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Short anagen hair with persistent synchronized pattern of scalp hair growth
Case reports 949
J AM ACAD DERMATOL VOLUME 49, NUMBER 5
10. Sorenson RH, Cheu SH. Accidental cutaneous coccidioidomycosis in an immune person. Calif Med 1964;100:44-7. 11. Overholt EL, Hornick RB. Primary cutaneous coccidioidomycosis. Arch Intern Med 1964;114:149-53. 12. Levan NE, Huntington RW. Primary cutaneous coccidioidomycosis in agricultural workers. Arch Dermatol 1965;92:215-20. 13. Winn WA. Primary cutaneous coccidioidomycosis: reevaluation of its potentiality based on a study of three new cases. Arch Dermatol 1965;92:221-8. 14. Carroll GF, Haley LD, Brown JM. Primary cutaneous coccidioidomycosis. Arch Dermatol 1977;113:933-6. 15. Bonifaz A, Saul A, Galindo J, Andrade R. Primary cutaneous coc-
cidioidomycosis treated with itraconazole. Int J Dermatol 1994; 33:720-2. 16. Galgiani J. Coccidioides immitis. In: Mandell GL, Bennet JE, Dolin R, editors. Mandell, Douglas and Bennett’s principles and practice of infectious diseases. 5th ed. Philadelphia: Churchill Livingstone; 2000. p. 2746-57. 17. Deresinski SC, Kemper C. Coccidioides immitis. In: Gorbach SL, Bartlett JG, Blacklow NR, editors. Infectious diseases. 2nd ed. Philadelphia: WB Saunders Co; 1998. p. 2344-61. 18. Mitchell T. Coccidioidomycosis. In: Armstrong D, Cohen J, editors. Infectious diseases. London: Harcourt Publishers; 1999. p. 8.27.7-10.
Short anagen hair with persistent synchronized pattern of scalp hair growth Keng-Ee Thai, MBBS, B Med Sci, and Rodney D. Sinclair, FACD, MBBS Melbourne, Australia Unlike other mammals that molt hair seasonally, human beings have scalp hair follicles that exhibit an asynchronized pattern of cycling, with the growth and subsequent shedding of each hair being independent of others around it. This mosaic cycling behavior is established early and continues throughout life. We describe a boy who continues to have a synchronized pattern of hair growth and shedding, and a short anagen duration at 4 years of age. He appears to never have established the expected asynchronized pattern of hair growth. (J Am Acad Dermatol 2003;49:949-51.)
T
he mosaic pattern of scalp hair growth is established in the first few months after birth. There is also the gradual transition of velluslike hairs to terminal hairs during the early years of life, with an accompanying lengthening of the anagen growth phase. Described here is a boy who, at 4 years of age, continues to exhibit synchronized waves of hair growth and shedding, and a short anagen duration.
CASE REPORT A 2.5-year-old boy was brought in for assessment of short scalp hair that failed to grow. He had good scalp hair density, but up until that time had not required a haircut. In addition, the pattern of hair growth and shedding was synchronized so that waves of short hairs could be seen moving in an anterior to posterior progression (Fig 1). Both parFrom the Department of Medicine–Dermatology, University of Melbourne, St Vincent’s Hospital. Funding sources: None. Conflicts of interest: None identified. Reprints not available from authors. Copyright © 2003 by the American Academy of Dermatology, Inc. 0190-9622/2003/$30.00 ⫹ 0 doi:10.1067/S0190-9622(03)00453-5
ents had normal scalp hair growth. He had an uneventful gestation and birth, and had been otherwise healthy. Review at 4 years old found no change in his maximal hair length or shedding pattern. He had required only 1 haircut per year since the last review, simply to trim the longer hairs to a neater length consistent with the shorter areas. On examination at 4 years old, he had short, dark-brown hairs at the frontal hairline and over the occipital area, from a few millimeters to about 1 to 2 cm long (Fig 2). The midscalp region had longer hairs that varied in length from about 5 to 10 cm (Fig 3). The hair density in this region seemed to be at the lower end of the expected normal range. Hair pull test performed in various regions of the scalp was negative. Eyebrow and eyelash hairs were present and normal in length and pattern. The rest of the integument, nails, and teeth were also normal. A thorough systemic examination found no other abnormalities. The patient’s parents declined our request for a scalp biopsy. Clippings were taken from the distal portion of hairs from the occipital and frontal regions. Under light microscopy, they all showed tapered tips at the distal end, consistent with hairs that had never been cut.
950 Case reports
J AM ACAD DERMATOL NOVEMBER 2003
Fig 1. Our patient at 21⁄2 years old: short hairs at frontal (A) and occipital (B) hairlines.
Fig 2. Our patient at 4 years old: shortened hairs are still seen at frontal (A) and occipital (B) hairlines.
DISCUSSION Mammalian hair follicles show an inherent rhythm of cycling. In lower mammals, the hair cycles of the entire coat are synchronized to molt 1 to 3 times each year. These seasonal changes are a result of the influence of various environmental stimuli, especially day length, and are mediated in part by the pineal and pituitary glands. Human beings also demonstrate seasonal changes in hair growth, but changes are subtle and not usually clinically detectable. The influence of estrogen during and after pregnancy on hair growth indicates that hormonal influences are also important in human beings.1 Waves of molting are established in utero in mammals. Complete anagen hair follicles are seen over the entire scalp in a 20-week-old human fetus. The transition to catagen and then telogen occurs during the 26th to 28th week of gestation, with the first hairs shed in utero. This change starts at the anterior hairline and progresses backward; the hairs in the occipital region typically remain in anagen
phase until the end of the pregnancy before an abrupt change to telogen follicles. Thus, at birth the scalp will have almost finished molting its first hair wave. The second hair wave will be in progress, also having started from the anterior hairline and progressing backward. Follicles in the occipital region will be in telogen phase shortly after birth, and hairs will not fall out until after 8 to 12 weeks postpartum. This timing gives rise to the often observed occipital alopecia of the newborn.2 In lower mammals, the persistent hair waves are seen as seasonal molting and come under the influence of various intrinsic and environmental factors. In human beings, synchronized hair waves are persistent at least for the first 4 months; the pattern of hair shedding typically becomes mosaic by the end of the first year of life. However, follicles are often grouped by 3, with each of these Meije´ res trio groups exhibiting synchronous cycling within themselves.1 There is a progressive transition of velluslike hairs to intermediate and, finally, terminal hairs dur-
J AM ACAD DERMATOL VOLUME 49, NUMBER 5
Fig 3. Midscalp hairs, longest being about 10 cm.
ing the first few years of life. The duration of anagen phase presumably lengthens to reflect the increased size and length of hair. A short anagen phase has been associated with various hair disorders. Kersey3 documented the trichodental syndrome as having a shortened anagen phase with an increase in the number of hairs in telogen phase. There appears to be an autosomal dominant pattern of inheritance with complex incomplete expression. There is hypotrichosis from birth and associated abnormal dentition. Hereditary hypotrichosis simplex4 is an autosomal dominant disorder. Children are born with a normal scalp hair growth pattern. The disease manifests between age 5 and 12 years, when hair growth slows and there is progressive diffuse hair loss. The terminal state is reached at about 20 to 25 years, when there are only a few sparse, fine, short scalp hairs remaining. On the basis of scalp histology findings, there is a progressive shortening of the anagen phase with disease progression; at the end stage is an anagen length similar to that of vellus follicles, producing cosmetically insignificant hair shafts. Several kindred with hereditary hypotrichosis simplex have been described.5-7 de Berker,8 in his review on congenital
Case reports 951
hypotrichosis, describes patients in whom a reduced duration of anagen hairs is the only suggested abnormality. These patients tend to present in early childhood and improve by puberty. A subset of telogen effluvium can also be explained by idiopathic shortening of anagen stage, with increased shedding and decreased hair length.9 A recent report describes 2 patients with isolated congenital hypotrichosis where a shortened anagen phase was also demonstrated.10 All the above conditions seem to either improve or worsen with time. None, however, describe persistent synchronized shedding as part of their syndrome. It is unclear what factors control the transition from synchronized to asynchronized hair growth, or what the exact triggers are. The patient described here retained the persistent molting waves established in utero. In addition, the duration of the anagen phase on the scalp seemed to be persistently short. The longest hairs were about 10 cm, indicating an anagen duration of approximately 10 months. To our knowledge, a short anagen syndrome with persistent hair waves has not been previously reported. There is no satisfactory explanation for this finding, but the coexistence of both short anagen phase and synchronized hair growth pattern may indicate that the control for the duration and pattern of hair growth are identical or closely related. REFERENCES 1. Messenger AG, Dawber RPR. The physiology and embryology of hair growth. In: Dawber RPR, editor. Diseases of the hair and scalp. 3rd ed. Oxford: Blackwell Science; 1997. p. 1-22. 2. Barth JH. The hair in infancy and childhood. In: Dawber RPR, editor. Diseases of the hair and scalp. 3rd ed. Oxford: Blackwell Science; 1997. p. 51-66. 3. Kersey PJW. Tricho-dental syndrome: a disorder with a short hair cycle. Br J Dermatol 1987;116:259-63. 4. Toribio J, Quinones PA. Hereditary hypotrichosis simplex of the scalp. Br J Dermatol 1974;91:687-96. 5. Just M, Ribera M, Fuente MJ, Bielsa I, Ferrandiz C. Hereditary hypotrichosis simplex. Dermatology 1998;196:339-42. 6. Kohn G, Metzker A. Hereditary hypotrichosis simplex of the scalp. Clin Genet 1987;32:120-4. 7. Diaz ER, Blasco GF, Pascual AM, Armijo M. Hereditary hypotrichosis simplex of the scalp. Dermatology 1995;191:139-41. 8. de Berker D. Congenital hyptorichosis. Int J Dermatol 1999;38: 25-33. 9. Headington JT. Telogen effluvium. Arch Dermatol 1993;129: 356-63. 10. Barraud-Klenovesk MM, Tru¨eb RM. Congenital hypotrichosis due to short anagen. Br J Dermatol 2000;143:612-7.
J AM ACAD DERMATOL VOLUME 49, NUMBER 5
10. Sorenson RH, Cheu SH. Accidental cutaneous coccidioidomycosis in an immune person. Calif Med 1964;100:44-7. 11. Overholt EL, Hornick RB. Primary cutaneous coccidioidomycosis. Arch Intern Med 1964;114:149-53. 12. Levan NE, Huntington RW. Primary cutaneous coccidioidomycosis in agricultural workers. Arch Dermatol 1965;92:215-20. 13. Winn WA. Primary cutaneous coccidioidomycosis: reevaluation of its potentiality based on a study of three new cases. Arch Dermatol 1965;92:221-8. 14. Carroll GF, Haley LD, Brown JM. Primary cutaneous coccidioidomycosis. Arch Dermatol 1977;113:933-6. 15. Bonifaz A, Saul A, Galindo J, Andrade R. Primary cutaneous coc-
cidioidomycosis treated with itraconazole. Int J Dermatol 1994; 33:720-2. 16. Galgiani J. Coccidioides immitis. In: Mandell GL, Bennet JE, Dolin R, editors. Mandell, Douglas and Bennett’s principles and practice of infectious diseases. 5th ed. Philadelphia: Churchill Livingstone; 2000. p. 2746-57. 17. Deresinski SC, Kemper C. Coccidioides immitis. In: Gorbach SL, Bartlett JG, Blacklow NR, editors. Infectious diseases. 2nd ed. Philadelphia: WB Saunders Co; 1998. p. 2344-61. 18. Mitchell T. Coccidioidomycosis. In: Armstrong D, Cohen J, editors. Infectious diseases. London: Harcourt Publishers; 1999. p. 8.27.7-10.
Short anagen hair with persistent synchronized pattern of scalp hair growth Keng-Ee Thai, MBBS, B Med Sci, and Rodney D. Sinclair, FACD, MBBS Melbourne, Australia Unlike other mammals that molt hair seasonally, human beings have scalp hair follicles that exhibit an asynchronized pattern of cycling, with the growth and subsequent shedding of each hair being independent of others around it. This mosaic cycling behavior is established early and continues throughout life. We describe a boy who continues to have a synchronized pattern of hair growth and shedding, and a short anagen duration at 4 years of age. He appears to never have established the expected asynchronized pattern of hair growth. (J Am Acad Dermatol 2003;49:949-51.)
T
he mosaic pattern of scalp hair growth is established in the first few months after birth. There is also the gradual transition of velluslike hairs to terminal hairs during the early years of life, with an accompanying lengthening of the anagen growth phase. Described here is a boy who, at 4 years of age, continues to exhibit synchronized waves of hair growth and shedding, and a short anagen duration.
CASE REPORT A 2.5-year-old boy was brought in for assessment of short scalp hair that failed to grow. He had good scalp hair density, but up until that time had not required a haircut. In addition, the pattern of hair growth and shedding was synchronized so that waves of short hairs could be seen moving in an anterior to posterior progression (Fig 1). Both parFrom the Department of Medicine–Dermatology, University of Melbourne, St Vincent’s Hospital. Funding sources: None. Conflicts of interest: None identified. Reprints not available from authors. Copyright © 2003 by the American Academy of Dermatology, Inc. 0190-9622/2003/$30.00 ⫹ 0 doi:10.1067/S0190-9622(03)00453-5
ents had normal scalp hair growth. He had an uneventful gestation and birth, and had been otherwise healthy. Review at 4 years old found no change in his maximal hair length or shedding pattern. He had required only 1 haircut per year since the last review, simply to trim the longer hairs to a neater length consistent with the shorter areas. On examination at 4 years old, he had short, dark-brown hairs at the frontal hairline and over the occipital area, from a few millimeters to about 1 to 2 cm long (Fig 2). The midscalp region had longer hairs that varied in length from about 5 to 10 cm (Fig 3). The hair density in this region seemed to be at the lower end of the expected normal range. Hair pull test performed in various regions of the scalp was negative. Eyebrow and eyelash hairs were present and normal in length and pattern. The rest of the integument, nails, and teeth were also normal. A thorough systemic examination found no other abnormalities. The patient’s parents declined our request for a scalp biopsy. Clippings were taken from the distal portion of hairs from the occipital and frontal regions. Under light microscopy, they all showed tapered tips at the distal end, consistent with hairs that had never been cut.
950 Case reports
J AM ACAD DERMATOL NOVEMBER 2003
Fig 1. Our patient at 21⁄2 years old: short hairs at frontal (A) and occipital (B) hairlines.
Fig 2. Our patient at 4 years old: shortened hairs are still seen at frontal (A) and occipital (B) hairlines.
DISCUSSION Mammalian hair follicles show an inherent rhythm of cycling. In lower mammals, the hair cycles of the entire coat are synchronized to molt 1 to 3 times each year. These seasonal changes are a result of the influence of various environmental stimuli, especially day length, and are mediated in part by the pineal and pituitary glands. Human beings also demonstrate seasonal changes in hair growth, but changes are subtle and not usually clinically detectable. The influence of estrogen during and after pregnancy on hair growth indicates that hormonal influences are also important in human beings.1 Waves of molting are established in utero in mammals. Complete anagen hair follicles are seen over the entire scalp in a 20-week-old human fetus. The transition to catagen and then telogen occurs during the 26th to 28th week of gestation, with the first hairs shed in utero. This change starts at the anterior hairline and progresses backward; the hairs in the occipital region typically remain in anagen
phase until the end of the pregnancy before an abrupt change to telogen follicles. Thus, at birth the scalp will have almost finished molting its first hair wave. The second hair wave will be in progress, also having started from the anterior hairline and progressing backward. Follicles in the occipital region will be in telogen phase shortly after birth, and hairs will not fall out until after 8 to 12 weeks postpartum. This timing gives rise to the often observed occipital alopecia of the newborn.2 In lower mammals, the persistent hair waves are seen as seasonal molting and come under the influence of various intrinsic and environmental factors. In human beings, synchronized hair waves are persistent at least for the first 4 months; the pattern of hair shedding typically becomes mosaic by the end of the first year of life. However, follicles are often grouped by 3, with each of these Meije´ res trio groups exhibiting synchronous cycling within themselves.1 There is a progressive transition of velluslike hairs to intermediate and, finally, terminal hairs dur-
J AM ACAD DERMATOL VOLUME 49, NUMBER 5
Fig 3. Midscalp hairs, longest being about 10 cm.
ing the first few years of life. The duration of anagen phase presumably lengthens to reflect the increased size and length of hair. A short anagen phase has been associated with various hair disorders. Kersey3 documented the trichodental syndrome as having a shortened anagen phase with an increase in the number of hairs in telogen phase. There appears to be an autosomal dominant pattern of inheritance with complex incomplete expression. There is hypotrichosis from birth and associated abnormal dentition. Hereditary hypotrichosis simplex4 is an autosomal dominant disorder. Children are born with a normal scalp hair growth pattern. The disease manifests between age 5 and 12 years, when hair growth slows and there is progressive diffuse hair loss. The terminal state is reached at about 20 to 25 years, when there are only a few sparse, fine, short scalp hairs remaining. On the basis of scalp histology findings, there is a progressive shortening of the anagen phase with disease progression; at the end stage is an anagen length similar to that of vellus follicles, producing cosmetically insignificant hair shafts. Several kindred with hereditary hypotrichosis simplex have been described.5-7 de Berker,8 in his review on congenital
Case reports 951
hypotrichosis, describes patients in whom a reduced duration of anagen hairs is the only suggested abnormality. These patients tend to present in early childhood and improve by puberty. A subset of telogen effluvium can also be explained by idiopathic shortening of anagen stage, with increased shedding and decreased hair length.9 A recent report describes 2 patients with isolated congenital hypotrichosis where a shortened anagen phase was also demonstrated.10 All the above conditions seem to either improve or worsen with time. None, however, describe persistent synchronized shedding as part of their syndrome. It is unclear what factors control the transition from synchronized to asynchronized hair growth, or what the exact triggers are. The patient described here retained the persistent molting waves established in utero. In addition, the duration of the anagen phase on the scalp seemed to be persistently short. The longest hairs were about 10 cm, indicating an anagen duration of approximately 10 months. To our knowledge, a short anagen syndrome with persistent hair waves has not been previously reported. There is no satisfactory explanation for this finding, but the coexistence of both short anagen phase and synchronized hair growth pattern may indicate that the control for the duration and pattern of hair growth are identical or closely related. REFERENCES 1. Messenger AG, Dawber RPR. The physiology and embryology of hair growth. In: Dawber RPR, editor. Diseases of the hair and scalp. 3rd ed. Oxford: Blackwell Science; 1997. p. 1-22. 2. Barth JH. The hair in infancy and childhood. In: Dawber RPR, editor. Diseases of the hair and scalp. 3rd ed. Oxford: Blackwell Science; 1997. p. 51-66. 3. Kersey PJW. Tricho-dental syndrome: a disorder with a short hair cycle. Br J Dermatol 1987;116:259-63. 4. Toribio J, Quinones PA. Hereditary hypotrichosis simplex of the scalp. Br J Dermatol 1974;91:687-96. 5. Just M, Ribera M, Fuente MJ, Bielsa I, Ferrandiz C. Hereditary hypotrichosis simplex. Dermatology 1998;196:339-42. 6. Kohn G, Metzker A. Hereditary hypotrichosis simplex of the scalp. Clin Genet 1987;32:120-4. 7. Diaz ER, Blasco GF, Pascual AM, Armijo M. Hereditary hypotrichosis simplex of the scalp. Dermatology 1995;191:139-41. 8. de Berker D. Congenital hyptorichosis. Int J Dermatol 1999;38: 25-33. 9. Headington JT. Telogen effluvium. Arch Dermatol 1993;129: 356-63. 10. Barraud-Klenovesk MM, Tru¨eb RM. Congenital hypotrichosis due to short anagen. Br J Dermatol 2000;143:612-7.