- Email: [email protected]
Chronic telogen effluvium: A study of 5 patients over 7 years
Chronic telogen effluvium: A study of 5 patients over 7 years Rodney Sinclair, MBBS, FACD Melbourne, Australia Chronic telogen effluvium is said to be self-limiting in the long run; the natural history of this condition, however, has not been investigated prospectively. Four women, aged between 18 and 64 years and diagnosed with chronic telogen effluvium between 1996 and 1997, were followed up prospectively for a minimum of 7 years. One (previously reported) woman diagnosed in 1998 developed female pattern hair loss confirmed on biopsy specimen within 18 months that was partially reversed by spironolactone. The remaining 4 women continued to experience chronic diffuse telogen hair shedding that fluctuated in severity. However, serial photography demonstrated no visible reduction in hair density, and serial scalp biopsy specimen showed no follicular miniaturization. Although 4 out of 5 of our patients showed no tendency toward development of female pattern hair loss or to spontaneous improvement, further work is required to define the natural history of chronic telogen effluvium and the relative risk of developing female pattern hair loss. ( J Am Acad Dermatol 2005;52:S12-6.)
C
hronic telogen effluvium (CTE) was described as a primary idiopathic disease entity in 1996.1 Women present with an abrupt onset of generalized shedding of telogen hairs from the scalp, with or without an identifiable trigger, that persists more than 6 months. The hair may come out in handfuls. The shedding is frequently accompanied by bitemporal recession. Importantly there is no frontoparietal hair loss with widening of the central part and these women characteristically present with a full, thick head of hair. Important differential diagnoses include female pattern hair loss (FPHL), thyroid disease, drug-induced hair loss, systemic lupus erythematosus, and nutritional deficiency.2 FPHL is the preferred term for female androgenetic alopecia. CTE can be differentiated from early FPHL histologically using a horizontally sectioned 4-mm punch biopsy specimen taken from the vertex scalp. At the midisthmus level, a ratio of terminal to vellus hairs (T:V) of less than 4:1 is considered diagnostic of
This supplement is made possible through the generous support of Stiefel Laboratories for the American Academy of Dermatology. From the Department of Dermatology, University of Melbourne. Funding sources: None. Conflicts of interest: None identified. Reprint requests: Rodney Sinclair, MBBS, FACD, University of Melbourne Department of Dermatology, St Vincent’s Hospital, 41 Victoria Parade, Fitzroy, 3065, Melbourne, Victoria, Australia. E-mail: [email protected]. 0190-9622/$30.00 ª 2005 by the American Academy of Dermatology, Inc. doi:10.1016/j.jaad.2004.05.040
S12
FPHL, whereas a ratio greater than 8:1 indicates CTE.3 A single biopsy specimen may underestimate FPHL due to inadequate sampling.4 Multiple punch biopsy specimens from immediately adjacent skin on the scalp increases the diagnostic reliability from 79% for a single biopsy specimen to 98% when 3 biopsy specimens are taken.5 The natural history of CTE has not been studied prospectively; however, it has been suggested that the hair shedding is self-limiting and that women with this condition do not go bald.1 There is limited evidence to support the use of oral antiandrogen therapy in the management of FPHL.6,7 Scalp biopsy may, therefore, be useful to identify women most likely to respond to this treatment and to exclude women not likely to benefit.
CASE 1 A 54-year-old woman presented in 1995 with an 18-month history of continuous excessive hair shedding. The hair shedding began suddenly and she was able to collect between 40 and 150 hairs per day (Fig 1). She estimated the thickness of her ponytail had decreased by 50% during the preceding 18 months. Her father had died at the age of 40 but was Hamilton stage IV at the time of his death. There was no other family history of FPHL; however, her sister had experienced a single patch of alopecia areata that had regrown spontaneously. Her history included Hashimoto’s thyroiditis; she was, however, being treated with thyroxine and was euthyroid. She was postmenopausal and not on hormone replacement therapy.
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J AM ACAD DERMATOL VOLUME 52, NUMBER 2
Fig 1. Patient brought in a bag full of hair at her initial presentation. Each bundle corresponds to the amount she shed in 1 day.
On examination she had shoulder length hair, with moderate bitemporal recession. There was no widening of the central part, and she appeared to have a normal head of hair (Fig 2). There was a positive hair pull of telogen hairs over the entire scalp. Iron studies, serum zinc, thyroid function tests, free androgen index, and dihydroepiandostendione levels were all normal and antinuclear antibody was negative. As CTE had not been described, a presumptive diagnosis of FPHL was made. Cyproterone acetate (100 mg daily) for 10 days each month was started in combination with ethinyl estradiol (0.0625 mg) and reviewed at 6-month intervals. During the next 18 months there was no reduction in daily hair shedding, and in 1997 a scalp biopsy was performed to determine whether she had FPHL or CTE. The histology was consistent with a diagnosis of CTE (Table I). The cyproterone acetate and ethinyl estradiol were ceased and topical minoxidil was started. Twelve months later, in 1998, the hair shedding had continued unabated, although there was still no reduction in hair density over the midfrontal or vertex scalp. She was counting the number of hairs she shed each day and kept a diary. She reported losing 150 hairs per day and 400 hairs when she shampooed. The hair pull test was positive. The topical minoxidil was ceased and a repeated scalp biopsy specimen showed 32 follicles on horizontal section, of which none were miniaturized. Three were in telogen. Repeated blood tests all revealed normal findings. Twelve months later, in 1999, the hair shedding was continuing. The hair pull test was positive. Standardized scalp photography was performed with her head placed in a stereotactic device (Fig 3). A repeated biopsy specimen sectioned horizontally showed 52 follicles of which 7 were vellus. Two catagen/telogen hairs were seen. The T:V was 9:1.
Fig 2. Hair at initial presentation.
On review in 2000 and again in 2001, the hair shedding was continuing at a rate of between 50 and 150 hairs per day and the hair pull test was positive; however, serial scalp photography showed no discernible reduction in hair density over the midfrontal (Fig 3) or vertex scalp. To reduce the possibility of underestimating FPHL through limited sampling, in 2001 three 4-mm punch biopsy specimens were taken from her midfrontal scalp and all were sectioned horizontally. The T:Vs were 13:1, 8:1, and 6:1, which again was consistent with CTE. On review in 2002, in spite of continuing hair shedding, the photos showed no loss of hair volume, and a biopsy was not performed. At the most recent review in 2003, the hair shedding continues. The hair pull test remains positive. She appears to have a full head of hair. Scalp photographs show no reduction in hair density, however, the bitemporal recession is largely unchanged (Fig 4). Repeated biopsy specimens sectioned horizontally showed no evidence of FPHL almost 10 years after her initial presentation and after 8 years of formal clinical, photographic, and histologic follow-up.
CASE 2 A 64-year-old woman presented with a 6-month history of diffuse hair shedding. She had collected 450 hairs in a single day. The loss was increased with
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J AM ACAD DERMATOL FEBRUARY 2005
Fig 3. Case 1, standardized serial midfrontal scalp photography with head positioned in stereotactic device, from 1999 until 2003, and lateral photography to monitor bitemporal recession.
Table I. The ratio of terminal to vellus hair follicles on scalp biopsy specimen for patients 1 to 5 at various time intervals from baseline to year 7 Second biopsy Patient
Initial biopsy
1 2 3 4 5
‘ (34:0) 16.5:1 7.2:1 31:1 15:1
A
‘ (32:0) 9:1 8.2:1 14:1 1:1
B
Third biopsy C
A
9:1 13.3:1 8.2 8:1 2.6:1
B
Fourth biopsy C
16:1
24:1
5.2:1
6.5:1
Fifth biopsy
A
B
C
A
B
C
13:1 8.6:1 8:1 18:1
8:1 8.3:1 5.2:1 5.5:1
6:1 31:1 8.2:1 6:1
9:1
7:1
7:1
1.9:1
hair washing, and consequently she now only washed her hair once every 2 weeks. There was no family history of androgenetic alopecia. She had a history of hyperthyroidism treated with radioactive iodine. She subsequently developed hypothyroidism and has been on thyroxine replacement therapy for more than 25 years. Investigation by her referring doctor revealed low T4 and elevated thyrotropin. Her thyroxine dose was increased from 100 g to 150 g. At the time of presentation, she had been euthyroid for 4 months. Iron studies and hormone levels all revealed normal findings. Her only other medication was sinvastatin. Scalp biopsy specimen was consistent with a diagnosis of telogen effluvium (Table I). The sinvastatin was ceased and topical minoxidil (2%) was started. Six months later there had been no improvement and she continued to shed up to 400 hairs per day. The patient was reviewed regularly, and serial standardized photography was begun in 1999. On review, 4 years after
initial presentation, the hair shedding continued. Repeated biopsy specimen was consistent with CTE. On further review 8 years after initial diagnosis, she was still shedding 50 to 150 hairs per day, however, scalp photography (Fig 4) showed no loss of hair volume and scalp biopsy specimen showed no decrease in follicle T:V.
CASE 3 A 19-year-old woman presented with an 8-month history of chronic diffuse hair shedding, reduction in the volume of her ponytail, bitemporal recession, but no widening of her central part. The hair shedding had started suddenly; no precipitant event was identified on questioning. Thyroid function tests, iron studies, and hormone parameters all revealed normal findings. She took no medications. Scalp biopsy specimen was consistent with CTE (Table I). On review at 6 months, 12 months, 2 years, 4 years, and 8 years, the hair shedding had continued, but
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J AM ACAD DERMATOL VOLUME 52, NUMBER 2
Fig 4. Patients 2 to 5, standardized serial midfrontal scalp photography with head positioned in stereotactic device.
the magnitude fluctuated between 50 and 300 hairs per day. The shedding seemed worse in spring and autumn. Repeated biopsy specimen at 12 months, 24 months, and 8 years revealed no decrease in follicle T:V to suggest androgenetic alopecia, and serial scalp photography begun in 1999 showed no reduction in hair density (Fig 4).
seemed worse in spring and autumn. Repeated biopsy specimen at 12 months, 4 years, and 8 years revealed no decrease in the follicle T:V to suggest androgenetic alopecia. Serial scalp photography from 1999 onward showed no reduction in hair density (Fig 4).
CASE 5 CASE 4 A 27-year-old woman presented with a 4-year history of increased hair shedding, loss of volume of her ponytail, bitemporal recession, but no widening of her central part. No trigger for the hair shedding was identified on history. Thyroid function tests, iron studies, and hormone parameters all revealed normal findings. Scalp biopsy specimen was consistent with CTE (Table I). On review at 6 months, 12 months, 2 years, 4 years, and 8 years, the hair shedding had continued, but the magnitude fluctuated between 50 and 300 hairs per day. The shedding
This case has been previously reported.4 A 16year-old girl presented with a 12-month history of generalized hair shedding from the scalp. The onset of the shedding coincided with the development of Hashimoto’s thyroiditis and iron deficiency. At the time of initial presentation, the Hashimoto’s thyroiditis had been treated with neomercazole and she was euthyroid. The iron stores were still low with a ferritin of 13 g/L and because she was a vegetarian, oral iron replacement therapy was commenced. Hair shedding fluctuated in intensity during the course of the year. On follow-up 6 months later, her iron stores
S16 Sinclair
were normal (ferritin 36 g/L). However, the hair shedding continued. On examination there was a positive hair pull test from both the vertex of the scalp and the occipital scalp. There was mild bitemporal recession but no widening of the central part and she appeared to have a full, thick head of hair. Additional investigations at that time revealed normal thyroid function and negative antinuclear antibody and syphilis serology. She was on no medication other than the neomercazole. Serum testosterone, dihydroepiandosterone, and sex hormone binding globulin levels were all normal. As the hair shedding was continuing, two 4-mm punch biopsy specimens were taken from the vertex of the scalp, and one sectioned horizontally and the other vertically. The histology was consistent with CTE (Table I) with a T:V of 15:1. No treatment was recommended. At follow-up 12 months later, the hair loss has progressed with widening of the central part (Fig 4). Repeated blood tests showed normal iron studies, thyroid function, and hormone parameters. Three 4-mm punch biopsy specimens were taken from the vertex of the scalp and sectioned horizontally. The T:V was now 1:1, 2.6:1, and 1.9:1. A diagnosis of androgenetic alopecia was made and oral spironolactone (200 mg/d) was started. There was a substantial response to the spironolactone with reduction in hair shedding and a marked increase in hair density.
DISCUSSION Although most women who present with chronic diffuse telogen hair shedding with no visible reduction in scalp hair density will be found on biopsy specimen to have FPHL, approximately 40% will have normal scalp biopsy specimen and be diagnosed with CTE.5 The pathogenesis of CTE is unknown, but it is theorized that CTE is caused by a reduction in the duration of the anagen growth phase without hair follicle miniaturization.1,8 A reduction in the duration of anagen also occurs in FPHL, and it has been argued that CTE is merely the earliest stages of FPHL,9 in an analogous way to acquired progressive kinking.10 Long-term follow-up in 4 of these 5 patients identified no evidence of evolution into FPHL. This suggests that CTE is a distinct clinical and histologic entity, with a natural history different from that of FPHL, although even longer follow-up will be required to confirm this. With respect to the fifth patient, the very rapid clinical transition makes it likely that a sampling error
J AM ACAD DERMATOL FEBRUARY 2005
failed to detect miniaturization and led to misdiagnosis of CTE. We subsequently demonstrated that biopsy specimens from immediately adjacent areas can show sufficient variation in T:V ratio to cause misdiagnosis and that diagnostic accuracy is improved by taking 3 punch biopsy specimens side by side.4 There is limited evidence to suggest that oral antiandrogens such as spironolactone or cyproterone acetate can be used to treat FPHL.6,7 In contrast, there is no evidence to support the use of antiandrogens in CTE. Therefore, in women who present with increased hair shedding but no visible reduction in scalp hair density, horizontally sectioned scalp biopsy specimen is indicated to distinguish CTE from FPHL. As the population prevalence of FPHL among women in their 60s is at least 25%,11 some women with CTE will develop FPHL coincidentally. Further studies are required to estimate the relative risk of developing FPHL among women with CTE. The authors would like to acknowledge Dr Jill Magee from Dorovitch Mayne pathology for taking the photomicrography, and Ms Rebecca Davies for taking the clinical photographs.
REFERENCES 1. Whiting DA. Chronic telogen effluvium: increased scalp hair shedding in middle-aged women. J Am Acad Dermatol 1996; 35:899-906. 2. Sinclair RD. Diffuse hair loss. Int J Dermatol 1999;38:8-18. 3. Whiting DA. Scalp biopsy as a diagnostic and prognostic tool in androgenetic alopecia. Dermatol Ther 1998;8:24-33. 4. Sinclair R. Chronic telogen effluvium or early androgenetic alopecia? Int J Dermatol 2004;43:842-3. 5. Sinclair R, Jolley D, Mallari R, Magee J. The reliability of horizontally sectioned scalp biopsies in the diagnosis of chronic diffuse telogen hair loss in women. J Am Acad Dermatol 2004;51:189-99. 6. Sinclair R, Wewerinke M, Jolley D. Treatment of female pattern hair loss with oral antiandrogens. Br J Dermatol (in press). 7. Harrison S, Sinclair R. Telogen effluvium. Clin Exp Dermatol 2002;27:389-95. 8. Dawber RPR, Simpson NB, Barth JH. Diffuse alopecia: endocrine, metabolic, and chemical influences on the follicular cycle. In: Dawber RPR, editor. Diseases of the hair and scalp. Oxford: Blackwell Science; 1997. p. 123-50. 9. Tosti A, Piraccini BM, Pazzaglia M, Misciali C. Acquired progressive kinking of the hair: clinical features, pathological study, and follow-up of 7 patients. Arch Dermatol 1999;135: 1223-6. 10. Sinclair R, Thai KE. Androgenetic alopecia. In: Lebwohl M, Heymann W, Berth-Jones J, Coulson I, editors. Treatment of dermatological disease. London: Harcourt Health Sciences; 2002. p. 35-7. 11. Norwood OT. Incidence of female androgenetic alopecia (female pattern alopecia). Dermatol Surg 2001;27:53-4.
C
hronic telogen effluvium (CTE) was described as a primary idiopathic disease entity in 1996.1 Women present with an abrupt onset of generalized shedding of telogen hairs from the scalp, with or without an identifiable trigger, that persists more than 6 months. The hair may come out in handfuls. The shedding is frequently accompanied by bitemporal recession. Importantly there is no frontoparietal hair loss with widening of the central part and these women characteristically present with a full, thick head of hair. Important differential diagnoses include female pattern hair loss (FPHL), thyroid disease, drug-induced hair loss, systemic lupus erythematosus, and nutritional deficiency.2 FPHL is the preferred term for female androgenetic alopecia. CTE can be differentiated from early FPHL histologically using a horizontally sectioned 4-mm punch biopsy specimen taken from the vertex scalp. At the midisthmus level, a ratio of terminal to vellus hairs (T:V) of less than 4:1 is considered diagnostic of
This supplement is made possible through the generous support of Stiefel Laboratories for the American Academy of Dermatology. From the Department of Dermatology, University of Melbourne. Funding sources: None. Conflicts of interest: None identified. Reprint requests: Rodney Sinclair, MBBS, FACD, University of Melbourne Department of Dermatology, St Vincent’s Hospital, 41 Victoria Parade, Fitzroy, 3065, Melbourne, Victoria, Australia. E-mail: [email protected]. 0190-9622/$30.00 ª 2005 by the American Academy of Dermatology, Inc. doi:10.1016/j.jaad.2004.05.040
S12
FPHL, whereas a ratio greater than 8:1 indicates CTE.3 A single biopsy specimen may underestimate FPHL due to inadequate sampling.4 Multiple punch biopsy specimens from immediately adjacent skin on the scalp increases the diagnostic reliability from 79% for a single biopsy specimen to 98% when 3 biopsy specimens are taken.5 The natural history of CTE has not been studied prospectively; however, it has been suggested that the hair shedding is self-limiting and that women with this condition do not go bald.1 There is limited evidence to support the use of oral antiandrogen therapy in the management of FPHL.6,7 Scalp biopsy may, therefore, be useful to identify women most likely to respond to this treatment and to exclude women not likely to benefit.
CASE 1 A 54-year-old woman presented in 1995 with an 18-month history of continuous excessive hair shedding. The hair shedding began suddenly and she was able to collect between 40 and 150 hairs per day (Fig 1). She estimated the thickness of her ponytail had decreased by 50% during the preceding 18 months. Her father had died at the age of 40 but was Hamilton stage IV at the time of his death. There was no other family history of FPHL; however, her sister had experienced a single patch of alopecia areata that had regrown spontaneously. Her history included Hashimoto’s thyroiditis; she was, however, being treated with thyroxine and was euthyroid. She was postmenopausal and not on hormone replacement therapy.
Sinclair S13
J AM ACAD DERMATOL VOLUME 52, NUMBER 2
Fig 1. Patient brought in a bag full of hair at her initial presentation. Each bundle corresponds to the amount she shed in 1 day.
On examination she had shoulder length hair, with moderate bitemporal recession. There was no widening of the central part, and she appeared to have a normal head of hair (Fig 2). There was a positive hair pull of telogen hairs over the entire scalp. Iron studies, serum zinc, thyroid function tests, free androgen index, and dihydroepiandostendione levels were all normal and antinuclear antibody was negative. As CTE had not been described, a presumptive diagnosis of FPHL was made. Cyproterone acetate (100 mg daily) for 10 days each month was started in combination with ethinyl estradiol (0.0625 mg) and reviewed at 6-month intervals. During the next 18 months there was no reduction in daily hair shedding, and in 1997 a scalp biopsy was performed to determine whether she had FPHL or CTE. The histology was consistent with a diagnosis of CTE (Table I). The cyproterone acetate and ethinyl estradiol were ceased and topical minoxidil was started. Twelve months later, in 1998, the hair shedding had continued unabated, although there was still no reduction in hair density over the midfrontal or vertex scalp. She was counting the number of hairs she shed each day and kept a diary. She reported losing 150 hairs per day and 400 hairs when she shampooed. The hair pull test was positive. The topical minoxidil was ceased and a repeated scalp biopsy specimen showed 32 follicles on horizontal section, of which none were miniaturized. Three were in telogen. Repeated blood tests all revealed normal findings. Twelve months later, in 1999, the hair shedding was continuing. The hair pull test was positive. Standardized scalp photography was performed with her head placed in a stereotactic device (Fig 3). A repeated biopsy specimen sectioned horizontally showed 52 follicles of which 7 were vellus. Two catagen/telogen hairs were seen. The T:V was 9:1.
Fig 2. Hair at initial presentation.
On review in 2000 and again in 2001, the hair shedding was continuing at a rate of between 50 and 150 hairs per day and the hair pull test was positive; however, serial scalp photography showed no discernible reduction in hair density over the midfrontal (Fig 3) or vertex scalp. To reduce the possibility of underestimating FPHL through limited sampling, in 2001 three 4-mm punch biopsy specimens were taken from her midfrontal scalp and all were sectioned horizontally. The T:Vs were 13:1, 8:1, and 6:1, which again was consistent with CTE. On review in 2002, in spite of continuing hair shedding, the photos showed no loss of hair volume, and a biopsy was not performed. At the most recent review in 2003, the hair shedding continues. The hair pull test remains positive. She appears to have a full head of hair. Scalp photographs show no reduction in hair density, however, the bitemporal recession is largely unchanged (Fig 4). Repeated biopsy specimens sectioned horizontally showed no evidence of FPHL almost 10 years after her initial presentation and after 8 years of formal clinical, photographic, and histologic follow-up.
CASE 2 A 64-year-old woman presented with a 6-month history of diffuse hair shedding. She had collected 450 hairs in a single day. The loss was increased with
S14 Sinclair
J AM ACAD DERMATOL FEBRUARY 2005
Fig 3. Case 1, standardized serial midfrontal scalp photography with head positioned in stereotactic device, from 1999 until 2003, and lateral photography to monitor bitemporal recession.
Table I. The ratio of terminal to vellus hair follicles on scalp biopsy specimen for patients 1 to 5 at various time intervals from baseline to year 7 Second biopsy Patient
Initial biopsy
1 2 3 4 5
‘ (34:0) 16.5:1 7.2:1 31:1 15:1
A
‘ (32:0) 9:1 8.2:1 14:1 1:1
B
Third biopsy C
A
9:1 13.3:1 8.2 8:1 2.6:1
B
Fourth biopsy C
16:1
24:1
5.2:1
6.5:1
Fifth biopsy
A
B
C
A
B
C
13:1 8.6:1 8:1 18:1
8:1 8.3:1 5.2:1 5.5:1
6:1 31:1 8.2:1 6:1
9:1
7:1
7:1
1.9:1
hair washing, and consequently she now only washed her hair once every 2 weeks. There was no family history of androgenetic alopecia. She had a history of hyperthyroidism treated with radioactive iodine. She subsequently developed hypothyroidism and has been on thyroxine replacement therapy for more than 25 years. Investigation by her referring doctor revealed low T4 and elevated thyrotropin. Her thyroxine dose was increased from 100 g to 150 g. At the time of presentation, she had been euthyroid for 4 months. Iron studies and hormone levels all revealed normal findings. Her only other medication was sinvastatin. Scalp biopsy specimen was consistent with a diagnosis of telogen effluvium (Table I). The sinvastatin was ceased and topical minoxidil (2%) was started. Six months later there had been no improvement and she continued to shed up to 400 hairs per day. The patient was reviewed regularly, and serial standardized photography was begun in 1999. On review, 4 years after
initial presentation, the hair shedding continued. Repeated biopsy specimen was consistent with CTE. On further review 8 years after initial diagnosis, she was still shedding 50 to 150 hairs per day, however, scalp photography (Fig 4) showed no loss of hair volume and scalp biopsy specimen showed no decrease in follicle T:V.
CASE 3 A 19-year-old woman presented with an 8-month history of chronic diffuse hair shedding, reduction in the volume of her ponytail, bitemporal recession, but no widening of her central part. The hair shedding had started suddenly; no precipitant event was identified on questioning. Thyroid function tests, iron studies, and hormone parameters all revealed normal findings. She took no medications. Scalp biopsy specimen was consistent with CTE (Table I). On review at 6 months, 12 months, 2 years, 4 years, and 8 years, the hair shedding had continued, but
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J AM ACAD DERMATOL VOLUME 52, NUMBER 2
Fig 4. Patients 2 to 5, standardized serial midfrontal scalp photography with head positioned in stereotactic device.
the magnitude fluctuated between 50 and 300 hairs per day. The shedding seemed worse in spring and autumn. Repeated biopsy specimen at 12 months, 24 months, and 8 years revealed no decrease in follicle T:V to suggest androgenetic alopecia, and serial scalp photography begun in 1999 showed no reduction in hair density (Fig 4).
seemed worse in spring and autumn. Repeated biopsy specimen at 12 months, 4 years, and 8 years revealed no decrease in the follicle T:V to suggest androgenetic alopecia. Serial scalp photography from 1999 onward showed no reduction in hair density (Fig 4).
CASE 5 CASE 4 A 27-year-old woman presented with a 4-year history of increased hair shedding, loss of volume of her ponytail, bitemporal recession, but no widening of her central part. No trigger for the hair shedding was identified on history. Thyroid function tests, iron studies, and hormone parameters all revealed normal findings. Scalp biopsy specimen was consistent with CTE (Table I). On review at 6 months, 12 months, 2 years, 4 years, and 8 years, the hair shedding had continued, but the magnitude fluctuated between 50 and 300 hairs per day. The shedding
This case has been previously reported.4 A 16year-old girl presented with a 12-month history of generalized hair shedding from the scalp. The onset of the shedding coincided with the development of Hashimoto’s thyroiditis and iron deficiency. At the time of initial presentation, the Hashimoto’s thyroiditis had been treated with neomercazole and she was euthyroid. The iron stores were still low with a ferritin of 13 g/L and because she was a vegetarian, oral iron replacement therapy was commenced. Hair shedding fluctuated in intensity during the course of the year. On follow-up 6 months later, her iron stores
S16 Sinclair
were normal (ferritin 36 g/L). However, the hair shedding continued. On examination there was a positive hair pull test from both the vertex of the scalp and the occipital scalp. There was mild bitemporal recession but no widening of the central part and she appeared to have a full, thick head of hair. Additional investigations at that time revealed normal thyroid function and negative antinuclear antibody and syphilis serology. She was on no medication other than the neomercazole. Serum testosterone, dihydroepiandosterone, and sex hormone binding globulin levels were all normal. As the hair shedding was continuing, two 4-mm punch biopsy specimens were taken from the vertex of the scalp, and one sectioned horizontally and the other vertically. The histology was consistent with CTE (Table I) with a T:V of 15:1. No treatment was recommended. At follow-up 12 months later, the hair loss has progressed with widening of the central part (Fig 4). Repeated blood tests showed normal iron studies, thyroid function, and hormone parameters. Three 4-mm punch biopsy specimens were taken from the vertex of the scalp and sectioned horizontally. The T:V was now 1:1, 2.6:1, and 1.9:1. A diagnosis of androgenetic alopecia was made and oral spironolactone (200 mg/d) was started. There was a substantial response to the spironolactone with reduction in hair shedding and a marked increase in hair density.
DISCUSSION Although most women who present with chronic diffuse telogen hair shedding with no visible reduction in scalp hair density will be found on biopsy specimen to have FPHL, approximately 40% will have normal scalp biopsy specimen and be diagnosed with CTE.5 The pathogenesis of CTE is unknown, but it is theorized that CTE is caused by a reduction in the duration of the anagen growth phase without hair follicle miniaturization.1,8 A reduction in the duration of anagen also occurs in FPHL, and it has been argued that CTE is merely the earliest stages of FPHL,9 in an analogous way to acquired progressive kinking.10 Long-term follow-up in 4 of these 5 patients identified no evidence of evolution into FPHL. This suggests that CTE is a distinct clinical and histologic entity, with a natural history different from that of FPHL, although even longer follow-up will be required to confirm this. With respect to the fifth patient, the very rapid clinical transition makes it likely that a sampling error
J AM ACAD DERMATOL FEBRUARY 2005
failed to detect miniaturization and led to misdiagnosis of CTE. We subsequently demonstrated that biopsy specimens from immediately adjacent areas can show sufficient variation in T:V ratio to cause misdiagnosis and that diagnostic accuracy is improved by taking 3 punch biopsy specimens side by side.4 There is limited evidence to suggest that oral antiandrogens such as spironolactone or cyproterone acetate can be used to treat FPHL.6,7 In contrast, there is no evidence to support the use of antiandrogens in CTE. Therefore, in women who present with increased hair shedding but no visible reduction in scalp hair density, horizontally sectioned scalp biopsy specimen is indicated to distinguish CTE from FPHL. As the population prevalence of FPHL among women in their 60s is at least 25%,11 some women with CTE will develop FPHL coincidentally. Further studies are required to estimate the relative risk of developing FPHL among women with CTE. The authors would like to acknowledge Dr Jill Magee from Dorovitch Mayne pathology for taking the photomicrography, and Ms Rebecca Davies for taking the clinical photographs.
REFERENCES 1. Whiting DA. Chronic telogen effluvium: increased scalp hair shedding in middle-aged women. J Am Acad Dermatol 1996; 35:899-906. 2. Sinclair RD. Diffuse hair loss. Int J Dermatol 1999;38:8-18. 3. Whiting DA. Scalp biopsy as a diagnostic and prognostic tool in androgenetic alopecia. Dermatol Ther 1998;8:24-33. 4. Sinclair R. Chronic telogen effluvium or early androgenetic alopecia? Int J Dermatol 2004;43:842-3. 5. Sinclair R, Jolley D, Mallari R, Magee J. The reliability of horizontally sectioned scalp biopsies in the diagnosis of chronic diffuse telogen hair loss in women. J Am Acad Dermatol 2004;51:189-99. 6. Sinclair R, Wewerinke M, Jolley D. Treatment of female pattern hair loss with oral antiandrogens. Br J Dermatol (in press). 7. Harrison S, Sinclair R. Telogen effluvium. Clin Exp Dermatol 2002;27:389-95. 8. Dawber RPR, Simpson NB, Barth JH. Diffuse alopecia: endocrine, metabolic, and chemical influences on the follicular cycle. In: Dawber RPR, editor. Diseases of the hair and scalp. Oxford: Blackwell Science; 1997. p. 123-50. 9. Tosti A, Piraccini BM, Pazzaglia M, Misciali C. Acquired progressive kinking of the hair: clinical features, pathological study, and follow-up of 7 patients. Arch Dermatol 1999;135: 1223-6. 10. Sinclair R, Thai KE. Androgenetic alopecia. In: Lebwohl M, Heymann W, Berth-Jones J, Coulson I, editors. Treatment of dermatological disease. London: Harcourt Health Sciences; 2002. p. 35-7. 11. Norwood OT. Incidence of female androgenetic alopecia (female pattern alopecia). Dermatol Surg 2001;27:53-4.