Loose Anagen Hair Research Articles

A Girl with Loose Anagen Hair Syndrome and Uncombable, Spun‐glass Hair

A 4-year-old girl presented with a 2-year history of scalp hair that had an odd texture, was difficult to manage, tended to "stick out" from the scalp, and was irregular in length. A hair pull test revealed that hairs could be easily and painlessly extracted. Light microscopic examination of the hair demonstrated anagen hairs with a ruffled cuticle and distorted bulb as well as an unusual undulation and grooving of the shafts. These findings are consistent with both loose anagen and uncombable hair syndromes. The occurrence of both syndromes in the same patient seems unlikely, and we propose that our patient has loose anagen hair syndrome with features resembling uncombable hair syndrome.

Síndrome dos cabelos anágenos frouxos associada à distrofia macular: descrição de uma família

Descreve-se uma família com síndrome dos cabelos anágenos frouxos (SCAF) associada à distrofia macular. Foram descritos os achados dermatológicos, oftalmológicos e de microscopia óptica e eletrônica de varredura de 11 indivíduos. Dois apresentaram SCAF, sem qualquer alteração ocular. Dos quatro indivíduos com alteração ocular, duas irmãs apresentaram SCAF e dispersões pigmentares em pólo posterior da retina, com coloboma da mácula. Em dois irmãos encontraram-se as mesmas características da SCAF e fundo de olho com dispersões pigmentares em pólo posterior, sem coloboma. Os achados sugerem que essa associação seja uma nova entidade nosológica dentro das displasias ectodérmicas.

Hair diseases in childhood

This paper focuses on four important hair diseases mainly occurring in children. Trichotillomania is the most relevant differential diagnosis of alopecia areata in childhood. Meticulous inspection and lack of telogen hairs in the trichogram from the margin of the lesion usually are sufficient for differentiation. The trichogram also plays a significant role for the diagnosis of the loose anagen hair (loose anagen syndrome), a fairly new, but not rare entity, especially in distinguishing it from telogen effluvium. Five different types of clinical presentation are distinguished in tinea capitis. For the necessary systemic therapy; the new antimycotics terbinafine and itraconazole represent good alternatives to the well-tried griseofulvin. Several effective therapeutic options are also available for head lice, the most frequent parasitary infestation of school age. However, because of its neurotoxicity and the increasing problem of lice resistance lindane should not be used any longer for the treatment of head lice.

Is the Loose Anagen Hair Syndrome a Keratin Disorder?

To report the clinical features of the loose anagen hair syndrome and to test the hypothesis that the typical gap between the hair and the inner root sheath may result from hereditary defects in the inner root sheath or the apposed companion layer. Case series. A pediatric dermatology unit (referral center). A consecutive sample of 17 children (13 girls). For 9 of them and their first-degree relatives, molecular analyses were performed in the K6HF gene with 50 appropriate controls. Minoxidil therapy (5% lotion) in 11 patients for 1 to 12 months. Clinical and follow-up features and determination of mutations in the K6HF gene. Most patients had easily pluckable hair with no sign of scalp inflammation or scarring. Ten patients seldom cut their hair, and 4 had unmanageable hair. One patient had hypodontia. Two patients had an additional clinical phenotype of diffuse partial woolly hair. The family history was positive for loose anagen hair syndrome in 5 patients. Marked improvement was noted after treatment with 5% minoxidil lotion in 7 of the 11 patients treated. Polymerase chain reaction analysis of the gene segments encoding the alpha-helical 1A and 2B subdomains of K6hf, the type II cytokeratin exclusively expressed in the companion layer, was performed in 9 families. In 3 of these 9 families, a heterozygous glutamic acid and lysine mutation, E337K, was identified in the L2 linker region of K6HF. Diffuse partial woolly hair can be associated with loose anagen hair syndrome. A keratin mutation, E337K in K6HF, was possibly causative in 3 of the 9 families studied. Another keratin, and possibly the type I partner of K6hf, could be responsible for loose anagen hair syndrome in other patients, or the gene involved may be a minor gene.

Descrição de nova distrofia macular associada à síndrome dos cabelos anágenos frouxos

Purpose: To describe the ophthalmological, dermatological, light microscopy (LM) and scanning electron microscopy (SEM) findings of a new macular dystrophy associated with the loose anagen hair syndrome (LAHS). Methods: A family of seven patients, four of them affected, was examined. The patients underwent a complete ophthalmological examination, color test (Ishihara and Farnsworth D-15), ecography, angiography, laboratory and dermatological tests, sweat test, light microscopy and scanning electron microscopy of the hair. Results: In two affected sisters we found on eye fundoscopy pigmentary dispersions in the posterior pole of the retina, with macular staphyloma. In two brothers, the same pigmentary dispersions in the posterior pole was found, with more pigmentation and a yellow coloring in the macular area and without staphyloma. The light microscopy and scanning electron microscopy evaluation of the affected individuals confirmed loose anagen hair syndrome. In a not affected woman and man all examinations were normal, except the light microscopy and scanning electron microscopy that showed some similarities with the affected individuals. As for the way of inheritance, the pedigree is compatible with autosomal recessive inheritance with partial expression of the heterozygote. Conclusions: There is only one report in the international literature of loose anagen hair syndrome association with ocular coloboma. In this study we describe the findings of a new macular dystrophy associated with the loose anagen hair syndrome, a dystrophy whose fundoscopy findings are different between men and women. Since it is the first report in the literature, the described findings strongly suggest that this association can be part of a new nosological entity.

Síndrome del cabello anágeno suelto

Abstract.—We report a case of loose anagen hair in a nineyear-old girl. The disease was diagnosed at the age of four. Five years later the patient shows a notable improvement

Uncombable Hair (cheveux incoiffables, pili trianguli et canaliculi) Syndrome: Brief Review and Role of Scanning Electron Microscopy in Diagnosis

Uncombable hair syndrome was first described some 3 decades ago as "cheveux incoiffables" and is also known as spun-glass hair and pili trianguli et canaliculi. Both inherited (autosomal dominant and recessive with variable levels of penetrance) and sporadic forms of uncombable hair syndrome have been described, both being characterized by scalp hair that is impossible to comb due to the haphazard arrangement of the hair bundles. A characteristic morphologic feature of hair in this syndrome is a triangular to reniform to heart shape on cross-sections, and a groove, canal or flattening along the entire length of the hair in at least 50%of hairs examined by scanning electron microscopy. Most individuals are affected early in childhood and the hair takeson a spun-glassappearance with the hair becoming dry, curly, glossy, lighter in color, and progressively uncombable. Only the scalp hair is affected. Several conditions are associated with uncombable hair, such as ectodermal dysplasia, retinal dysplasia/ pigmentary dystrophy, juvenile cataract, digit abnormalities, tooth enamel anomalies, oligodontia, and phalangoepiphyseal dysplasia. Other syndromes with hair abnormalities may also mimic uncombable hair syndrome clinically and these include, Rapp-Hodgkin ectodermal dysplasia; loose anagen hair syndrome; ectodermal dysplasia, ectrodatyly, cleft lip/ palate (EEC) syndrome; and familial tricho-odonto-onchyial ectodermal dysplasia with syndactyly. Unlike other conditions with an uncombable hair component, uncombable hair syndrome alone (cheveux incoiffables, pili trianguli etcanaliculi) is not associated with physical, neurologic, or mental abnormalities. In most cases of uncombable hair syndrome, the hair is grossly abnormal in infancy and early childhood, but may have improved manageability later in life. Scanning electron microscopy of hair samples provides definitive evidence for diagnosis of clinically suspected uncombable hair syndrome and eliminates other hair abnormalities from the differential diagnosis.

Ｌｏｏｓｅ Ａｎａｇｅｎ Ｈａｉｒの１例

Ｌｏｏｓｅ Ａｎａｇｅｎ Ｈａｉｒの１例

The presence of loose anagen hairs obtained by hair pull in the normal population.

To help determine the specificity of "loose anagen" (LA) hairs in Loose Anagen Syndrome, the presence or absence of LA hairs on a gentle but firm hair pull was evaluated in 110 normal subjects from a 0.5 to 83 y old. In children < or =10 y old, 61% had LA hairs on hair pull evaluation and 73% of all hairs obtained were LA hairs. In contrast, LA hairs were found in only two of 87 (2%) normal postpubescent subjects. The number of LA hairs was small in normal children (1-2 per hair pull) and a maximum of one out of every 6-7 hair pulls in adults, far less than that reported with Loose Anagen Syndrome. Although the mere presence of LA hairs on a hair pull test is thus not specific for LAS in children, the number per hair pull may have diagnostic significance. Correlation of these findings with the various hair disorder phenotypes currently termed Loose Anagen Syndrome will be important.

Alopecia Areata with Features of Loose Anagen Hair

A child with alopecia areata (AA) presented initially with clinical features of loose anagen hair (LAH) and had features consistent with LAH on microscopic hair analysis. However, a scalp biopsy specimen demonstrated peribulbar inflammatory infiltrates characteristic of AA. We suggest that AA may mimic LAH clinically and a scalp biopsy may help in making the correct diagnosis.

Loose Anagen Hair

To review clinical and pathologic features and the long-term follow-up of patients with loose anagen hair (LAH). Clinical evaluation and long-term follow-up. A university medical center. Beginning in January 1990, 14 children and 5 adults (age range, 8 months to 47 years) were diagnosed as having LAH. Associated diseases included alopecia areata in a 3-year-old boy and Noonan syndrome in a 5-year-old boy. Two adult patients were parents of 2 affected children; the other 3 adults were the only members of their families with LAH. These 3 patients presented with a diffuse hair shedding that had suddenly developed 1 to 3 years before our observation. In all cases, findings of a trichogram showed a predominance of anagen hairs (80% to 100%) devoid of sheaths. None. In 4 children and 1 adult the condition remained stable; in 2 children and 1 adult, a considerable improvement in hair density was noticed. The pathologic study of hair from 5 patients did not reveal morphological abnormalities of the hair follicles except for a high incidence of fragmentations of the inner root sheath. Analysis of our patients with LAH reveals that the condition does not develop exclusively during childhood but can occasionally manifest itself later in life. The development of LAH may be sporadic, occur in association with developmental or acquired conditions, or, less commonly, be a familial disorder. While adult-onset LAH may not be exceptional, it can be easily misdiagnosed as telogen hair loss. The pathologic findings of LAH do not demonstrate any specific features and are of little value in the diagnosis of this condition.

Loose anagen hair

Loose anagen hair

LOOSE ANAGEN SYNDROME AND LOOSE ANAGEN HAIR

Loose anagen syndrome, or loose anagen hair, is a recently described condition of unknown etiology that may be under-recognized. The typical patient is a child with sparse fine hair that can easily be pulled out. The diagnosis is confirmed by microscopic examination of firmly pulled hairs, many of which are in the anagen phase but lacking an inner and outer root sheath and demonstrating a ruffled cuticle. Some presentations of alopecia areata may be confused with this condition, but the pull test analysis serves to differentiate them. A variety of theories have been postulated to explain the pathophysiology of loose anagen syndrome. In some cases, there is an autosomal dominant pattern of inheritance. In most cases, this condition spontaneously improves with age.

Loose Anagen Hair as a Cause of Hereditary Hair Loss in Children

The loose anagen hair syndrome is a recently described disorder with enhanced hair loss in which anagen hairs can be painlessly pulled from the scalp. In three families, we have observed an autosomal dominant form of inheritance. We have confirmed that the extracted bulbs consist only of cortical cells and that the hair shafts have an abnormal shape. Using routine light and electron microscopy, we observed disadhesion between all layers of the follicle and within some layers and premature keratinization of the inner root sheath of the follicle. In addition, abnormal maturation of the various layers was observed at the electron microscopic level, including regions of the cortex that contained decreased numbers of filaments. The disorder appears to be cyclic, since relatively normal and markedly affected regions of the shaft can be observed within a single follicle. Possible mechanisms responsible for the disease include abnormal intercellular signaling and disturbances of desmosomal components and/or cadherins.

Loose anagen hair as a cause of hereditary hair loss in children

Loose anagen hair as a cause of hereditary hair loss in children

Loose Anagen Hair in a Child with Noonan’s Syndrome

We report on a 4 1/2-year-old girl affected by loose anagen hair and Noonan's syndrome. The girl had short, blond, easily pluckable hair that had never been cut. The trichogram showed an absolute prevalence of abnormally shaped anagen bulbs lacking inner and outer root sheaths. A scalp biopsy evidenced a marked cleft formation between fragmented inner root sheaths and irregularly shaped hair shafts.

Loose anagen hair of childhood: The phenomenon of easily pluckable hair

We report two unrelated boys aged 4 and 9 years who had the recently delineated phenomenon of easily pluckable hair. Hair tufts could be pulled out without effort and without pain. Alopecia without any sign of scalp inflammation or scarring was noted. In the older child spontaneous remission occurred within 3 months, whereas the condition remained active in the younger boy for a follow-up period of 1 year. Trichograms from clinically involved and uninvolved areas disclosed a striking predominance of anagen hairs (98% to 100%) and complete absence of telogen hairs. A loss of the normal round or oval configuration of many hair shafts could be well visualized by the shrinking tube technique. Horizontal and transverse sections of scalp obtained for biopsy revealed marked cleft formations between hair shafts and regressively altered inner root sheaths. We suggest the name loose anagen hair of childhood for this newly described disease.