Presentation
We present a report of two Indian male siblings aged 24 and 21, who presented with thinning and hair loss from the scalp in male pattern distribution and multiple skin-colored follicular papules over the nape of the neck and bilateral forearms since childhood [ijtrichology.com]
However, using trichogram analysis, pseudomoniletrix hair does not present with dystrophic constrictions but with flattened irregular beading. [orpha.net]
A 4-year-old girl presented with sparse, brittle hair on her entire scalp and keratosis pilaris on the nape of her neck. Subtle microscopic and macroscopic diagnostic features presented a challenge for physicians. [ncbi.nlm.nih.gov]
That was observed in our cases too, both siblings presented with variable severity of the same condition (the boy was affected more than his sister). [docksci.com]
Entire Body System
- Turkish
AIM: To identify the mutation in a consanguineous Turkish family with three affected children and apparently unaffected parents. METHODS: Sequencing analysis of the genes DSG4 and KRT86 was performed. [ncbi.nlm.nih.gov]
Pitfalls of mapping a large Turkish consanguineous family with vertical monilethrix inheritance. Genet Couns 2009;20:1-8. [PUBMED] 6. Horev L, Glaser B, Metzker A, Ben-Amitai D, Vardy D, Zlotogorski A. [ijtrichology.com]
"Pitfalls of mapping a large Turkish consanguineous family with vertical monilethrix inheritance". Genetic Counseling (Geneva, Switzerland). 20 (1): 1–8. PMID 19400537. ^ Freedberg; et al. (2003). [en.wikipedia.org]
- Weakness
Eventually, the hair may become so weak that it splits or breaks off completely. More usually, this splitting and breakage occurs to old hair, that is, toward the end of the hair fiber. [webmd.com]
A review of the research on various hair shaft disorders points out to weak hair formation due to disruption of the metabolism and biology of hair shaft formation. [omicsonline.org]
The hair snaps and becomes weak at the follicle causing hair loss. [aderanshaircentre.co.uk]
- Asymptomatic
2011 Patient details Age Child Location Iraq Localisation Head / scalp Primary Lesions Alopecia Alopecia / non scarring Diffuse hair shaft disorder Pathophysiology genetic disorders / adnexae genetic disorders / hair dysplasias / monilethrix Symptoms asymptomatic [dermquest.com]
Patient was asymptomatic, and there was no history suggestive of impaired psychomotor development. He did not give any history of similar illness in his family members. [ijtrichology.com]
Within 2-3 months of age, the parents noticed gradual development of multiple, asymptomatic, keratotic, tiny papules on the scalp along with breakage of hair and alopecia. [ijdvl.com]
- Falling
This promotes hair growth, but it also can make your hair fall out when you begin to use it because it gets rid of the non-active hair shafts so there is room for more healthy, growing ones. [riskswork93.blogspot.com]
What’s more, when you get Monilethrix treatment you can be assured of getting a top treatment for hair fall in India. [hairtreatmentmumbai.com]
The disease can exist with the birth, but more often manifests itself on the first year of life, when the fall out vellus hair, and the normal do not grow or appear broken. [oatext.com]
A 22 year young female of non-consanguineous marriage presented with thinning of hair, slow growth, dry lustreless and coarse hair, broken hair falling all over, especially seen on the pillow every morning, excessive hair loss during combing and shampoo [omicsonline.org]
Skin
- Alopecia
Use of dermoscopy in hair disorders has been described in Alopecia Areata, androgenetic alopecia, trichotillomania and cicatricial alopecias, and is referred to as trichoscopy. [1], [2], [3], [4] Diagnosis of hair shaft disorders is typically based on [ijtrichology.com]
Differential diagnosis Differential diagnosis includes pseudomonilethrix that presents with diffuse alopecia or alopecia limited to the occipital area. [orpha.net]
Figure 1: (a) Diffuse alopecia on the parietal scalp with broken hair. [ijdvl.com]
A 2-year-old boy presented with diffuse alopecia and persistent fragile hair for several months. Clinical examination revealed alopecia with hairs broken several millimeters from the scalp. [ncbi.nlm.nih.gov]
Case Overview Monilethrix Member Rated 0 Patient case no. 10964 Date added 05 April 2011 Patient details Age Child Location Iraq Localisation Head / scalp Primary Lesions Alopecia Alopecia / non scarring Diffuse hair shaft disorder Pathophysiology genetic [dermquest.com]
- Keratosis
The background scalp appearance of keratosis pilaris persisted in these areas. This suggests that the beading alone is influenced by etretinate and is not solely an artefact secondary to the hyperkeratotic condition at the follicular opening. [ncbi.nlm.nih.gov]
In this family the disease was expressed in four of 12 cases only as a follicular keratosis of the neck, elbows and knees, and without clinical or historical evidence of hair anomalies: non-penetrance in an obligate carrier was also observed. [eprints.soton.ac.uk]
- Papule
Dermoscopy of the keratotic papules revealed short, brittle hair arising from them with similar features Figure 5]. [ijtrichology.com]
Dermoscopy of the keratotic papules on neck showed beaded hair arising from the papules [Figure 2] b. The forearm showed no hair clinically but on dermoscopy, beaded short hairs were seen. [ijdvl.com]
Hair was absent on the rest of the body and multiple skin colored horny papules were seen on the upper back. [edoj.org.eg]
- Hyperkeratosis
Alopecia, associated with follicular hyperkeratosis and perifollicular erythema, can involve only the occiput and the nape of the neck or, in severe forms, the entire scalp, the eyebrows and eyelashes, and secondary hair. [orpha.net]
Abstract Mutations in the hair keratins hHb1 and hHb6 have been recently reported to cause monilethrix, an autosomal dominant hair shaft disorder, characterized by variable degrees of hair fragility and follicular hyperkeratosis. [ncbi.nlm.nih.gov]
[…] the hair keratins hHb1 and hHb6 have been recently reported to cause monilethrix, an autosomal dominant hair shaft disorder, characterized by variable degrees of hair fragility and follicular hyperkeratosis. [digital.csic.es]
Can be seen small nodules hyperkeratosis. When microscopy on the rods hair determined by the spindle - shaped thickening and waist. It was established diagnosis - monilethrix. [oatext.com]
- Follicular Hyperkeratosis
Alopecia, associated with follicular hyperkeratosis and perifollicular erythema, can involve only the occiput and the nape of the neck or, in severe forms, the entire scalp, the eyebrows and eyelashes, and secondary hair. [orpha.net]
Abstract Mutations in the hair keratins hHb1 and hHb6 have been recently reported to cause monilethrix, an autosomal dominant hair shaft disorder, characterized by variable degrees of hair fragility and follicular hyperkeratosis. [ncbi.nlm.nih.gov]
Neurologic
- Irritability
Liver About the Anus & Rectum About the Gallbladder and Biliary Tract About the Pancreas Diseases & Conditions Colon Cancer Diverticular Disease Foodborne Illness Gastroesophageal Reflux Disease (GERD) Gastrointestinal Cancers Inflammatory Bowel Disease Irritable [drdeguzman.com]
E, preparations containing phytin( calcium magnesium salt phytic acid), iron and food gelatin; - the local treatment - softening keratinized particles skin, exfoliating and mitigating ointment( 1% salicylic, ointment with 5% urea); - treatments with irritating [oatext.com]
This can severely irritate the stomach lining, leading to severe ulceration. It is possible to die from trichophagia. Treating trichotillomania is difficult; therapists can probably help more than dermatologists. [webmd.com]
Workup
There were no hematological abnormalities noted in routine workup. Her serum copper and ceruloplasmin levels were also normal. The examination of the parents and the elder brother was unremarkable. [ijpd.in]
Treatment
There is no known treatment that successfully cures the condition. In this report we present a case of monilethrix in a 7-year-old girl treated with oral acitretin. A very good clinical and cosmetic result was obtained while treatment was continued. [ncbi.nlm.nih.gov]
Management and treatment Treatment is disappointing. The best measure is to avoid the mechanical action of combing and brushing. Depilation only achieves repopulation with normal looking hair for a short while. [orpha.net]
[…] result was obtained while treatment was continued with oral acitretin in a 7-year-old girl. [e-ijd.org]
Your doctor will probably start your Monilethrix treatment in Mumbai, India with a topical treatment method. The Monilethrix treatment will start with prescribing you a 2% minoxidil solution. [hairtreatmentmumbai.com]
Prognosis
Prognosis The course of the localized form of monilethrix can be favorable with a reduction of hypotrichosis with time. In general, hypotrichosis improves with age, although remissions have been noted during puberty or pregnancy. [orpha.net]
Differential Diagnosis Some other hair shaft anomalies merit consideration in the differential diagnosis of monilethrix, such as pseudo-monilethrix, congenital alopecia, trichorrhexis invaginata, and ectodermal dysplasia.[9] Prognosis The prognosis of [statpearls.com]
Etiology
In conclusion, our data strongly suggest that monilethrix is a disease of the hair cortex, whose etiology is interesting in that causative mutations seem to be restricted to type II hair keratins. [ncbi.nlm.nih.gov]
Synopsis alopecia perifollicular hyperkeratosis microscopy: beaded hair Etiology mutations in the hair cortex keratin genes KRTHB1 (MIM.602153) or KTHB6 (MIM.601928) See also KRTHs [humpath.com]
Etiology Four genes have been associated with monilethrix: KRT81, KRT83 and KRT86, coding for the type II hair keratins Hb1, Hb3 and Hb6, and are responsible for the autosomal dominant form of the disease. [orpha.net]
Picture Diseasesof the Skin and its Appendages Lethal Mutations Surviving by Mosaicism References Clinical Aspects Chapter 15Structural Anomalies of the Hair Shaft 6Diagnosis and Treatment Anomalies Without Increased Hair Fragility References Monilethrix Etiology [books.google.com]
Objectives: Explain the etiology of monilethrix. Describe the typical presentation of monilethrix. Summarize the treatment for monilethrix. [statpearls.com]
Epidemiology
Summary Epidemiology The prevalence and incidence are not known. Clinical description Monilethrix has an early onset in infancy. [orpha.net]
Sections Continuing Education Activity Introduction Etiology Epidemiology Pathophysiology History and Physical Evaluation Treatment / Management Differential Diagnosis Prognosis Complications Enhancing Healthcare Team Outcomes Review Questions References [pubmed.ncbi.nlm.nih.gov]
Other treatment is symptomatic and supportive.[6] Epidemiology[edit] Currently, the frequency for this disease is unknown and affects both men and women. [en.wikipedia.org]
The suggestion is that the autosomal recessive monilethrix results from mutations in the desmoglein 4 gene (DSG4), the only desmoglein expressed in the keratinizing zone of the hair shaft cortex.[4] Epidemiology The prevalence and incidence are not known [statpearls.com]
Pathophysiology
Case Overview Monilethrix Member Rated 0 Patient case no. 10964 Date added 05 April 2011 Patient details Age Child Location Iraq Localisation Head / scalp Primary Lesions Alopecia Alopecia / non scarring Diffuse hair shaft disorder Pathophysiology genetic [dermquest.com]
Chapter 15Structural Anomalies of the Hair Shaft 6Diagnosis and Treatment Anomalies Without Increased Hair Fragility References Monilethrix Etiology and Pathogenesis Hypomelanotic Hair Disorders 2Modelsfor Cultivating Follicular Keratinocytes In Vitro Pathophysiological [books.google.com]
Pathophysiology The exact mechanisms causing the periodic change in the diameter of the hair in monilethrix are still not clear. [statpearls.com]
Sections Continuing Education Activity Introduction Etiology Epidemiology Pathophysiology History and Physical Evaluation Treatment / Management Differential Diagnosis Prognosis Complications Enhancing Healthcare Team Outcomes Review Questions References [pubmed.ncbi.nlm.nih.gov]
Prevention
How can Monilethrix be Prevented? Currently, Monilethrix may not be preventable, since it is a genetic disorder. [dovemed.com]
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The FDA acknowledged and endorsed the effectiveness of low level laser therapy (LLLT) to prevent baldness in women and men. LLLT is recommended by American and European professionals to stimulate growth and thickening of hair. [grivamax.com]
Monilethrix treatments Can you prevent monilethrix? If you have any medical questions or concerns, please talk to your healthcare provider. [ro.co]