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Monilethrix

Moniliform Hair Syndrome


Presentation

  • However, using trichogram analysis, pseudomoniletrix hair does not present with dystrophic constrictions but with flattened irregular beading.[orpha.net]
  • The presentation may be of alopecia (baldness). Individuals vary in severity of symptoms. Nail deformities may also be present as well as hair follicle keratosis and follicular hyperkeratosis.[en.wikipedia.org]
  • 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]
Sneezing
  • The parents gave a history of recurrent sneezing, repeated episodes of rhinitis and dryness of skin. There was a history of similar but milder complaints in an older sister.[ijdvl.com]
Rhinitis
  • The parents gave a history of recurrent sneezing, repeated episodes of rhinitis and dryness of skin. There was a history of similar but milder complaints in an older sister.[ijdvl.com]
Gagging
  • In the Russian family, we found a G to A transition at the first base of codon 402, resulting in a lysine substitution (GAG to AAG), designated E402K.[ncbi.nlm.nih.gov]
Venous Insufficiency
  • We present a case of pseudo-Kaposi’s sarcoma without clinical evidence of venous insufficiency but later detected by Doppler studies, and responded well to treatment with dapsone. The histological features were characteristic.[docksci.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]
  • Nail deformities may also be present as well as hair follicle keratosis and follicular hyperkeratosis.[en.wikipedia.org]
Dry, Brittle Hair
  • Lusterless, dry, brittle hair with alopecia and keratosis pilaris mainly on the occiput. The hair of this 4-year-old boy was never cut. Monilethrix that affects the entire scalp. Pathophysiology The etiology of monilethrix remains obscure.[emedicine.medscape.com]
Nail Deformity
  • Nail deformities may also be present as well as hair follicle keratosis and follicular hyperkeratosis.[en.wikipedia.org]
  • Hair follicle keratosis, follicular hyperkeratosis & nail deformities may also be manifested. There is no known effective treatment for the disorder.[signssymptoms.org]
  • Patients with monilethrix are also prone to developing keratosis pilaris (small bumps on the skin that often appear on the scalp and/or extensor surfaces of arms and thighs) in addition to nonspecific nail deformities such as koilonychia ("spoon nails[aocd.org]
Sparse Eyebrows
  • Fig 2: Short and sparse eyebrows and eyelashes. Discussion This genetic disorder of hair shaft structure with increased fragility was first described by Walter Smith in 1879 as "a rare nodose condition of the hair" [ 1 ].[edoj.org.eg]
Suggestibility
  • It has been suggested that mutation in hHb1 produces a less severe phenotype.[ncbi.nlm.nih.gov]

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]
  • Monilethrix treatment Once the condition has been diagnosed, treatment can be sought out from a trained trichologist. As it is a congenital condition, monilethrix can’t be permanently treated.[qr678.in]
  • 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]
  • […] result was obtained while treatment was continued with oral acitretin in a 7-year-old girl.[e-ijd.org]

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]
  • The prognosis of this disease is unknown.[oatext.com]
  • Phenotype and prognosis in monilethrix due to mutations of hair keratins hHb 1 and hHb 6. Br J Dermatol 1999; 141 Suppl. 55: 102–7 Google Scholar 6. Zlotogorski A, Christiano AM. Mapping of monilethrix and papular atrichia [letter].[link.springer.com]
  • Prognosis Monilethrix is a lifelong disease. Symptoms spontaneously regress during puberty and pregnancy, but the condition never disappears completely. Zlotogorski A, Marek D, Horev L, Abu A, Ben-Amitai D, Gerad L, et al.[emedicine.medscape.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]
  • 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]
  • Pathophysiology The etiology of monilethrix remains obscure. Results of genetic linkage analysis suggest that monilethrix is likely caused by a mutation in a hair keratin.[emedicine.medscape.com]

Epidemiology

  • Summary Epidemiology The prevalence and incidence are not known. Clinical description Monilethrix has an early onset in infancy.[orpha.net]
  • Congenital monilethrix and hereditary unilateral external auditory canal atresia were found to be co-inherited in a Chinese pedigree with recurrent KRT86 mutation. [16] A novel missense KRT86 mutation was documented in a Chinese family with monilethrix. [17] Epidemiology[emedicine.medscape.com]
Sex distribution
Age distribution

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 etiology of monilethrix remains obscure. Results of genetic linkage analysis suggest that monilethrix is likely caused by a mutation in a hair keratin.[emedicine.medscape.com]

Prevention

  • How can Monilethrix be Prevented? Currently, Monilethrix may not be preventable, since it is a genetic disorder.[dovemed.com]
  • […] a Priority in 2019 Here's How the Government Shutdown Could Affect Your Health More Proof High-Fiber Diets Help Prevent Cancers, Heart Disease Foot Stools Move Human Stool Along Not found Sorry, the article you requested could not be found[drdeguzman.com]
  • This will define the cause of your specific hair loss condition and identify the best hair treatment that may prevent further hair loss . To find out more go to hair & scalp analysis .[aderanshaircentre.co.uk]
  • There is no cure for monilethrix, but patients can take preventive measures such as wearing protective wigs or hats to help protect their natural hair.[wisegeek.com]

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