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 similar history was present in mother, maternal aunts, and maternal grandfather. On cutaneous examination, hairs were sparse, short, blonde colored and dry. [ijpd.in]
It may remain un-presented until beyond adolescence. In children the hairshafts exhibit swellings or nodes with intervening constrictions. [hairscientists.org]
Respiratoric
- 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]
Gastrointestinal
- 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]
Cardiovascular
- 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]
Skin
- No Pubic Hair
Eyelashes, eyebrows, pubic hair, limb hair and axillary hair remain largely unaffected. Diagnosis Trichorrhexis invaginata or Bamboo hair condition is a similar hair disorder, and monilethrix has to be differentially diagnosed from this condition. [qr678.in]
In more severe cases, hair over the whole scalp can be affected, as well as pubic hair, underarm hair, eyebrows, eyelashes or hair on the arms and legs. The hair snaps and becomes weak at the follicle causing hair loss. [aderanshaircentre.co.uk]
The condition is described mainly on the scalp but can be observed and pubic hair or other body areas. [medicaldb.blogspot.com]
In more severe cases, it can be affected hair all over the scalp and pubic hair, underarm hair, eyebrows, eyelashes and hair on the arms and legs. Sometimes, skin and nails are also affected. [ivami.com]
- Hair Growth Increased
Case Report A 2-year-old female born of a nonconsanguineous marriage presented to us with a history of decreased hair growth, increased hair fragility, and sparsity of the hair over the scalp and eyebrows since birth. [ijpd.in]
Psychiatrical
- Suggestibility
It has been suggested that mutation in hHb1 produces a less severe phenotype. [ncbi.nlm.nih.gov]
- Low Self-Esteem
self-esteem and negative body perception, hence the need to assess its impact on the quality of life.[12] Other than psychological impact, complications related to the treatment modalities. [statpearls.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]
Other Pathologies
- Lymphocytic Infiltrate
Histopathology of a keratotic papule revealed abnormal hair shaft with constriction and bulges associated with perifollicular fibroplasia and sparse lymphocytic infiltrate [Figure 7]. [8] Monilethrix is a term of Greek and Latin derivation meaning "beaded [ijtrichology.com]
Treatment
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]
[…] 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]
The prognosis of this disease is unknown. [oatext.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]
Objectives: Explain the etiology of monilethrix. Describe the typical presentation of monilethrix. Summarize the treatment for monilethrix. [statpearls.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.com]
Epidemiology
Summary Epidemiology The prevalence and incidence are not known. Clinical description Monilethrix has an early onset in infancy. [orpha.net]
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]
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.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]
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.com]
Prevention
[…] 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]
How can Monilethrix be Prevented? Currently, Monilethrix may not be preventable, since it is a genetic disorder. [dovemed.com]
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]
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]