Loose anagen syndrome

The main pathological cause of loose anagen syndrome is the absence or insufficient amount of the inner root sheath in the anagen staged hairs. This creates a gap in the linking between the inner root sheath and the hair cuticles. Both of these hair features stimulate hair growth but with this detachment in the linking, loose anagen hairs are not able to grow to a long length. These mutations account for the phenotypical features of loose anagen syndrome. In the Huxley cells of the inner root sheath of abnormal anagen hairs, there is the formation of vacuoles and the build-up of fluid which is not usually seen. The inner root sheath of normal anagen hairs usually do not have keratin in the Huxley cells, Henle cells and inner root sheath. They are usually organised in an orderly manner and densely compacted. Genetics is one of the causing factors of loose anagen syndrome. It has been studied that this hair condition is autosomal dominantly inherited. == Diagnosis ==

Patients with loose anagen syndrome do not report the same hair abnormalities. This may be the result of the deformations of the hair shaft. These changes in texture limits the ability for adjacent loose anagen hairs to rest flat on the scalp. The patient can have thinning and loss of hair around the whole scalp, at the occipital scalp or around the scalp in odd, irregular patches. Loose anagen syndrome hairs at the occipital scalp can be knotted easily when there's movement of those hairs against the pillow at night. There are 3 different types of loose anagen hair syndrome. Loose anagen syndrome type A is when the hair of the patient appears to be fine and thinly distributed. Patients with type A loose anagen syndrome experience a decrease in the density of their hairs. Patients diagnosed with type B loose anagen hair syndrome tend to have hair that is difficult to manage and has a disordered, unruly appearance. Type C loose anagen syndrome is when the hair sheds but the appearance of their hair appears to be normal. Loose anagen syndrome type A and B are more prevalent in children. Loose anagen hair syndrome type C is most frequently seen in adults. There is not specific treatment for each type of loose anagen syndrome. It is also unknown as to how each type is developed. Differential diagnosis Loose Anagen Syndrome can commonly be misdiagnosed for other skin and hair disorders such as short anagen syndrome, alopecia areata, telogen effluvium, trichotillomania and toxic ingestion. Similar symptoms and signs appear in these conditions, however there are different aspects that distinguish each one from another. Short anagen syndrome and loose anagen syndrome are similar as both conditions lead to the inability to grow long hair and patients rarely need to get their hair cut. A difference between these two hair disorders is that in loose anagen syndrome there are dystrophic hairs which are absent in patients with short anagen syndrome. Patients with loose anagen syndrome may experience the thinning of their hair which is also a factor of alopecia areata. In order to distinguish between these two conditions, if dots, hairs with tapered roots or vellus hairs are visible in a microscopic examination of the skin this will lead to the diagnosis of alopecia areta rather than loose anagen syndrome. Hair loss can lead to other symptoms and signs such as weight loss, behavioural changes, short temper, abdominal pain and problems with learning. These signs are also observed in children with toxic ingestion which is one of the differential diagnosis. A pull test is also used in the diagnosis of telogen effluvium. The hairs that are removed in a telogen effluvium pull test are telogen staged hairs instead of being at the anagen stage as seen in loose anagen hair syndrome. Associated conditions Loose anagen syndrome usually appears as an individual condition. Loose anagen syndrome can be present with other conditions related to genetics or developmental conditions. Examples of these conditions include; Coloboma, Noonan syndrome, Hypohidrotic ectodermal dysplasia, EEC (ectrodactyly- ectodermal dysplasia-clefting) syndrome, Neurofibromatosis, Trichorhinophalangeal syndrome, Trichotillomania, Nail-patella syndrome and Woolly hair. Loose anagen hair syndrome has also been associated with AIDS. Alopecia areata patients can also develop loose anagen syndrome. == Treatment ==

chemical structure Loose anagen syndrome usually does not need treatment as this hair condition tends to improve with age and is self-limited. Many patients affected by loose anagen hair syndrome encounter spontaneous recovery by adulthood. The thickness and length of the hair of loose anagen syndrome patients have a tendency to improve and becomes more enhanced, especially post puberty. For some patients, improvement is observed from the ages of 8 or 9. There is still a defect in the connection of the hair shaft and inner root sheath, however it is not as severe and the hairs are not as easily detached as it was for these patients when they were young children. Patients can seek assistance and treatment can be pursued. The aims of treatment is to reduce the trauma being enacted on loose anagen hairs and to reassure loose anagen hair syndrome patients that over time there will be improvement in the cosmetics of their hair. Paediatricians, paediatric nurse practitioners and physician assistances are usually the first people to detect this hair condition in children. There are no surgical, physical or systemic medical treatment options for loose anagen syndrome. Patients with more severe cases of loose anagen hair syndrome can be treated with minoxidil, a topical medical treatment, as a first line treatment. Minoxidil can extend the duration of the hair in the anagen stage and restore the hair follicles for further growth or for the full period of the anagen phase. The hair of an individual without loose anagen hair syndrome is in the anagen hair phase for approximately 3 to 5 years. Minoxidil can be used as it enriches the cells of the hair by heightening the blood supply of local cutaneous. From this, there is intensified DNA synthesis occurring in both follicular keratinocytes and non-follicular, as well as an increase in cell multiplication. The degree of hair shedding is reduced with the use of minoxidil. 7 in 11 loose anagen hair syndrome patients find visible improvement after using minoxidil lotion. Treatment involving the maintenance of nutrients within the patient and biotin have not made any noted impacts to the improvement of loose anagen hair syndrome. Management For children, changes to their lifestyle and daily routine are altered and improved to reduce trauma to hair. This includes modifications in hair maintenance such as different washing, styling and combing techniques are adapted, as an attempt to minimise trauma to loose anagen syndrome hairs. Young girls are also recommended to avoid activities such as extreme braiding. Caring for hair gently will minimise the amount of loosely anchored hairs being removed from scalp. == History ==

The first published report on loose anagen syndrome was made in 1986 by Nodl and then by Zaun. Loose anagen syndrome was originally referred to as ‘syndrome of loosely attached hair in childhood’. The name ‘loose anagen syndrome’ was first adapted in 1989 by Price, Gummer, Hamm and Traupe in American literature. Early literature on loose anagen syndrome all reported identical findings. The genetic aspects and potential mechanisms for the cause of loose anagen syndrome was first discovered in 1992 by Baden, Kvedar and Magro. The ultrastructural changes in loose anagen hairs were examined by Mirmirani, Price and Uno. == See also ==