short anagen syndrome in a girl with curly dark hair and consanguineous parents
TRANSCRIPT
Fig 1. Short anagen syndrome. Occipital scalp.
Fig 2. Short anagen syndrome: mild increased hairvolume after application of 2% topical minoxidil for 12months.
J AM ACAD DERMATOL
VOLUME 67, NUMBER 6Letters e279
Correspondence to:DrLucaStingeni,MD,Departmentof Medical and Surgical Specialities and PublicHealth, University of Perugia, Polo Ospedaliero-Universitario, Perugia, Italy 06132
E-mail: [email protected]
REFERENCES
1. Alikhan A, Lynch PJ, Eisen DB. Hidradenitis suppurativa: a
comprehensive review. J Am Acad Dermatol 2009;62:539-61.
2. Boer J, Nazary M. Long-term results of acitretin therapy for
hidradenitis suppurativa. Br J Dermatol 2011;164:170-5.
3. Haslund P, Lee RA, Jemec GBR. Treatment of hidradenitis
suppurativa with tumor necrosis factor-� inhibitors. Acta
Derm Venereol 2009;89:595-600.
4. Rose RF, Goodfield MJD, Clark SM. Treatment of recalcitrant
hidradenitis suppurativa with oral ciclosporin. Clin Exp Derma-
tol 2006;31:154-5.
5. Amor KT, Ryan C, Menter A. The use of cyclosporine in
dermatology: Part I. J Am Acad Dermatol 2010;63:925-46.
http://dx.doi.org/10.1016/j.jaad.2012.06.011
Short anagen syndrome in a girl with curlydark hair and consanguineous parents
To the Editor:A 3-year-old healthy Caucasian girl wasreferred for evaluation of short hair since birth (Fig1). She was the only child of consanguineous parents(cousins). The hair pull test, performed on severalareas of the scalp, produced positive results.Dermatoscopy showed normal hair density withnumerous vellus and intermediate hairs. A tricho-gram of approximately 50 parietal hair fibers re-vealed 31% telogen hairs. Tapered ends indicatedthat the hair had never been cut. Maximal hair lengthwas 4 cm. There was no evidence of hair breakage orhair shaft abnormalities.
Her mean hair growth rate was calculated byshaving an area of approximately 4 cm2 at the vertexof the scalp and measuring hair regrowth over theshaved area. The overall hair length was 2.5 cm after4 weeks. Based on the resultant mean hair growthrate of 0.26 mm/d, we concluded that the anagenphase was 5 months (normal range 2-6 years).
After application of 2% minoxidil twice dailyfor 12 months, a mild improvement was noted(Fig 2).
This case represents a rare congenital disordermanifesting as short scalp hair in an otherwisehealthy child. The main differential diagnoses in-cluded short anagen syndrome (SAS) and looseanagen syndrome (LAS). SAS was first described in1999 by Whitmore and Tausk.1 It is characterized bythe inability to grow long hair and an increasedproportion of telogen hairs.2 Most cases have oc-curred in Caucasians with fine blond hair. There is1 report of SAS in an African American woman.3
The presence of normal hair fibers with a shortanagen phase4 and increased number of telogenhairs on the trichogram are diagnostic. Previousreports showed anagen duration to range from 4 to13 months compared with the normal value of 2 to 6years.2,5
LAS was excluded as this diagnosis requires atleast 70% anagen hairs on the trichogram and notelogen hairs.3 The short hair in LAS may be causedby a defect in keratin genes responsible for optimaladhesion of the hair shafts to the follicle. Hairs areeasily extracted during the anagen phase. In mostchildren, LAS tends to improve with time.5
Although SAS is a benign condition, it can rarelybe associated with systemic diseases such as tricho-dental dysplasia,4 congenital hypotrichosis,2 linearscleroderma, and occluded lacrimal duct.1 Most
Fig 1. Left optic neuritis associated with tumor necrosisfactor-alfa antagonist use. Magnetic resonance imaging ofbrain and orbits ( patient 1). Coronal, short tau inversionrecovery image demonstrates increased signal in retrobul-bar portion of left optic affected nerve (white arrow).
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DECEMBER 2012e280 Letters
cases are sporadic. A familial case in which the hairgrowth improved with onset of puberty suggestedautosomal dominant inheritance.2
Recurrent episodes of hair shedding can occur asa result of synchronization of the hair cycle causedby the short anagen phase.5
Treatment options are limited. There was sponta-neous improvement after puberty in some reports,2
but not in others. Topical minoxidil can be used toprolong the anagen duration and hair growth,although its efficacy has not been well established.
To our knowledge, this is the first report of SAS ina girl with curly dark hair and consanguineousparents. SAS should be considered in children andyoung adults who report that their hair does notgrow long. Further research is needed to understandthe origin of this rarely reported condition.
Isabella Doche, MD,a Aline Donati, MD,a NeusaSakai Valente, MD, PhD,a Ricardo Romiti, MD,PhD,a and Maria K. Hordinsky, MDb
Departments of Dermatology at Hospital dasClinicas, University of S~ao Paulo, Brazil,a andUniversity of Minnesota, Minneapolisb
Funding sources: None.
Conflicts of interest: None declared.
Correspondence to: Isabella Doche, MD, RuaCubat~ao 929 cj 49, Vila Mariana, S~ao Paulo eSP, Brazil 04013-043
E-mail: [email protected]
REFERENCES
1. Whitmore SE, Tausk FA. Short anagen syndrome. J Clin
Dermatol 1999;2:30-2.
2. Barraud-Klenovsek MM, Tr€ueb RM. Congenital hypotrichosis
due to short anagen. Br J Dermatol 2000;143:612-7.
3. Avashia N, Woolery-Lloyd H, Tosti A. Short anagen syndrome in
an African American woman. J Am Acad Dermatol
2010;63:1092-3.
4. Kersey PJ. Tricho-dental syndrome: a disorder with a short hair
cycle. Br J Dermatol 1987;116:259-63.
5. Antaya RJ, Sideridou E, Olsen EA. Short anagen syndrome. J Am
Acad Dermatol 2005;53(Suppl):130-4.
http://dx.doi.org/10.1016/j.jaad.2012.06.012
Optic neuritis associated with tumor necrosisfactor-alfa antagonists for the treatment ofpsoriasis
To the Editor: Antietumor necrosis factor (TNF)-alfadrugs have shown striking efficacy in the treatmentof moderate to severe psoriasis1 and are widely usedin this indication. Optic neuritis is an acute dysim-mune demyelinating disorder of the optic nerve thatmay be the presenting sign of multiple sclerosis in
38% of patients.2 We report 4 cases of optic neuritisoccurring more than 1 year after initiation of adali-mumab (n ¼ 2) and etanercept (n ¼ 2) treatment forsevere psoriasis. Optic neuritis characteristics andantieTNF-alfa posologies and treatment durations inthese 4 patients are fully detailed in Table I.
All patients were receiving antieTNF-alfa forplaque psoriasis and had no concomitant medica-tion. They reported no personal or familial history ofdemyelinating disorder. Optic neuritis occurred, re-spectively, 14, 24, 12, and 16 months after onset oftreatment and presented as acute unilateral loss ofvisionwith variable homolateral eye painwith ocularmovements. Optic neuritis was confirmed by abnor-mal magnetic resonance imaging signal of the opticnerve in 2 patients (Fig 1) or by visually evokedpotential test result abnormalities in the 2 otherpatients. No other clinical or paraclinical signs ofmultiple sclerosis were found. Optic neuritis re-gressed spontaneously in 2 cases ( partially in1 case) and after high-dose intravenous methylpred-nisolone (1000 mg given intravenously on 3 consec-utive days) in 2 cases. In these 4 cases, the temporalassociation between antieTNF-alfa initiation andoptic neuritis onset, the lack of any other cause ofoptic neuritis, and the clinical improvement afterantieTNF-alfa withdrawal suggest a causal associa-tion. Other causes of optic neuritis, including infec-tion (eg, syphilis, Lyme disease), autoimmunedisorders (eg, lupus), sarcoidosis, drugs (eg, chlor-amphenicol, ethambutol), vasculitis, and diabetes,were ruled out in all patients. Of note, 3 patients hadreceived efalizumab, but for all of them this latterbiologic was stopped more than 4 years before thefirst symptoms, making very unlikely its imputabilityin the onset of optic neuritis.