REPORT
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LOOSE ANAGEN HAIR SYNDROME BRIAN PATRICK O'DONNELL, M.D., LEONARD C. SPERLING, M.D., AND WILLIAM D. JAMES, M.D.
Abstract
DISCUSSION
This recently described entity begins in childhood and is characterized by actively growing hairs that can be easily and painlessly removed from the scalp, leading to alopecia.
Normal Anatomy Before describing the findings seen in patients with LAHS, it would be useful to review some of the normal features of human scalp hair. The vast majority of hairs are anagen, or actively growing hairs. Most of the remainder are telogen, or "resting" hairs that are about to be shed. Tbe normal range for numbers of telogen hairs is 4 to 25%, with a mean of about 15%.^ Telogen counts above this range are presumptively abnormal.' A gentle hair pull performed on a normal scalp may sometimes yield several hairs, but these hairs will always be telogen hairs. The presence of anagen hairs on a gentle hair pull is clearly abnormal. To obtain anagen bairs from the normal scalp, the hair must be forcibly plucked, requiring an instrument such as a rubber-tipped hemostat. The reason that such force is required is due to special anatomic features of the follicle, which are reviewed in detail elsewhere.^ The free edges of the cuticular cells of the inner root sheath and hair shaft interlock with each other. The cells of the inner root sheath cuticle are flat and overlap like shingles on a roof. The free edges of these cells point downward and inward, interlocking with the hair shaft cuticular cells, which point upward and outward. This intimate association requires that the inner root sheath move upward with the hair shaft
The loose anagen hair syndrome (LAHS), described in two recent reports, features relatively short, sparse, uneven hair that is easily removed from the scalp.'-^ A 1986 report in the German literature may describe the same syndrome.^ We describe a patient with LAHS and report a new observation, the loss of both inner and outer root sheaths from extracted hairs. The etiology, clinical features, light microscopy, histopathology, prognosis, and treatment of this condition are also reviewed. Case Report A 3-year-old white boy was referred for evaluation of sparse, increasingly unmanageable scalp hair. The patient seldom required a hair cut because the hair did not grow sufficiently long. He was in good health and had no abnormalities of his skin, teeth, nails, or eyes. He was taking no medicines and his diet was normal. His mother noted the onset of the graying of her hair at the age of 10, but her hair is otherwise normal. The remainder of the family history was also unremarkable. Physical examination of the scalp revealed diffuse thinning of the hair. It was light brown and appeared dry and 'blow away," especially on the occipital region (Fig. 1). Locks of hair were short and uneven. There was no inflammation of the scalp, scarring, bald patches, or unevenness of hair color. The process did not involve the eyebrows, eyelashes, or nails. A gentle hair pull was performed, using the thumb and forefinger to grasp and slowly pull strands of 20 hairs. Multiple hair pulls yielded an average of five anagen hairs that were easily and painlessly removed. The hair pull specimens from our patient revealed anagen hairs with absent outer and inner root sheaths, and ruffling of the hair cuticle (Fig. 2).
From the Dermatology Service, Walter Reed Army Medical Genter, Washington, D.G. The opinions expressed are those of the authors and are not to be construed as official or as reflecting the views of the Department of the Army or the Department of Defense. Address for correspondence: Brian P. O'Donnell, M.D., Dermatology Service, Walter Reed Army Medical Genter, Washington, DG 20307-5001.
Figure 1. Difficult to manage, "blow away" hair is most evident over the occipital region. 107
International Journal of Dermatology Vol. 31, No. 2, February 1992
"uncombable hair." This feature is also absent in some patients.^ In our patient, light microscopy revealed only a few hairs which appeared grooved. All the extracted anagen hairs in our case were missing their outer and inner root sheaths. Price and Gummer' noted the absence of the outer root sheaths in their cases. Hamm and Traupe^ found that the inner root sheaths were missing. In fact both root sheaths are left behind in the dermis. This is confirmed by the photomicrographs supplied by Price and Gummer, which demonstrate the typical "ruffling" of the hair shaft cuticle that occurs when the shaft is torn or pulled free from the inner root sheath.'' In the LAHS, even gentle pulling is sufficient to loosen the shaft and matrix from the normally adherent root sheaths. This appears to be the basic defect of LAHS: the normally tenacious bond between the lower hair shaft and the inner root sheath is pathologically weak, allowing trivial amounts of traction to separate the shaft from the sheath.' At present, the structural and/or biochemical abnormalities responsible for this weakness remain unexplained. Certainly the hair shafts in LAHS are often abnormal, given tbe ridging and fluting that has been found with scanning electron microscopy. It would be reasonable to suppose that a significant defect of the inner root sheath is also present. For example, aberrant keratinization could interfere with the normal interlocking of the inner root sheath cuticle and the hair cuticle and compromise the anchoring of a normal anagen hair.''^ Since the keratinized inner root sheath may affect the shape of the hair shaft, an abnormality of the inner root sheath could also explain the occasional fluting and ridging found in patients with LAHS.
Figure 2. Light microscopy of two loose anagen hairs with absent inner and outer root sheaths. as the latter grows out of the follicle. Only at the isthmus, where the inner root sheath disintegrates, does the hair shaft become free of the tenacious root sheath. Hairs that are forcibly plucked are usually removed with both inner and outer sheath intact. Occasionally the hair shaft is extracted leaving one or both root sheaths behind in the scalp. This is merely an artifact of forceful plucking.'' In specimens where the separation occurs between shaft and inner root sheath, the extracted hair will often show a "ruffling" of its cuticle.'' This is a natural consequence of the forceable separation of the inner root sheath cuticle and hair shaft cuticle, as described above. Clinical Findings
•
The loose anagen hair syndrome has been described in 24 children and five adults,'-^ and is characterized by a diffuse or patchy alopecia.^ Most patients are 2 to 5 years of age.' It is often associated with dry, lusterless hair. The hair can be easily and painlessly removed with a gentle pull using a thumb and forefinger. Often, the hair color is blond, and occasionally, the occipital hair is matted and feels sticky.' There have been no reports of associated abnormalities. The condition arises in early childhood and is described more often in girls.' It appears that with the onset of adolescence, the scalp hair grows progressively longer, denser, and darker in color; however, there is persistence of loose anagen hair.' Although most cases of LAHS have been isolated (nonfamilial), three families have been reported to have two affected members each.' Additional cases will require study to clarify the extent and pattern of inheritance.
Histologic Findings
'• -
Hamm and Traupe^ describe cleft formation between hair shafts and inner root sheaths, although this is found in normal follicles as an artifact of fixation and processing.'' Price and Gummer describe no abnormality of the hair follicles on multiple scalp biopsies.
CONCLUSIONS
Currently, there is no treatment available for Price and Gummer report that hair density and length increased in patients they followed for periods ranging from 2 to 18 years. Thus, patients and parents can be reassured that the more obvious clinical findings will at least partially resolve with time.
Extracted Hairs REEERENCES
The easily epilated hair shafts in LAHS are often irregularly shaped with longitudinal grooves and twisting along their axes,^ although not to the degree seen in
1.
108
•
Price VH, Gummer GL. Loose anagen syndrome. J Am Acad Dermatol 1989; 20:249-256.
Loose Anagen Hair Syndrome O'Donnell, Sperling, and James
Hamm H, Traupe H. Loose anagen hair of childhood: The phenomenon of easily pluckahle hair. J Am Acad Dermatol 1989; 20:242-248. Nodi F, Zaun H, Zinn KH. Gesteigerte Epilierbarkeit von Anagenhaaren bei Kindern als Folge eines Reifungsdefekts der Follikel mit gestorter Verhaftung von Haarschaft und Wurzelscheiden. Das Phanomen der leicht ausziehbaren Haare. Aktuel Dermatol 1986; 12:55-57.
Kligman AM. Pathologic dynamics of human hair loss. Arch Dermatol 1961; 83:175-198. Sperling LG, Samlaska GP. Hair anatomy as seen hy light microscopy. J Assoc Milit Dermatol 1989; 15: 16-28. Lever W, Schaumburg-Lever G. Histopathology of the skin. 7th ed. Philadelphia: JB Lippincott, 1990:27. Ebling F. Biology of hair follicles. In: Fitzpatrick TB, Eisen AZ, Wolff K, Freedberg I, Austen KF, eds. Dermatology in general medicine. New York: McGrawHill, 1987:213.
Maguire HG, Kligman AM. Hair plucking as a diagnostic tool. J Invest Dermatol 1964; 43:77-79.
Syphilis in the 16th Century The prevalence of syphilis in the upper classes of society and its liability to infect other members of the patient's family are well set forth in the colloquy between the soldier and the Carthusian monk in which the latter exclaims: "What spoils will you carry home to your Wife and Children? The Leprosy? for that Scab is only a Species of the Leprosy; and it is only not accounted so, because it is the Disease in Fashion, and especially among Noblemen: and for this very Reason, it should be the more carefully avoided. And now you will infect with it those that ought to be dearest to you of any in the World, and you yourself will all your days carry about a rotten carcase." The following extract from a letter (quoted by Jeanselme), though written by Erasmus in 1520, when the outbreak was beginning to abate, shows how deadly syphilis was at that time:— "If I were asked which is the most destructive of all diseases I should unhesitatingly reply, it is that which for some years has been raging with impunity. What contagion has ever extended so quickly to all the countries of Europe, Asia and Africa? What contagion does thus invade the whole body, so much resist medical art, becomes inoculated so readily, and so cruelly tortures the patient? It combines in itself all the terrible features of other contagions, pain, infection, danger of death, disagreeable and repugnant treatment and one which does not produce a complete cure." The manner of its acquirement, chronicity of the disease, severity of its course and inadequacy of treatment are also illustrated in the colloquy entitled "Epicurus": "Hedonius: When young men by whoring, as it commonly falls out, get the Pox, which by way of Extenuation they call the Neapolitan itch, by which they are often brought to death's door in their Life-Time and carry about a dead Carcase do they not Epicurise gloriously? "Spudceus: Yes, if coming often to the Powdering-Tub be doing so. "Hedonius: When once a man has got the Pox he's plagued with it all his LifeTime and forced to suffer a Sort of Death a great many Times over before the Time comestodie." . Perhaps the most interesting passage in Erasmus's writings dealing with syphilis is that contained in the colloquy entitled "The Unequal Marriage," which relates the marriage of Ulrich von Hutten under the name of Pompilius Blennus to a young and beautiful girl, although he was at that time suffering from a most hideous form of the disease. The colloquy contains advice regarding the use of separate beds, cups, linen and razors for the patients. As regards the behaviour of the luckless bride, it is recommended that she sbould clap her hands before her mouth whenever her husband offers to kiss her and should put on armour when she goes to bed with him. From Rolleston JD. Venereal disease in literature. Br J Venereal Dis 1934;X:147-182. 109
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LOOSE ANAGEN HAIR SYNDROME BRIAN PATRICK O'DONNELL, M.D., LEONARD C. SPERLING, M.D., AND WILLIAM D. JAMES, M.D.
Abstract
DISCUSSION
This recently described entity begins in childhood and is characterized by actively growing hairs that can be easily and painlessly removed from the scalp, leading to alopecia.
Normal Anatomy Before describing the findings seen in patients with LAHS, it would be useful to review some of the normal features of human scalp hair. The vast majority of hairs are anagen, or actively growing hairs. Most of the remainder are telogen, or "resting" hairs that are about to be shed. Tbe normal range for numbers of telogen hairs is 4 to 25%, with a mean of about 15%.^ Telogen counts above this range are presumptively abnormal.' A gentle hair pull performed on a normal scalp may sometimes yield several hairs, but these hairs will always be telogen hairs. The presence of anagen hairs on a gentle hair pull is clearly abnormal. To obtain anagen bairs from the normal scalp, the hair must be forcibly plucked, requiring an instrument such as a rubber-tipped hemostat. The reason that such force is required is due to special anatomic features of the follicle, which are reviewed in detail elsewhere.^ The free edges of the cuticular cells of the inner root sheath and hair shaft interlock with each other. The cells of the inner root sheath cuticle are flat and overlap like shingles on a roof. The free edges of these cells point downward and inward, interlocking with the hair shaft cuticular cells, which point upward and outward. This intimate association requires that the inner root sheath move upward with the hair shaft
The loose anagen hair syndrome (LAHS), described in two recent reports, features relatively short, sparse, uneven hair that is easily removed from the scalp.'-^ A 1986 report in the German literature may describe the same syndrome.^ We describe a patient with LAHS and report a new observation, the loss of both inner and outer root sheaths from extracted hairs. The etiology, clinical features, light microscopy, histopathology, prognosis, and treatment of this condition are also reviewed. Case Report A 3-year-old white boy was referred for evaluation of sparse, increasingly unmanageable scalp hair. The patient seldom required a hair cut because the hair did not grow sufficiently long. He was in good health and had no abnormalities of his skin, teeth, nails, or eyes. He was taking no medicines and his diet was normal. His mother noted the onset of the graying of her hair at the age of 10, but her hair is otherwise normal. The remainder of the family history was also unremarkable. Physical examination of the scalp revealed diffuse thinning of the hair. It was light brown and appeared dry and 'blow away," especially on the occipital region (Fig. 1). Locks of hair were short and uneven. There was no inflammation of the scalp, scarring, bald patches, or unevenness of hair color. The process did not involve the eyebrows, eyelashes, or nails. A gentle hair pull was performed, using the thumb and forefinger to grasp and slowly pull strands of 20 hairs. Multiple hair pulls yielded an average of five anagen hairs that were easily and painlessly removed. The hair pull specimens from our patient revealed anagen hairs with absent outer and inner root sheaths, and ruffling of the hair cuticle (Fig. 2).
From the Dermatology Service, Walter Reed Army Medical Genter, Washington, D.G. The opinions expressed are those of the authors and are not to be construed as official or as reflecting the views of the Department of the Army or the Department of Defense. Address for correspondence: Brian P. O'Donnell, M.D., Dermatology Service, Walter Reed Army Medical Genter, Washington, DG 20307-5001.
Figure 1. Difficult to manage, "blow away" hair is most evident over the occipital region. 107
International Journal of Dermatology Vol. 31, No. 2, February 1992
"uncombable hair." This feature is also absent in some patients.^ In our patient, light microscopy revealed only a few hairs which appeared grooved. All the extracted anagen hairs in our case were missing their outer and inner root sheaths. Price and Gummer' noted the absence of the outer root sheaths in their cases. Hamm and Traupe^ found that the inner root sheaths were missing. In fact both root sheaths are left behind in the dermis. This is confirmed by the photomicrographs supplied by Price and Gummer, which demonstrate the typical "ruffling" of the hair shaft cuticle that occurs when the shaft is torn or pulled free from the inner root sheath.'' In the LAHS, even gentle pulling is sufficient to loosen the shaft and matrix from the normally adherent root sheaths. This appears to be the basic defect of LAHS: the normally tenacious bond between the lower hair shaft and the inner root sheath is pathologically weak, allowing trivial amounts of traction to separate the shaft from the sheath.' At present, the structural and/or biochemical abnormalities responsible for this weakness remain unexplained. Certainly the hair shafts in LAHS are often abnormal, given tbe ridging and fluting that has been found with scanning electron microscopy. It would be reasonable to suppose that a significant defect of the inner root sheath is also present. For example, aberrant keratinization could interfere with the normal interlocking of the inner root sheath cuticle and the hair cuticle and compromise the anchoring of a normal anagen hair.''^ Since the keratinized inner root sheath may affect the shape of the hair shaft, an abnormality of the inner root sheath could also explain the occasional fluting and ridging found in patients with LAHS.
Figure 2. Light microscopy of two loose anagen hairs with absent inner and outer root sheaths. as the latter grows out of the follicle. Only at the isthmus, where the inner root sheath disintegrates, does the hair shaft become free of the tenacious root sheath. Hairs that are forcibly plucked are usually removed with both inner and outer sheath intact. Occasionally the hair shaft is extracted leaving one or both root sheaths behind in the scalp. This is merely an artifact of forceful plucking.'' In specimens where the separation occurs between shaft and inner root sheath, the extracted hair will often show a "ruffling" of its cuticle.'' This is a natural consequence of the forceable separation of the inner root sheath cuticle and hair shaft cuticle, as described above. Clinical Findings
•
The loose anagen hair syndrome has been described in 24 children and five adults,'-^ and is characterized by a diffuse or patchy alopecia.^ Most patients are 2 to 5 years of age.' It is often associated with dry, lusterless hair. The hair can be easily and painlessly removed with a gentle pull using a thumb and forefinger. Often, the hair color is blond, and occasionally, the occipital hair is matted and feels sticky.' There have been no reports of associated abnormalities. The condition arises in early childhood and is described more often in girls.' It appears that with the onset of adolescence, the scalp hair grows progressively longer, denser, and darker in color; however, there is persistence of loose anagen hair.' Although most cases of LAHS have been isolated (nonfamilial), three families have been reported to have two affected members each.' Additional cases will require study to clarify the extent and pattern of inheritance.
Histologic Findings
'• -
Hamm and Traupe^ describe cleft formation between hair shafts and inner root sheaths, although this is found in normal follicles as an artifact of fixation and processing.'' Price and Gummer describe no abnormality of the hair follicles on multiple scalp biopsies.
CONCLUSIONS
Currently, there is no treatment available for Price and Gummer report that hair density and length increased in patients they followed for periods ranging from 2 to 18 years. Thus, patients and parents can be reassured that the more obvious clinical findings will at least partially resolve with time.
Extracted Hairs REEERENCES
The easily epilated hair shafts in LAHS are often irregularly shaped with longitudinal grooves and twisting along their axes,^ although not to the degree seen in
1.
108
•
Price VH, Gummer GL. Loose anagen syndrome. J Am Acad Dermatol 1989; 20:249-256.
Loose Anagen Hair Syndrome O'Donnell, Sperling, and James
Hamm H, Traupe H. Loose anagen hair of childhood: The phenomenon of easily pluckahle hair. J Am Acad Dermatol 1989; 20:242-248. Nodi F, Zaun H, Zinn KH. Gesteigerte Epilierbarkeit von Anagenhaaren bei Kindern als Folge eines Reifungsdefekts der Follikel mit gestorter Verhaftung von Haarschaft und Wurzelscheiden. Das Phanomen der leicht ausziehbaren Haare. Aktuel Dermatol 1986; 12:55-57.
Kligman AM. Pathologic dynamics of human hair loss. Arch Dermatol 1961; 83:175-198. Sperling LG, Samlaska GP. Hair anatomy as seen hy light microscopy. J Assoc Milit Dermatol 1989; 15: 16-28. Lever W, Schaumburg-Lever G. Histopathology of the skin. 7th ed. Philadelphia: JB Lippincott, 1990:27. Ebling F. Biology of hair follicles. In: Fitzpatrick TB, Eisen AZ, Wolff K, Freedberg I, Austen KF, eds. Dermatology in general medicine. New York: McGrawHill, 1987:213.
Maguire HG, Kligman AM. Hair plucking as a diagnostic tool. J Invest Dermatol 1964; 43:77-79.
Syphilis in the 16th Century The prevalence of syphilis in the upper classes of society and its liability to infect other members of the patient's family are well set forth in the colloquy between the soldier and the Carthusian monk in which the latter exclaims: "What spoils will you carry home to your Wife and Children? The Leprosy? for that Scab is only a Species of the Leprosy; and it is only not accounted so, because it is the Disease in Fashion, and especially among Noblemen: and for this very Reason, it should be the more carefully avoided. And now you will infect with it those that ought to be dearest to you of any in the World, and you yourself will all your days carry about a rotten carcase." The following extract from a letter (quoted by Jeanselme), though written by Erasmus in 1520, when the outbreak was beginning to abate, shows how deadly syphilis was at that time:— "If I were asked which is the most destructive of all diseases I should unhesitatingly reply, it is that which for some years has been raging with impunity. What contagion has ever extended so quickly to all the countries of Europe, Asia and Africa? What contagion does thus invade the whole body, so much resist medical art, becomes inoculated so readily, and so cruelly tortures the patient? It combines in itself all the terrible features of other contagions, pain, infection, danger of death, disagreeable and repugnant treatment and one which does not produce a complete cure." The manner of its acquirement, chronicity of the disease, severity of its course and inadequacy of treatment are also illustrated in the colloquy entitled "Epicurus": "Hedonius: When young men by whoring, as it commonly falls out, get the Pox, which by way of Extenuation they call the Neapolitan itch, by which they are often brought to death's door in their Life-Time and carry about a dead Carcase do they not Epicurise gloriously? "Spudceus: Yes, if coming often to the Powdering-Tub be doing so. "Hedonius: When once a man has got the Pox he's plagued with it all his LifeTime and forced to suffer a Sort of Death a great many Times over before the Time comestodie." . Perhaps the most interesting passage in Erasmus's writings dealing with syphilis is that contained in the colloquy entitled "The Unequal Marriage," which relates the marriage of Ulrich von Hutten under the name of Pompilius Blennus to a young and beautiful girl, although he was at that time suffering from a most hideous form of the disease. The colloquy contains advice regarding the use of separate beds, cups, linen and razors for the patients. As regards the behaviour of the luckless bride, it is recommended that she sbould clap her hands before her mouth whenever her husband offers to kiss her and should put on armour when she goes to bed with him. From Rolleston JD. Venereal disease in literature. Br J Venereal Dis 1934;X:147-182. 109
