A Case of Pili Annulati Following Resolution of Alopecia Areata

Karan Lal, BS,* Charlotte Noorollah, DO**

*Medical Student, New York Institute of Technology College of Osteopathic Medicine, Old Westbury, NY **Dermatologist, Brooklyn Dermatology, Brooklyn, NY

Abstract Pili annulati (PA) is a primarily inherited condition that has been reported to occur concurrently with alopecia areata (AA) and following resolution of AA. PA represents a non-fragile disorder that becomes more evident in adulthood, allowing it to be missed in many patients. Microscopic examination of affected reveals a characteristic banded, alternating dark-and-light appearance. There is no formal treatment for PA, but proper hair care can prevent harm from external factors. Introduction Pili annulati (PA) is a rare, non-fragile hair disorder, often inherited in an autosomal- dominant fashion, though there are reports of sporadic cases. PA clinically presents as light spangled hair. Microscopically, it is characterized by alternating dark and light bands initiating from the hair sheath that are present in a random fashion along the length of the upper Figure 2. Microscopic examination revealing hair shaft with areas of alternating dark and light bands three quarters of the hair shaft.1,2 The condition with sharply cut edges. may be diagnosed in the beard, scalp, pubic 3-5 hair regrowth with only textural changes, and revealed any differences between PA and normal and axillary regions. The alternating dark and 1 triamcinolone therapy was discontinued (Figure hair specimens. However, antibodies to aspects light bands appear as such due to the presence 1). A hair sample was sent for pathological of the lamina densa, , and anchoring of air-filled pockets within the of the hair 6 examination to rule out a structural hair-shaft fibrils have been noted on immunohistochemical shaft at varying locations. Upon microscopic deformity. Examination of the hair shaft under studies, which is supported by transmission examination, these pockets scatter light and polarized light revealed alternating dark and light electron microscopy of PA hair specimens that appear dark due to the decreased transmission of bands with sharp-appearing edges indicative of a demonstrate reduplication of the lamina densa light; however, clinically they correspond to the 8 diagnosis of pili annulati (Figure 2). in the hair root bulb. Linkage analysis within light strands, because under reflective light the families with autosomal-dominant PA has also “microscopically dark” areas actually reflect more 6 been performed, revealing a gene locus on 12q light. PA has been reported concomitantly in with specific linkage between 12q24.32–24.33 patients with alopecia areata (AA) and following 9,10 regions. Although no known mutations have the resolution of AA. been isolated, having this specific locus provides an opportunity for further exploration. Case Report The diagnosis of PA is often made by light A 6-year-old male presented to the dermatology microscopy, with increased sensitivity when office with the primary complaint of “hair not a liquid medium is used to fix the hair.11 The growing the way it used to.” Hair loss had been differential diagnosis of PA includes pseudopili occurring in a non-discrete pattern for two annulati and monilethrix. months’ duration. The patient reportedly had very thick hair, and changes were particularly notable Pseudopili annulati, a twisted-hair phenotype, after a recent haircut prior to presenting to the characteristically appears in an elliptical shape office. The patient had a past medical history of with a normal hair shaft under light microscopy, hay fever. Past surgical and family histories were Figure 1. Dark brown hairs exhibiting but clinically it displays banding.12 Monilethrix non-contributory. alternating shiny and dull segments along the is an inherited, autosomal-dominant condition shafts with a speckled appearance. that has an alternating node and constricted On physical examination, the patient had appearance on microscopy, resembling PA, but round patches of non-scarring alopecia with unlike PA it is a fragile hair disorder.13,14 mild scaling. A fungal culture was performed for suspected tinea capitis, and the patient was Discussion PA has been documented to appear in conjunction prescribed 2% ketoconazole topical lotion to be Hair abnormalities are subtle and therefore with AA and post AA, and has also been applied daily, pending cultures. require extensive investigation. Pili annulati is reported to disappear after resolution of AA. One a rare, autosomal-dominant hair shaft disorder, European study examined the presence of PA Baseline laboratory examination revealed a discovered in 1866, that has an unknown concomitant with AA and found a statistically mildly elevated white blood cell count, while the 1,7 pathogenesis. Possible explanations for significant 9% prevalence of concurrent AA in remainder of the exam was within normal limits. development of PA include a structural protein the PA group.15 The study, however, suggested Fungal cultures did not reveal any growth, at which defect within the extra cellular matrix, a basement that the concurrence was coincidental, as the point ketoconazole therapy was discontinued membrane zone defect, a cytokeratin abnormality genes identified in AA do not appear to have and triamcinolone 0.1% cream with twice daily and a genetic defect on the long arm of the a relationship to the gene locus identified in application was initiated for suspected alopecia th 1,8,9 12 chromosome. Immunohistochemical PA. 15 However, the study size was too small to areata. Further follow-up at two months revealed examination for cytokeratin anomalies have not determine an actual relationship. Our patient, on Page 42 A CASE OF PILI ANNULATI FOLLOWING RESOLUTION OF ALOPECIA AREATA the other hand, had PA after resolution of AA. References 17. Smith SR, Kirkpatrick RC, Kerr JH, et al. A similar case has been reported by Cruz et al., 1. Giehl KA, Dean D, Dawber RP, et al. Alopecia areata in a patient with pili annulati. J in which a 31-year-old female with severe AA in Cytokeratin expression in pili annulati hair Am Acad Dermatol. 1995;32(5, pt 1):816-8. locations of the scalp as well as other hair-bearing follicles. Clin Exp Dermatol. 2005;30:426-8. 18. Castelli E, Fiorella S, Caputo V. Pili Annulati areas had resolution of her AA after being Coincident with Alopecia Areata, Autoimmune treated with several courses of intramuscular 2. Streck AP, Moncores M, Sarmento DF, et al. Thyroid Disease, and Primary IgA Deficiency: triamcinolone. Hair shaft examination of this Study of nanomechanical properties of human Case Report and Considerations on the patient’s regrown hair revealed PA.16 In contrast, hair shaft in a case of pili annulati by atomic Literature. Case Rep Dermatol. 2012;4(3):250-5. a case has been reported of a patient with a past force microscopy. J Eur Acad Dermatol Venereol. history of PA who presented with hair loss, and 2007;21:1109-10. after treatment with intralesional triamcinolone 3. Amichai B, Grunwald MH, Halevy S. Hair Correspondence: Charlotte Noorollah, DO; and topical minoxidil had re-growth of normal, abnormality present since childhood. Pili [email protected] non-PA hair.17 annulati. Arch Dermatol. 1996;132:575, 578. PA has also been identified in a patient with 4. Musso LA. Pili annulati. Australas J Dermatol. a complex condition of autoimmune disease 1970;11:67–75. comprised of IgA deficiency, thyroid disease, 5. Montgomery RM, Binder AI. Ringed hair. and AA. Although there may be no association Arch Dermatol Syphilol. 1970;58:177–9. between PA and AA, the presence of autoimmune diseases may relay some connection to antigenic 6. Moffitt DL, Lear JT, de Berker DA, Peachey changes within the hair root bulb and the RD. Pili Annulati Coincident with Alopecia polygenic nature of AA as well as the distinct Areata. Pediatr Dermatol.1998;15(4):271-3. locus of PA.18 7. Landois L. Das plötzliche Ergrauen Although there is no treatment for PA, patients der Haupthaare. Arch Path Anat Physio. with co-existing hair loss may benefit from topical 1866;35:575–99. minoxidil therapy, for it is thought to allow for 8. Giehl KA, Ferguson DJ, Dead D, et al. normal matrix production, which in turn could Alterations in the zone in theoretically repair the structural defect.17 pili annulati hair follicles as demonstrated by electron microscopy and immunohistochemistry. Conclusion Br J Dermatol. 2004;150:722-7. Pili annulati is a rare hair phenotype that has been 9. Green J, Fitzpatrick E, De Berker D, Forrest documented to occur with AA, after resolution of SM, Sinclair RD. A Gene for Pili Annulati Maps AA, and in combination with other autoimmune to the Telomeric Region of Chromosome 12q. J disorders. Due to its inheritable nature, a Invest Dermatol. 2004;123(6):1070-2. family history of PA may warrant examination and education of patients affected with this 10. Giehl KA, Eckstein GN, Benet-Pages A, Tosti condition. Although no treatment exists, patients A, De Berker DA, et al. A Gene Locus Responsible should be reassured and taught proper hair care for the Familial Hair Shaft Abnormality Pili management for this non-fragile condition. Annulati Maps to Chromosome 12q24.32-24.33. J Invest Dermatol. 2004;123(6):1073-7. 11. Giehl KA, Ferguson DJ, Dawber RP, et al. Update on detection, morphology and fragility in pili annulati in three kindreds. J Eur Acad Dermatol Venereol. 2004;18:654-8. 12. Lee SS, Lee YS, Giam YC. Pseudopili annulati in a dark-haired individual: A light and electron microscopic study. Pediatr Dermatol. 2001;18:27–30. 13. Bindurani S, Rajiv S. Monilethrix with Variable Expressivity. Int J Trichology. 2013;5(1):53-5. 14. Rakowska A, Slowinska M, Kowalska- Oledzka E, Rudnicka L. Trichoscopy in Genetic Hair Shaft Abnormalities. J Dermatol Case Rep. 2008;2(2):14-20. 15. Giehl K, Schmuth M, Tosti A, De Berker D, et al. Concomitant Manifestation of Pili Annulati and Alopecia Areata: Coincidental Rather than True Association. Acta Derm Venereol. 2011;91(4):459-62. 16. Cruz AP, Liang CA, Gray JP, Robinson- Bostom L, et al. The Appearance of Pili Annulati Following Alopecia Areata. . 2012;89(3):145-7.

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