sign in sign up
<<
Home , Alopecia areata, Hair loss, Hypertrichosis, Loose anagen syndrome, Noonan syndrome, Telogen effluvium

in a 2-year-old Female: A Case Report and Review of the Literature

Mathew Koehler, DO,* Anne Nguyen, MS,** Navid Nami, DO***

* Resident, 2nd year, Opti-West/College Medical Center, Long Beach, CA ** Medical Student, 4th Year, Western University of Health Sciences, College of Osteopathic Medicine, Pomona, CA *** Dermatology Residency Program Director, Opti-West/College Medical Center, Long Beach, CA

Abstract Loose anagen syndrome is a rare condition of abnormal cornification leading to excessive and painless loss of anagen from the . The condition most commonly affects young females with blonde hair, but males and those with darker hair colors can be affected. Patients are known to have short, sparse hair that does not need cutting, and hairs are easily and painlessly plucked from the scalp. No known treatment exists for this rare disorder, but many patients improve with age. Case Report neck line. The patient had no notable medical Discussion We present the case of a 27-month-old female history and took no daily medicines. An older Loose anagen syndrome is an uncommon presenting to the clinic with a chief complaint brother and sister had no similar findings. She condition characterized by loosely attached hairs of diffuse for the last five months. The was growing well and meeting all developmental of the scalp leading to diffuse thinning with poor mother stated that she began finding large clumps milestones. The mother denied any major growth, thus requiring few haircuts. It was first of hair throughout the house, most notably in the traumas, psychologically stressful periods or any described in 1984 by Zaun, who called it “syndrome child’s play area. She stated that the condition major illnesses that the patient or the family of loosely attached hair in childhood.”1 A few had progressed to where she is afraid to wash experienced in the last year. The mother denied years later, Price and Gummer along with Hamm or her hair and is exceptionally careful any hair manipulation or hair-pulling behaviors and Traupe began describing similar cases in the changing her clothes, as even minor pulling on and stated that the daughter is so concerned about American literature and coined the current term the hair will result in additional loss. The mother her hair being pulled out she is now refusing to “loose anagen hair syndrome,” or LAHS.2,3 The reports that her once long, curly locks are now play in close proximity to her siblings or friends. annual incidence has been estimated at 2.5 cases short and straight, and no hair will grow past her On , we found a shy white per million, with 6:37 cases in boys as compared 4 female with sparse blond hair. Her hair reached to girls. However, it has been suggested that the Figure 1 only to the neck, and the mother stated that she condition may be underestimated in boys due to 5 does not need haircuts (Figures 1 and 2). She differences in . Cases described within appeared generally healthy and eventually began families occurring in an autosomal-dominant playing in the examination room. She had no pattern further suggest a ratio that is probably 6-8 other , dental or findings. Her , closer to 1. and eye lashes were unaffected. The classical clinical picture is that of a young girl Laboratory evaluation done by her pediatrician, with blonde hair that can be easily and painlessly including complete blood count, renal panels, plucked. Even so, cases do occur frequently in liver panel, anti-nuclear antibody and boys and adults, as well as in individuals with studies, were all within normal limits. dark hair. Recent formal reports document cases from Egypt and India.8,9 Three phenotypes, types A hair-pull test was done, with more than 10 hairs A, B, and C, have been described. In Type A, hair being pulled without pain. The mom and patient is sparse and does not grow long. In Type B, the were very upset when this test was done, as it was individual has unruly hair that is either diffuse or not fully explained, and refused further hair pulls. patchy. In Type C, the hair appears normal but They did allow me to pull lightly on individual 10 has excessive shedding and loose anagen hairs. hairs, which repeatedly were easily pulled from 7,11 The eyebrows and are not affected. the child’s head without pain. A trichogram was done, which showed a distorted anagen bulb with A diagnosis relies on the presence of loose anagen a “rumpled sock” appearance (Figure 3). Figure 3 Based on the history and physical examination, a diagnosis of loose anagen syndrome (LAS) was made. The mother and child were advised on the natural history of this condition and were offered a trial of 5% hair solution to be applied to her scalp daily. We encouraged her to continue being mindful of and avoid activities that would result in further hair loss such as combing, shampooing and pulling narrow-necked clothing over her head. The patient will continue to follow with us, and although not happy with her condition, they were relieved to have received a diagnosis.

Figure 2

KOEHLER, NGUYEN, NAMI Page 37 Table 1. of Pediatric Alopecia examination, the hair bulb is long, tapered, and twisted along the long axis. The appears as Condition Clinical Findings Pathology Hair-pull Test a “rumpled sock.”2,12,15 Loose Anagen Diffusely thin hair that “Rumpled sock” look >10 hairs painlessly Abnormalities in keratinization have prompted Syndrome tends to not grow beyond to anagen bulb on pulled. Individual molecular analysis studies. Particular attention the shoulders. Bald patches trichogram. hairs easily pulled. has been devoted to identifying the may be present. Hair may be within the , , dull, unruly, or matted. and companion layer. Of significance, Chaplain Erythematous and Follicular red dots Normal et al. identified a G-to-A substitution plaques with scale. Lesions on trichoscopy. On in the cytokeratin K6hf of the companion layer expand centrifugally. , vacuolar leading to replacement of glutamic acid by lysine. Follicular plugging with interface change with It is hypothesized that this mutation may lead to atrophy, scarring, and chronic of instability of the intermediate filament network telangiectasia. Dark- eccrine sweat glands and and thus poor anchoring of the hair shaft to the 7 skinned individuals arrector pili. Increased sheath. It was not until recently that keratins of 16,17 may have peripheral dermal mucin. IgG and the inner root sheath, K25-28, were described. with C3 deposition at D-E Molecular analysis for possible mutation in these central hypopigmentation. junction. genes has yet to be done. Non-scarring, round-to- Yellow dots, exclamation May be positive in Although the majority of cases have been sporadic, oval patch of hair loss. mark appearance, and the diffuse variant. as previously mentioned, there is some evidence Totalis, universalis, dystrophic hairs on of autosomal-dominant inheritance with variable and reticular variants. Nail trichoscopy. penetrance.6,7 There have been associations with changes may be present. certain conditions such as Noonan’s syndrome,13,18 May be chronic and coloboma,19 hypohidrotic ectodermal dysplasia,20 relapsing. and woolly hair.21 Patchy or full alopecia of On histology, Normal There is no agreed upon or universally effective hair-bearing areas, most incomplete, disrupted treatment for LAS. In some individuals, the commonly the scalp. Patches follicular anatomy, condition improves with age, most notably have bizarre and irregularly trichomalacia, pigment around . However, in some individuals the shaped borders with hairs casts. condition persists into adulthood. A recent case of varying lengths. Occiput report showed good results using daily therapy sparing. with minoxidil without any side effects in a 2-year- 22 Most commonly presents Comma hair on Normal old patient. While minoxidil is generally safe as alopecia with or without trichoscopy. and inexpensive, there are some considerations scale. Presentation can range with T. tonsurans (>90% when prescribing to pediatric patients. Rare cases from a non-inflammatory of cases in the U.S.) of reversible generalized have been scaling resembling results in the classic reported in children using excessive amounts of minoxidil for alopecia areata, so caution should seborrheic to black dot appearance. 23 severe pustulosis also known be used. Another consideration in pediatric as a kerion. patients is excessive systemic absorption, which could potentially cause cardiovascular symptoms Thinning involving the Mixture of normal Positive for two such as tachycardia, palpitations and dizziness, so entire scalp and other hair- anagen and telogen hairs or more normal patients and their caregivers should be advised to bearing regions. with >20% telogen hairs. telogen hairs. monitor for side effects.24 Loose anagen syndrome is an uncommon hairs that when examined under the microscope precise pathogenesis of this syndrome has yet condition that can cause a significant psychosocial display derangements involving the inner and to be elucidated. The reigning theory is that of impact in patients and families. More research possibly the outer root sheaths.12 A hair-pull inner-root-sheath derangement leading to poor is needed to fully understand the cause of this test or trichogram can be performed in order to adhesion between the cuticle of the inner root condition and to improve the limited treatment support the diagnosis, although there are several sheath and that of the hair shaft, causing poor options available. Patients should be advised that drawbacks. Few controlled studies have been anchoring. Normal anagen hair is a complex this condition is thought to be benign in nature, done in order to properly define the parameters structure requiring orderly development and and many patients’ hair normalizes with age. We for a positive test. Authors have suggested using maturation in order to achieve the proper hair have chosen to recommend minoxidil to our greater than 10 loose anagen hairs, compared to follicle. Deranged anagen follicles of LAS exhibit patient while warning the mother of the potential the usual one or two hairs in normal subjects, characteristic features under both light and of cardiovascular side effects and hypertrichosis. as the cutoff for constituting a positive pull electron microscopy.7,12,13 The keratinized cell We will continue to follow her progress. test.10 On trichogram, greater than 70% loose sheath portion of the Henle layer is abnormally anagen hairs compared to the normal 10% is thickened and tortuous. Cells are irregularly considered positive. To avoid overdiagnosis, one shaped and contain nuclear debris. In addition, References 1. Zaun H Differential diagnosis of alopecia must keep in mind that anagen hairs can be there is premature keratinization and dyskeratosis in children. In: Happle R, Grosshans E, eds. found on normal scalp; their presence is neither with pyknotic nuclei, sparse filaments, and Pediatric dermatology. Berlin: Springer; 1987. pathognomonic nor specific.13 The differential for granules, in an edematous cytoplasm. 157-166 p. LAS should include alopecia areata, tinea capitis, The Huxley layer also exhibits premature trichotillomania, , and secondary keratinization with . Lastly, the cuticle cells 2. Price VH, Gummer CL. Loose anagen .14 See Table 1 for differential. of the hair shaft and Henle layer contain vacuoles syndrome. J Am Acad Dermatol. 1989;20(2 Pt 12 1):249-56. Much research has been done, although the with irregularly arranged cells. On gross

Page 38 LOOSE ANAGEN SYNDROME IN A 2-YEAR-OLD FEMALE: A CASE REPORT AND REVIEW OF THE LITERATURE. 3. Hamm H, Traupe H. Loose anagen hair of familial association between ocular coloboma childhood: the phenomenon of easily pluckable and loose anagen syndrome. Clin Genet. hair. J Am Acad Dermatol. 1989;20(2 Pt 1):242- 1995;47(4):214-6. 8. 20. Azon-masoliver A, Ferrando J. Loose anagen 4. Sinclair R, Cargnello J, Chow CW. Loose hair in hypohidrotic ectodermal dysplasia. Pediatr anagen syndrome. Exp Dermatol. 1999;8(4):297- Dermatol. 1996;13(1):29-32. 8. 21. García-hernández MJ, Price VH, Camacho 5. Pham CM, Krejci-manwaring J. Loose anagen FM. Woolly hair associated with loose anagen hair syndrome: an underdiagnosed condition in hair. Acta Derm Venereol. 2000;80(5):388-9. males. Pediatr Dermatol. 2010;27(4):408-9. 22. Chandran NS, Oranje AP. Minoxidil 5% 6. Baden HP, Kvedar JC, Magro CM. Loose solution for topical treatment of loose anagen hair anagen hair as a cause of hereditary hair loss in syndrome. Pediatr Dermatol. 2014;31(3):389-90. children. Arch Dermatol. 1992;128(10):1349-53. 23. Herskovitz I, Freedman J, Tosti A. Minoxidil 7. Chapalain V, Winter H, Langbein L, et al. induced hypertrichosis in a 2 year-old child. Is the loose anagen hair syndrome a F1000Res. 2013;2:226. disorder? A clinical and molecular study. Arch 24. Georgala S, Befon A, Maniatopoulou Dermatol. 2002;138(4):501-6. E, Georgala C. Topical use of minoxidil in 8. Dey V, Thawani M. Loose anagen hair children and systemic side effects. Dermatology. syndrome in black-haired Indian children. 2007;214(1):101-2. Pediatr Dermatol. 2013;30(5):579-83. 9. Abdel-raouf H, El-din WH, Awad SS, et al. Loose anagen hair syndrome in children of Upper Correspondence: Matthew Michael Koehler, Egypt. J Cosmet Dermatol. 2009;8(2):103-7. DO; [email protected] 10. Olsen EA, Bettencourt MS, Coté NL. The presence of loose anagen hairs obtained by hair pull in the normal population. J Investig Dermatol Symp Proc. 1999;4(3):258-60. 11. Chapman DM, Miller RA. An objective measurement of the anchoring strength of anagen hair in an adult with the loose anagen hair syndrome. J Cutan Pathol. 1996;23(3):288-92. 12. Mirmirani P, Uno H, Price VH. Abnormal inner root sheath of the in the loose anagen hair syndrome: an ultrastructural study. J Am Acad Dermatol. 2011;64(1):129-34. 13. Tosti A, Piraccini BM. Loose anagen hair syndrome and loose anagen hair. Arch Dermatol. 2002;138(4):521-2. 14. Bolognia J, Jorizzo JL, Schaffer JV. Dermatology. 3rd edition. Philadelphia (PA): Saunders; 2012. 1093p. 15. Dicle O, Velipasaoglu S, Ozenci CC, Akkoyunlu G, Demir N. Report of a new case with loose anagen hair syndrome and scanning electron microscopy findings. Int J Dermatol. 2008;47(9):936-8. 16. Porter RM, Corden LD, Lunny DP, Smith FJ, Lane EB, Mclean WH. Keratin K6irs is specific to the inner root sheath of hair follicles in mice and humans. Br J Dermatol. 2001;145(4):558-68. 17. Langbein L, Rogers MA, Praetzel S, Winter H, Schweizer J. K6irs1, K6irs2, K6irs3, and K6irs4 represent the inner-root-sheath-specific type II epithelial keratins of the human hair follicle. J Invest Dermatol. 2003;120(4):512-22. 18. Mazzanti L, Cacciari E, Cicognani A, Bergamaschi R, Scarano E, Forabosco A. Noonan-like syndrome with loose anagen hair: a new syndrome?. Am J Med Genet A. 2003;118A(3):279-86. 19. Murphy MF, Mcginnity FG, Allen GE. New

KOEHLER, NGUYEN, NAMI Page 39