presence of NOA, initial occurrence and restoration time of NOA, baby’s sleeping position, hair volume at birth, and the presence of maternal postpartum telogen effluvium were asked to assess maternal correlation and fetal risk factors of NOA. Results: Among 193 respondents, the presence of NOA was in 39 babies (20.2%). Bivariate analyses showed that NOA was not significantly associated with sleeping position, hair volume at birth, birth weight, gender of baby, and parity and postpartum telogen effluvium of mother, but was significantly associated with gestational age, maternal age, and delivery method (P \.05). In multivariate logistic regression analyses, the maternal risk of NOA was higher in subjects delivered after 38 weeks’ gestational age (odds ratio [OR], 3.46; 95% confidence interval [CI], 1.22- 9.79), in subjects younger than 36 years of parturition age (OR, 3.80; 95% CI, 1.06- 13.65), and in subjects not undergoing a Caesarean section (OR, 2.38; 95% CI, 1.06- 5.33). Conclusion: The fetus-related factors (eg, the baby’s sleeping position, etc) are not associated with NOA, while the maternal pregnancy-related factore, such as mature gestational age, younger gravida ( \36 years old), and nonsurgical delivery are associated with babies’ NOA in this study population. This finding raises possibility that NOA is a physiologic condition resulting from synchronised shedding of telogen hairs initiated in utero, because it is more affected by usual pregnant state than by unusual pregnant state in our study. Commercial support: None identified. MARCH 2010 JAM ACAD DERMATOL AB75

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Page 1: Loose anagen hair syndrome: An unusual cause of alopecia in a child

P2102Profile of alopecia areata in 655 Chinese patients

SiSi Qi, MD, Department of Dermatology, Huashan Hospital, Fudan University,Shanghai, China; Feng Xu, MD, Department of Dermatology, Huashan Hospital,Fudan University, Shanghai, China; QinPing Yang, MD, Department ofDermatology, Huashan Hospital, Fudan University, Shanghai, China; WeiWeiDai, DO, Texas College of Osteopathic Medicine, University of North TexasHealth Science Center, Fort Worth, TX, United States

Background: Alopecia areata (AA) is an autoimmune disorder requiring both geneticand environmental factors for manifestation. The native Chinese population maydiffer from other ethnicities in both factors, contributing to clinical variability. Theclinical profile of AA in the Chinese population has not been adequately studied.

Objective: To investigate the demographic features, clinical characteristics, andquality of life (QoL) of AA in Chinese patients.

Methods: Six hundred fifty-five patients (320 men and 335 women) newly diagnosedwith AA were recruited as part of a prospective study between January 2006 andMay 2008 at Huashan Hospital, Shanghai, China. Demographic data, medical history,and family history were collected. Patients were categorized by disease subtype.Each patient was evaluated for disease onset, duration, severity, and relapse.Psychological impact of AA was assessed by a validated Chinese version ofDermatology Life Quality Index (DLQI) questionnaires.

Results: Three clinical subtypes of alopecia were observed in this study: patchyalopecia, alopecia totalis (AT), and alopecia universalis (AU). Overall mean age ofonset was 35.2 years with a mean duration of 14.79 months. Patchy alopecia wasobserved in the majority (86.6%) of patients while a minority (13.4%) exhibitedalopecia totalis or universalis (AT/AU). However, AT/AU patients exhibited earlierage of onset (29.4 vs 36.1 years; P ¼.001) and longer disease duration (43.1 vs 10.3months; P\.001) than patchy AA. Disease relapse was present in 29.8% of patients.Patients with recurrent versus primary disease also exhibited an earlier age of onset(28.6 vs 38.0 years; P \.001) and longer disease duration (32.5 vs 7.8 months; P \.001). Females demonstrated a higher rate of relapse (P ¼ .004). Positive familyhistory was observed in 12.6% of patients, with a higher frequency in females (P ¼.007). A medical history of at least one atopic disorder was observed in 9.9% ofpatients. Atopy was found to be associated with an earlier age of onset (P¼.026) andmore extensive disease (P¼.022). QoL, demonstrated by DLQI scores, ranged from0 to 29 with a mean 6 SD of 5.4 6 5.5. Mean scores of patients with AT/AU werehigher compared to patchy AA (8.0 vs 5.1; P\.001). Patients with recurrent versusprimary disease also exhibited higher scores (6.8 vs 4.9; P \.001).

Conclusion: In this study, patients with AT/AU had earlier age of onset, longerdisease duration, and quality of life was more severely impacted than patients withpatchy AA. Similar observations were made for patients with recurrent disease. Inaddition, history of atopy was associated with greater disease severity.

MARCH 2

cial support: None identified.

Commer

P2103Loose anagen hair syndrome: An unusual cause of alopecia in a child

Felicidade Santiago, MD, Dermatology Department, Coimbra, Portugal; AmericoFigueiredo, MD, PhD, Dermatology Department, Coimbra, Portugal; RicardoVieira, MD, Dermatology Department, Coimbra, Portugal

Introduction: Loose anagen hair syndrome (LAHS) is characterized by the ability toeasily and painlessly extract unsheathed anagen hairs from the scalp with only agentle hair pull. This is an unusual benign hair disorder of childhood that isconsidered to be underdiagnosed, with no more than 100 patients previouslyreported in the literature.

Case report: A 5 year-old girl was referred to our clinic because of a 5-month historyof diffuse hair loss. Her hair was blond and dull with evident patches of alopecia.There were no signs of scalp inflammation or scarring. The condition did not affectthe eyebrows or eyelashes. The girl was in good health and had no abnormalities ofher nails, teeth, skin, or eyes. A hair pull test revealed multiple hairs, easily andpainlessly extracted. Light microscopic examination and trichogram were consis-tent with loose anagen hair syndrome.

Discussion: The diagnosis of LAHS is essentially clinical and is confirmed by firmlypulling locks of hair and microscopically examination of typical loose anagen hairs.With the present case report, the authors highlight that even though hair loss inchildren raises a considerable concern, LAHS is of cosmetic importance only andimprovement occurs with aging. Diagnostic tests, other than trichogram and hairmicroscopic examination, and unnecessary treatments should be avoided.

cial support: None identified.

Commer

010

P2104Alopecia areata and primary sclerosing cholangitis

Ana Moreira, MD, CHVNGaia/Espinho, Vila Nova de Gaia, Portugal; ArmandoBaptista, MD, CHVNGaia/Espinho, Vila Nova de Gaia, Portugal; Paulo Varela, MD,CHVNGaia/Espinho, Vila Nova de Gaia, Portugal; Rita Guedes, MD,CHVNGaia/Espinho, Vila Nova de Gaia, Portugal; Rolando Pinho, MD,CHVNGaia/Espinho, Vila Nova de Gaia, Portugal

We present a 33-year-old man with a 2-year history of extensive alopecia areata withpatchy, confluent hair loss of the scalp. The remaining dermatologic examinationwas normal. He was referred by the gastroenterology consultation with thediagnosis of small duct primary sclerosing cholangitis. He presented chroniccholestasis with persistent high levels of alkaline phosphatase (AlkP), g-glutamyltranspeptidase (GGT), alanine aminotranferase (ALT), and bilirubin discrete eleva-tion. The patient had no symptoms and these alterations were noticed in a routineserum analyses. The peripheral antineutrophil cytoplasmic antibody (P-ANCA) waspositive (1/160). The hepatic histology was consistent with primary sclerosingcholangitis showing intrahepatic bile duct inflammation and fibrosis but withnormal findings on magnetic resonance cholangiography. A normal colonoscopyand upper endoscopy excluded the hypothesis of inflammatory bowel disease. Drughistory, viral serologies, a1 antitrypsin, and ceruloplasmin were negative. The renaland thyroid functions were normal as well as the remaining immunologic study.Improvement of alopecia areata has been difficult despite treatment with oralcyclosporine (4mg/kg/day), topical, intralesional, and systemic glucocorticoids, andPUVA. Control of hepatic disease parallels alopecia areata although ingestion ofursodeoxycholic acid (12mg/kg/day). There is an increased incidence of autoim-mune diseases in patients with alopecia areata, particularly thyroid-related disease.Its pathogenesis is still obscure, although most authors tend to classify alopeciaareata as an autoimmune disease. To our knowledge, this is the third case thatreports the association between alopecia areata and primary sclerosing cholangitis.

cial support: None identified.

Commer

P2105Incidence and risk factors for neonatal occipital alopecia: A retrospectivestudy

Bong Seok Shin, MD, Department of Dermatology, School of Medicine, ChosunUniversity, Gwangju, South Korea; Chan Ho Na, MD, Department ofDermatology, School of Medicine, Chosun University, Gwangju, South Korea

Background: For many years, the etiology of neonatal occipital alopecia (NOA)ocurring on the occiput during the early months of life has been thought to befriction caused by the neonate’s sleeping position. It is recently clear that NOA hasno relationship with the sleep position but with the physiologic shedding of hair inearly neonatal life. However, very few studies have focused on epidemiology andetiology of NOA.

Objective: We sought to evaluate the incidence and risk factors for NOA.

Methods: Medical records of 240 postpartum patients delivered in the obstetricsdepartment at Chosun University Hospital between January 2006 and June 2007were reviewed to obtain data on gravid females and their neonatal baselinecharacteristics. Telephone interviews with 193 respondents among them wereconducted to investigate the actual conditions of babies’ NOA. Along with recordreviews, the related clinical data including the presence of NOA, initial occurrenceand restoration time of NOA, baby’s sleeping position, hair volume at birth, and thepresence of maternal postpartum telogen effluvium were asked to assess maternalcorrelation and fetal risk factors of NOA.

Results: Among 193 respondents, the presence of NOA was in 39 babies (20.2%).Bivariate analyses showed that NOA was not significantly associated with sleepingposition, hair volume at birth, birth weight, gender of baby, and parity andpostpartum telogen effluvium of mother, but was significantly associated withgestational age, maternal age, and delivery method (P\.05). In multivariate logisticregression analyses, the maternal risk of NOA was higher in subjects delivered after38 weeks’ gestational age (odds ratio [OR], 3.46; 95% confidence interval [CI], 1.22-9.79), in subjects younger than 36 years of parturition age (OR, 3.80; 95% CI, 1.06-13.65), and in subjects not undergoing a Caesarean section (OR, 2.38; 95% CI, 1.06-5.33).

Conclusion: The fetus-related factors (eg, the baby’s sleeping position, etc) are notassociated with NOA, while the maternal pregnancy-related factore, such as maturegestational age, younger gravida (\36 years old), and nonsurgical delivery areassociated with babies’ NOA in this study population. This finding raises possibilitythat NOA is a physiologic condition resulting from synchronised shedding of telogenhairs initiated in utero, because it is more affected by usual pregnant state than byunusual pregnant state in our study.

cial support: None identified.

Commer

J AM ACAD DERMATOL AB75