Loose anagen syndrome

Loose anagen hair syndrome is a disorder characterized by anagen hairs with abnormal morphology are ripped from the scalp easily and painlessly. Parul este subtire si nu creste dupa ceafa gitului. The hair is thin and does not grow on the head neck.
Pierderea difuza de par care apare in loose anagen syndrome ridica probleme cosmetice printre pacienti. Diffuse hair loss that occurs in loose anagen syndrome among patients pose cosmetic. Desi nu este disponibil inca nici un tratament conditia este doar semnificativa cosmetic, iar simptomele se amelioreaza in timp. Although there is still no treatment available is only significant cosmetic condition and the symptoms get better over time. Sunt afectati in deosebi copiii. Especially children are affected. Nu se observa modificari de tip inflamator ale scalpului. No apparent inflammatory changes of the scalp. Afectiunea este sporadica desi s-au inregistrat si cazuri familiale. The disease is sporadic and although there were family cases.

Patogenie si cauze Pathogenesis and causes
Patogeneza loose anagen syndrome nu este cunoscuta. Pathogenesis loose anagen syndrome is not known. Teaca radacinii parului joaca un rol important in ancorarea firului de par in folicul. Hair root sheath play an important role in anchoring the hair in the follicle. In acest sindrom mutatiile din gena care codeaza citokeratinele determina keratinizare anormala a radacinii interne. In this syndrome, mutations in the gene that encodes citokeratinele internal root cause abnormal keratinization. Aceasta keratinizare deficitara conduce la o aderenta alterata a tecii deformate a firului de par la folicul. This leads to poor adhesion keratinization altered deformed sheath of the hair follicle. Aceasta anomalie determina incetarea prematura a fazei de anagen si scurtarea lungimii parului. This anomaly lead to premature termination of anagen phase and shortening the length of hair.

Incidenta afectiunii este tipic sporadica dar s-au observat si cazuri familiale. The incidence of disease is typically sporadic but familial cases have been observed. Ereditatea pare a fi un model autosomal dominant cu penetranta variabila. Heredity appears to be an autosomal dominant pattern with variable penetrance. Loose anagen syndrome nu a fost asociat cu alte afectiun, dar unii indivizi prezinta urmatoarele conditii patologice: Loose anagen syndrome was not associated with other disease, but some individuals has the following pathological conditions:
-sindromul Noonan, sindromul tricoinofalangian Syndrome, Noonan syndrome tricoinofalangian
-sindromul unghie-patela, displazia ectodermica hipohidrotica Nail-patella-syndrome, ectodermal dysplasia hipohidrotica
-infectia HIV , alopecia areata , parul de lina. -Infection of HIV , alopecia areata , the hair smooth.

Semne si simptome Signs and symptoms
Pacientul clasic cu loose anagen syndrome este o fetita de 2-5 ani, cu parul blond . Loose anagen syndrome patient with classic 2-5 years is a little girl with blond hair. Pacientii raporteaza subtierea parului, iar tunsorile nu sunt necesare. Patients reported thinning hair and haircuts are not necessary. Tractiunea parului determina smulgerea acestuia, nedureroasa si usoara . Traction pulling his hair cause, painless and easy. Multi pacienti caracterizeaza parulu ca fiind fara luciu, uscat, imposibil de coafat . Many patients parulu characterized as dull, dry and impossible to style. Parintii sau fratii bolnavului pot prezenta aceiasi afectiune. Parents or siblings may be the same disease patient. Copii afectati sunt sanatosi in general, fara deficiente nutritionale sau alte boli. Affected children are generally healthy without nutritional deficiencies or other diseases. Dezvoltarea staturo-ponderala este normala. Staturo-weight development is normal.
Examenul fizic arata un par fin, scalp cu alopecie difuza sau in pete fara inflamatie sau cicatrizare . Physical examination showed a fine hair, scalp alopecia, diffuse or in patches without inflammation or scarring. Tractiunea gentila determina inlaturarea firelor de par fara durere. Parul nu este fragil sau sfarimicios . Gentle traction cause painless hair removal. Hair is fragile or sfarimicios. In mod particular parul de pe occiput pare grosolan si lipicios . In particular the hair on the occiput seems rough and sticky. Nu sunt afectate sprincenele sau genele. No eyebrows or eyelashes are affected. Alte structuri de origine ectodermica (dinti, piele, unghii) nu sunt afectate. Other structures of ectodermal origin (teeth, skin, nails) are not affected.

Diagnostic Diagnosis
Pentru a diagnostica afectiunea se efectueaza un test simplu in care se tractioneaza usor o bucla de par. To diagnose the disease is made a simple test in which a loop tractioneaza seem easy. La o persoana normala rezultatul va arata 0-2 fire de par smulse. The result will look like a normal person 0-2 hairs plucked. La pacientul cu loose anagen syndrome rezultatul arata mult mai multe fire smulse. The loose anagen syndrome patient with the result looks much more pulled threads. La examenul microscopic aceste fire sunt in faza anagen. On microscopic examination these threads are in anagen phase.
Examen histologic. Se observa formareade fisuri intre teaca parului si radacina interna. Histological examination. Formareade Note cracks between internal root sheath and hair. Modificarile egresive ale radacinii interne, incluzind omogenizarea si degenerarea pot fi observate. Egresive changes of internal root, including homogenization and degeneration can be observed. Parul poate demonstra configuratie anormala. Hair may show abnormal configuration. Foliculii din anagen prezinta keratinizare prematura a celulelor Henle si straturilor Huxley. Premature keratinization in anagen follicles shows the layers of Huxley and Henle cells. Nu se descriu infiltrate inflamatorii. No inflammatory infiltrates are described.
Diagnosticul diferential se face cu urmatoarele afectiuni: alopecia areata, lupus eritematos acut, discoid, pseudopelada, telogen effluvium, tricotilomania, tinea capitis, hipo- hipertiroidism , anemia feripriva . The differential diagnosis is made with the following conditions: alopecia areata, lupus erythematosus, acute discoid, pseudopelada, telogen effluvium, trichotilomania, tinea capitis, hypo- hyperthyroidism , iron deficiency anemia .

Tratament Treatment
Nu exista inca o terapie pentru acest sindrom. There is still a therapy for this syndrome. Procesul are doar semnificatie cosmetica si se amelioreaza cu inaintarea in virsta. The process has only cosmetic significance and improves with age.