Pili torti (singular pilus tortus; also known as "twisted hairs") is characterized by short and brittle hairs that appear flattened and twisted when viewed through a microscope.[1]: 638 [2]: 764 [3]
The diagnosis of pili torti is made via trichoscopic and microscopic inspection. Pili torti does not have any specific treatment.
Signs and symptoms
Clinically, the hair of individuals with pili torti is dry, coarse, brittle, and fragile. It could lead to patchy alopecia.[4] The most common area affected is the scalp, particularly the occipital and temporal regions. But the eyelashes, pubic hair, axilla, and eyebrows could also be affected.[5] Typically, pili torti just impact a portion of the hair and do not alter the entire length of the hair.[6] Occasionally, isolated pili torti might be discovered in a typical scalp. But it could be connected to a lot of regional and systemic issues.[7]
Causes
Pili torti can be aqquired or inherited.[4] There are three types of inherited pili torti: classic early onset (Ronchese type), late onset (Beare type), and pili torti linked to hereditary illnesses or disorders.[8]
Starting in early life, the classic (Ronchese) type is an autosomal dominant or recessive disease. The third month to the third year of life is when the sickness first manifests. Blond girls are more frequently impacted. Hair twist clusters are typically seen in early-onset pili torti. With age, the disease frequently gets better, especially after puberty.[8][9]
Usually manifesting after puberty, late onset type is an autosomal dominant condition. People with dark hair seem to experience it more frequently. The hair twists in the late onset form are typically solitary, in contrast to the early onset kind.[8][9]
Uneven growth of the outer root sheath cells is the source of hair twisting in hereditary forms.[4] The hair shaft and inner root sheath are unevenly molded as a result of cell vacuolation and unequal outer root sheath thickness at the suprabulbar region.[74] In acquired forms, the hair follicle is deformed and rotational forces are generated by a perifollicular inflammation followed by fibrosis.[75]
Diagnosis
Examination under a microscope and trichoscope is used to diagnose pili torti. Low magnification trichoscopy in pili torti reveals the hair shafts bent at random intervals and at varied angles. At high magnification, the hair shaft's regular twists along its long axis are visible.[4] Upon microscopic inspection, the shaft exhibits irregularly spaced clusters of three or four twists at random intervals.[6]
Classification
Pili torti can be aqquired or inherited.[4] There are three types of inherited pili torti: classic early onset (Ronchese type), late onset (Beare type), and pili torti linked to hereditary illnesses or disorders.[8] Acquired pili torti may result from medication side effects or be linked to a variety of systemic and dermatological disorders.[4]
Treatment
Pili torti does not have a specific treatment. It is advised to keep the hair from suffering damage. Additional techniques of care include dyeing, braiding, heat treatments, avoiding over-grooming, and sleeping on a satin pillowcase.[4] Shampoos that are gentle might be helpful.[76][69]
After puberty, congenital pili torti may heal on their own.[4] Drug-induced instances usually go away as the offending substance is stopped.[70][71] The most crucial aspect of treating acquired pili torti is treating the underlying ailment.[4]
The use of pharmaceuticals to treat pili torti has minimal efficacy.[22] Topical minoxidil has been proposed as a good therapeutic alternative for individuals with higher fragility in their hair shaft abnormalities. Nevertheless, it does not cause a causative treatment; rather, it solely affects hair density.[4]
History
In 1932, Ronchese and Galewsky separately published the first descriptions of pili torti, also referred to as "twisted hair."[77][8]
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^Yung, A.; Newton-Bishop, J.A. (2005-08-05). "A case of Bazex-Dupré-Christol syndrome associated with multiple genital trichoepitheliomas". British Journal of Dermatology. 153 (3). Oxford University Press (OUP): 682–684. doi:10.1111/j.1365-2133.2005.06819.x. ISSN0007-0963. PMID16120174.
^Richards, Kristen A.; Mancini, Anthony J. (2002). "Three members of a family with pili torti and sensorineural hearing loss: The Bjornstad syndrome". Journal of the American Academy of Dermatology. 46 (2). Elsevier BV: 301–303. doi:10.1067/mjd.2002.107969. ISSN0190-9622. PMID11807445.
^PETIT, A.; DONTENWILLE, M.M.; BLANCHET BARDON, C.; CIVATTE, J. (1993). "Pili torti with congenital deafness (Bjornstad's syndrome)- report of three cases in one family, suggesting autosomal dominant transmission". Clinical and Experimental Dermatology. 18 (1). Oxford University Press (OUP): 94–95. doi:10.1111/j.1365-2230.1993.tb00983.x. ISSN0307-6938. PMID8440069.
^Patel, Harish P.; Unis, Mark E. (1985). "Pili torti in association with citrullinemia". Journal of the American Academy of Dermatology. 12 (1). Elsevier BV: 203–206. doi:10.1016/s0190-9622(85)80018-9. ISSN0190-9622. PMID3973120.
^Silengo, Margherita; Valenzise, Mariella; Pagliardini, Severo; Spada, Marco (2003). "Hair changes in congenital disorders of glycosylation (CDG type 1)". European Journal of Pediatrics. 162 (2). Springer Science and Business Media LLC: 114–115. doi:10.1007/s00431-002-1054-1. ISSN0340-6199. PMID12607543.
^Crandall, Barbara F.; Samec, Lawrence; Sparkes, Robert S.; Wright, Stanley W. (1973). "A familial syndrome of deafness, alopecia, and hypogonadism". The Journal of Pediatrics. 82 (3). Elsevier BV: 461–465. doi:10.1016/s0022-3476(73)80121-0. ISSN0022-3476. PMID4698933.
^Rybojad, M; Moraillon, I; Bonafé, J L; Cambon, L; Evrard, P (December 1998). "Pilar dysplasia: an early marker of giant axonal neuropathy". Annales de dermatologie et de venereologie (in French). 125 (12): 892–893. PMID9922862.
^Lurie, R.; Ben-Amitai, D.; Laron, Z. (2004). "Laron Syndrome (Primary Growth Hormone Insensitivity): A Unique Model to Explore the Effect of Insulin-Like Growth Factor 1 Deficiency on Human Hair". Dermatology. 208 (4). S. Karger AG: 314–318. doi:10.1159/000077839. ISSN1018-8665. PMID15178913.
^Spiegl, B; Hundeiker, M (November 1979). "Congenital hereditary hypotrychosis. Generalized autosomal dominant hypotrichosis with pili torti (hypotrichosis congenita hereditaria Marie Unna)". Fortschritte der Medizin (in German). 97 (44): 2018–2022. PMID511082.
^Pierini, A M; Ortonne, J P; Floret, D (1981). "Cutaneous manifestations of McCune–Albright syndrome: report of a case". Annales de dermatologie et de venereologie (in French). 108 (12): 969–976. PMID7337370.
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^Pietrzak, Aldona; Bartosinska, Joanna; Filip, Agata A.; Rakowska, Adriana; Adamczyk, Michal; Szumilo, Justyna; Kanitakis, Jean (2015-03-21). "Steatocystoma multiplex with hair shaft abnormalities". The Journal of Dermatology. 42 (5). Wiley: 521–523. doi:10.1111/1346-8138.12837. ISSN0385-2407. PMID25808203.
^Tay, Chong Hai (1971-07-01). "Ichthyosiform Erythroderma, Hair Shaft Abnormalities, and Mental and Growth Retardation: A New Recessive Disorder". Archives of Dermatology. 104 (1): 4–13. doi:10.1001/archderm.1971.04000190006002. ISSN0003-987X. PMID5120162.
^Itin, Peter H. (2014-04-08). "Etiology and pathogenesis of ectodermal dysplasias". American Journal of Medical Genetics Part A. 164 (10). Wiley: 2472–2477. doi:10.1002/ajmg.a.36550. ISSN1552-4825. PMID24715647.
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^Abramovits-Ackerman, William; Bustos, Tania; Simosa-Leon, Venancio; Fernandez, Luis; Ramella, Marcos (1992). "Cutaneous findings in a new syndrome of autosomal recessive ectodermal dysplasia with corkscrew hairs". Journal of the American Academy of Dermatology. 27 (6). Elsevier BV: 917–921. doi:10.1016/0190-9622(92)70287-p. ISSN0190-9622. PMID1479096.
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^Gómez-Quispe, Heidy; Elena de las Heras-Alonso, María; Lobato-Berezo, Alejandro; Velasco-Tamariz, Virginia; Pindado-Ortega, Cristina; Moreno-Arrones, Oscar Muñoz; Vañó-Galván, Sergio; Saceda-Corralo, David (2021). "Trichoscopic findings of discoid lupus erythematosus alopecia: A cross-sectional study". Journal of the American Academy of Dermatology. 84 (3). Elsevier BV: 804–806. doi:10.1016/j.jaad.2020.05.144. ISSN0190-9622. PMID32502590.
^ abGold, S.C.; Delaney, T.J. (1974). "(11) Familial acne conglobata, hidradenitis suppurativa, pili torti and cataracts*". British Journal of Dermatology. 91 (s10): 54–57. doi:10.1111/j.1365-2133.1974.tb12514.x. ISSN0007-0963.
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^ abKremer, Noa; Martinez, Helena; Leshem, Yael Anne; Hodak, Emmilia; Zer, Alona; Brenner, Baruch; Amitay-Laish, Iris (2021). "The trichoscopic features of hair shaft anomalies induced by epidermal growth factor receptor inhibitors: A case series". Journal of the American Academy of Dermatology. 85 (5). Elsevier BV: 1178–1184. doi:10.1016/j.jaad.2020.03.055. ISSN0190-9622. PMID32244022.