به نام خداوند جان و خرد Hair shaft abnormalitiesclues to diagnosis treatment. Presented by Dr . Anahita Vali Dermatologist داستان زال و رودابه بدان پرورا نيدم اين تار را كه تا دستگيري كند يار را تو اين را دروغ و فسانه مدان استحكام عجيب موها يك رشته مو توانائي بلند كردن وزنه 100گرمي را دارد بدون آنكه پاره و گسسته شود و حتي ميتوان ريسمان محكمي از موها بافت. What is the similarities between hair & plants ? The commonest causes of hair fragility and breakage are cosmetic. Repeated physical injury from overzealous grooming, Avoid/Avoid/Avoid backcombing, braiding and traction and long exposure to heat and ultraviolet light can cause weathering and damage to the hair. Chemical injury from perm lotions, straighteners and hair dyes can cause similar changes. Rare inherited conditions like monilethrix, pili torti, trichorrhexis nodosa, and trichothiodystrophy and trichorrhexis invaginata cause brittle hair and obvious hair loss from breakage. Gentle hair care should be the prime concern. Avoid شكل ظاهري مو متاثر از پيوندهاي شيميائي داخل آن است پيوندهاي دي سولفيدي پيوندهاي هيدروژني قوي ترو مستحكم تر تنها راه تغئير آن ها مواد شيميائي تعداد كمتر ضعيف تر تعداد بيشتر مسئول انعطاف پذيري مو ها درزمان خيس شدن مو باندهاي هيدروژني به دفعات شكسته ميشوند و در زمان خشك شدن آن دوباره شكل ميگيرند Hair shaft abnormalities are characterized by changes in color, density, length and structure. Hair dysplasias dry and looks lusterless. easily broken, slow-growing, and seldom required cutting. Office diagnosis of hair shaft abnormalities Good clinical history emphasizes other skin appendages such as the nails, sweat and sebaceous glands. 2. Careful examination of the hair and scalp 3. Evaluation of affected hair shafts under , the light microscope . important note :To take representive hairs From of the scalp . 1. Different areas 4.Trichoscopy Good Idea Examine the Proximal 1-2 cm of the affected shaft adjacent to scalp to exclude the effects of Weathering . Microscopic examination With normal transmitted light or with polarized light. Hairs can be examined dry between two glass slide . Better and under higher magnification with a mounting medium The hairs placed side by side on slide without overlap under the coverslip . The electron microscope (scanning or transmission) Before making the diagnosis of a Hair shaft abnormality , it is Important to have a through Knowledge of the Variation in normal hairs . It should be remembered that normal hairs differ considerably in length , diameter , cross section , color and Cuticular pattern even in the Same patient . Normal hair variations 1) 2) 3) 4) Straight Wavy Curly ( helical ) Woolly or peppercorn ( spiral ) سلسله بالست دام حلقه دوست موي هركه در اين حلقه نيست فارغ از اين ماجراست NO biochemical differences Have been detected among the Various forms of human hair . Physical Properties of hairs are derived from the central portion of the hair shaft , the Cortex , which comprises 90% of the hair shaft . Variations among races wide structural variability even in normal hair – Asiatic hair : visually straight – Caucasian hair : may be straight , wavy or curly . – Negroid hair : woolly or peppercorn . Hair shaft Defects A. B. Inherited eg . Trichorrhexis invaginata Acquired eg . Trichoptilosis Trichoscopy Dermoscopy is a method based on epiluminescence microscopy, which is used to visualize living skin in magnification.Videodermoscopy of hair and scalp (trichoscopy) is a new method, which allows viewing hair shafts in vivo in many-fold magnification without the need of pulling hair for diagnostic purposes of examination Trichoscopy in genetic hair shaft abnormalities 20-fold and 70-fold magnification were analysed. In selected cases 160-fold magnification was used for better visualization of hair shafts. Several reports indicate the usefulness of trichoscopy in diagnosing hair and scalp disorders, including androgenic alopecia, alopecia areata or lipedematous alopecia. Conclusion Genetic hair shaft abnormalities may be diagnosed by trichoscopy in a single diagnostic session without the need of plucking or cutting them for diagnostic purposes. Trichoscopy of hair shafts demonstrated a “crawling snake” appearance, with short wave cycles under 20× magnification We liken this appearance to the head of the mythical character Medusa, a character in Greek mythology, whose scalp hair was made of living snakes. Apart from that, the examination demonstrated broken hair shafts. Evenly pigmented, coarsetextured, and extremely curly scalp hair crawling snake” appearance Hair abnormalities are extremely common in genoder-matoses. Hair changes may be a significant finding or even the initial presentation of a syndrome giving the clue to the diagnosis, in diseases such as trichothiodystrophy, Netherton syndrome, or Woolly hair syndrome. Most hair shaft abnormalities Can be seen sporadically in normal hair so need to be found Consistently in different areas of the Scalp to be of Diagnostic importance Hair Shaft Defects in Children Children may be born with a good head of hair, or relatively little hair, or no hair at all. At birth, any of these may be normal events, but at 12 months of age, the average child will have diffuse scalp hair growth and by 2 years should have normal scalp hair. If funnylooking hair is present, or if scalp hair is totally absent in a child aged 1 years old, then that should cause concern. Hair shaft defects in children may only involve certain areas such as the nape of the neck and the temples, or may affect much or all of the scalp hair. The Netherton syndrome is characterized by three major clinical features: ichthyosiform dermatosis (nonbullous ichthyosiform erythroderma or as ichthyosis linearis circumflexa), atopy and a characteristic hair abnormality. Clinically hair appears sparse, dull, brittle and short. The hair shaft abnormality, called bamboo hair or trichorhexis invaginata is microscopically characterized by an invagination of the distal portion of the hair shaft into its proximal portion forming a "ball in cup" appearance and is considered pathognomonic for Netherton syndrome. B. ● ● ● ● ● Without fragility , have no functional significance . Pili annulati (ringed hair ) Woolly hair Woolly hair nevus Uncombable hair syndrome (cheveux incoiffables ) Straight hair nevus Hair shaft Defects A. ● ● ● ● ● With fragility causing patchy hair loss Monilethrix Pseudomonilethrix Pilitorti Bamboo hair ( nethertons syndrome ) Trichorrhexis nodosa Weathering ● Sun ● Wind ● Swimming ● Washing ● Friction ● Excessive hair styling Trichoclasis Trichoclasis refers to the common “greenstick” fracture of the hair shaft, consisting of a transverse fracture splinted by an intact cuticle. There is no constant abnormality of the cuticle or cortex in this condition. Trichoclasis can be found in different hair shaft abnormalities or simply following excessive hair care and trauma. Usually when physical trauma accounts for trichoclasis, the affected area shows sporadic and patchy damage, which can be reversed with gentle hair care. Pili Multigemini and Pili Bifurcati In pili multigemini, hairs are formed from 2 to 8 hair papillae and matrices. The hair shafts are surrounded by a common outer root sheath but each hair has its own inner root sheath, arising from one follicular canal. Given this tight arrangement, emerging hairs may look flattened, triangular, or ovoid with possible longitudinal grooves. In adults, pili multigemini is usually found in the beard region, sometimes in a linear distribution. In children, the scalp is typically the affected area. Pili bifurcati refers to hair shafts that emerge from a single papilla but then split into two shafts with their own distinct cuticles which can then rejoin as one shaft, even more than one time. It represents a restricted from of pili multigemini and occurs on the scalp rather than the beard. آسيبهاي اكتسابي ساقه مو فركردن يا صاف كردن موي سر رنگ كردن يا رنگ بري موي سر پوش دادن يا شانه كردن برعكس استفاده نامناسب از اتو مويا سشوار آفتاب گرفتن استفاده زياد از استخر دفترچه خاطرات هفت ساله نرم كننده هاي مو يا حالت دهنده ها پانتنول نرم كننده هاي جديد مشتقي از ويتامين ب مولكولهاي بزرگ داراي بار الكتريكي مثبت به داخل تار مو جذب وباعث رطوبت دهي آن ميشود مو خود اندكي بار منفي دارد چسبيدن مولكولها به لبه فلسهاي به داخل پوست سر نيز نفوذ مي كند آسيب ديده كوتيكول و باعث رطوبت رساني فوليكولها در نتيجه قابليت آراستن و براق نيز ميشود بودن مو افزايش مي يابد. در نتيجه رطوبت مناسبي را در زمان رويش مو در اختيار آن مي گذارد PHموي انسان در حدود 6است نقطه ايزوالكتريك موئي كه هيچ تغئيري در آن ايجاد نشده 3/67است در اين پي اچ تارهاي مو تمايلي به انتقال بار الكتريكي ندارند اثرات كلر بر روي موي سر حتي در حضور مقادير بسيار كم كلر 1PPM كلر باعث تخريب پروتئين در سطح اپي كوتيكول ميشود بكار گيري شامپو در زمان استفاده از آب حاوي كلر آسيب را كاهش مي دهد With light microscopy hair shaft abnormalities can be divided into four Categories , these includes : 1) Fractures of the hair shaft 2) Irregularities of the hair shaft 3) Hair shaft coiling and twisting 4) Exogenous matter on the hairshaft Fractures of the hair shaft Transverse ( Trichoschesis , Trichorrhexis nodosa ) Oblique ( Tapered fractured , eg anagen effluvium ) Longitudinal (Trichoptilosis ) Acquired trichorrexis nodosa (more common ) ● ● ● ● ● ● ● ● ● Trauma Excessive weathering Back combing Stressed hair styles Head rolling and banging Habit tic Trichotillomania Scratching Perming and dyeing of hairs Congenital trichorrhexis nodosa ● ● ● 1. 2. 3. 4. Rare May occur as an isolated entity May occurs with a metabolic abnormality such as Arginosuccinic aciduria Citrullinemia Menkes disease Trichothiodystrophy .( hair sulfur <50% normal) Heat is a great enemy to hair . (the dryer must always be used with great care and at a moderate setting ) هيچ درماني براي موهاي آسيب ديده از حرارت زياد وجود ندارد بكار گيري منظم،مرتب و درست از حالت دهنده ها پوشش كامل مو بصورت بتونه كردن ميتواند باعث كاهش بروز مو خوره شود Pili torti regular twists of the hair shaft along the long axis Hair Shaft Coiling and Twisting- Pili Torti Pili torti refers to the hair shaft that is twisted on its own axis leading to irregularly spaced beads from the twists, making the hair brittle and break easily. Twisted hairs can be seen normally on a scalp, particularly the pubic and axillary hair are normally twisted. Pili torti is usually a congenital condition but can be acquired as well. Children with neural hearing loss have been seen with pili torti which would lead the investigator to insist on early auditory testing. Bjornstad’s syndrome is a rare condition characterized by hearing loss and pili torti. Hypogonadism can be present (Crandall’s syndrome) along with mental retardation. As mentioned, Menkes’ kinky hair syndrome can have pili torti, trichorrhexis nodosa, trichoclasis, and trichoptilosis, making the hair feel like steel wool. A unique form of pili torti is known as corkscrew hair in which many hairs are twisted in a double spiral, which can occur associated with widely spaced teeth and joined fingers and toes (syndactyly). Monilethrix autosomal dominant hair disorder periodic thinning of hair shafts /tendency to fracture at constricted points. clinically in hair fragility and patchy dystrophic alopecia Follicular abnormalities seen in monilethrix range from subtle perifollicular erythema and hyperkeratosis to horny follicular papule formation. [Mutations in hair specific keratins, especially hHb1 and hHb6, which are primarily expressed in cortical trichocytes, cause predominantly structure abnormalities of hair cortex. pili annulati showed a random pattern of intermittent abnormal cavities .Light bands observed by clinical examination appeared as dark bands when imaged by light microscopy, reflecting cortical spacers containing air in the light bands and fluid in the dark bands. Trichothiodystrophy Light microscopy showed hair shafts with an irregular, undulating contour and transverse fractures through the hair shaft (trichoschisis). Polarized light microscopy showed bright and dark .Hair shafts were notably flattened, folded most probably during the hair mounting process. Trichoscopy in trichotiodystrophy patients was non-specific. At a higher magnification hairs showed a non-homogenous structure reassembling grains of sand within the hair shaft and a slightly wavy contour These features are visible in patients with dark and light hairs. This has to be distinguished from a symptom called intermittent medulla, observed in normal thick hair shafts, which may be wrongly diagnosed as pili annulati. In these cases, the medulla visible as light-colored constitutes less than 50% of the hair shaft width, the light bands are short and the intervals between them are usually shorter than the bands' own length. Our study shows that this feature can be distinguished by trichoscopy from the light bands in pili annulati, which take up more than a half of hair shaft width. The boarders of bands are not clear-cut and the intervals between these bands are usually longer than the bands' own length. Oblique Fractures- Tapered Fracture Fractures of the hair that cause a pencil point appearance are due to inhibition of nucleic acid and protein synthesis and therefore mitosis during anagen. Progresive narrowing of the emerging hair shaft may lead to a detached fractured hair with the thinner end near the scalp end. This is most commonly seen in anagen effluvium from cytotoxic chemotherapy drugs that disturb anagen hair shafts. Trichostasis Spinulosa Trichostasis spinulosa refers to multiple hairs that are present in one Trichostasis spinulosa refers to multiple hairs that are present in one follicular canal with 5 to 50 vellus hairs that are embedded in a large comedo. Trichostasis is follicular canal with 5 to 50 vellus hairs that are embedded in a large comedo. Trichostasis is now regarded as a normal, age-related process that is a variant of a comedo. It principally affects the chest and back, especially the scapular area and is less common on the abdomen, face, and neck. Topical retinoic acid is the most effective treatment. Prophylaxis of acquired hair shaft Defects ●Minimize damage from hair cosmetics ● Avoid harsh shampoos ● To comb dry hair ( since friction is higher in wet hairs ) Hair dryers applied on wet hair at high temperatures may also provoke the well – known phenomenon of Bubble hair . Conditioning agents help to protect hair . Therapy of hair shaft disorders should focus on the cause. In addition, minimizing traumatic influences to hair shafts, such as drying hair with an electric dryer or permanent waves and dyes, is important. A short hairstyle is more suitable for patients with hair shaft disorders.