Presentation
The case of a 37-year-old male suffering from Gottron's acrogeria and cutaneous sarcoidosis is presented together with a thorough review of the literature. So far about 40 cases of Gottron's acrogeria have been described. [ncbi.nlm.nih.gov]
There is hyperpigmentation present irregularly which is the increased color of the skin which is probably present as patches of hyper-colored skin. Along with this fine hair is seen to be present on the skin in almost all the cases of Acrogeria. [epainassist.com]
Acrogeria was originally described by Gottron in 1941, when he noticed premature cutaneous aging localized on the hands and feet in two brothers, which were present ever since birth. [en.wikipedia.org]
Entire Body System
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Myxedema
FSH insensitivity LH/hCG insensitivity posterior (Neurogenic diabetes insipidus) general Empty sella syndrome Pituitary apoplexy Sheehan's syndrome Lymphocytic hypophysitis Thyroid Hypothyroidism Iodine deficiency Cretinism Congenital hypothyroidism Myxedema [theinfolist.com]
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Goiter
[…] hyperthyroxinemia Hashitoxicosis Thyrotoxicosis factitia Graves' disease Thyroiditis Acute infectious Subacute De Quervain's Subacute lymphocytic Autoimmune/chronic Hashimoto's Postpartum Riedel's Goitre Endemic goitre Toxic nodular goitre Toxic multinodular goiter [theinfolist.com]
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Euthyroid
[…] insensitivity LH/hCG insensitivity posterior (Neurogenic diabetes insipidus) general Empty sella syndrome Pituitary apoplexy Sheehan's syndrome Lymphocytic hypophysitis Thyroid Hypothyroidism Iodine deficiency Cretinism Congenital hypothyroidism Myxedema Euthyroid [theinfolist.com]
Musculoskeletal
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Coxa Valga
Other features were osteolysis of the clavicles, soft tissue calcification, osteoporosis and coxa valga, which have not been described in previous reported cases. [ncbi.nlm.nih.gov]
[…] hypertension - Contractures / cramps / trismus / tetania / claudication / opisthotonos - Ecchymoses - Epicanthic folds - Face / facial anomalies - Hemorrhage / hemorrhagic syndrome / excessive / long-lasting bleeding - Hip dislocation / dysplasia / coxa [csbg.cnb.csic.es]
valga,9,10 but no skeletal defects were present in our patient. [docksci.com]
Workup
Diagnosis of Acrogeria Since there are no molecular workups that can aid in the diagnoses of Acrogeria, it’s mainly and exclusively just a clinical diagnosis- a proper history including family history and clinical examination. [epainassist.com]
Treatment
The complications of Acrogeria, Gottron type may include: Severe skin abnormalities Difficulty in walking Decreased quality of life Complications may occur with or without treatment, and in some cases, due to treatment also. [dovemed.com]
CLOSE Medical Disclaimer The medical information on this site is provided as an information resource only, and is not to be used or relied on for any diagnostic or treatment purposes. [diseaseinfosearch.org]
What are the treatments for Acrogeria- Gottron Type? Since Acrogeria- Gottron Type is a genetic condition, there is no cure. Treatment is based on the type and severity of each patient and is very individualized. [patientworthy.com]
Treatment of Acrogeria As of now, there is no known treatment for Acrogeria. However, this isn’t a major problem considering that Acrogeria has an excellent prognosis. [epainassist.com]
Treatment[edit] There is currently no specific treatment available for either of these so-called progeroid syndromes. [theinfolist.com]
Prognosis
Prognosis is a term used to explain the positive progress of a disease- such as how a disease responds to the treatment used. [epainassist.com]
Prognosis of these patients is good as they have no tendency to develop atheroma or diabetes mellitus. We report a case 11 year old female child of acrogeria for its rarity. Crossref - DOI Crossref - DOI Prefix: 10.18535/jmscr [jmscr.igmpublication.org]
With this in mind, what is most important when making a differential diagnosis with them is based on the prognosis, which appears to be far better in acrogeria. [diki.pl]
Etiology
Copyright by The Korean Orthopaedic Association Byung Woo Ahn, Sang Youp Lee, Chong Kwan Kim, Chan Hyup Park and Yong Hi Chung Abstract Gottron's acrogeria syndrome is a rare genetic disease of unknown etiology with some features suggestive of accelerated [synapse.koreamed.org]
The etiology of acrogeria is still not well determined. With this in mind, what is most important when making a differential diagnosis with them is based on the prognosis, which appears to be far better in acrogeria. [diki.pl]
[…] have cardiac abnormalities Mitral valve prolapse Aortic root dilation with occasional rupture Scoliosis Pes planus (flatfoot) Molluscoid pseudotumors (calcified hematomas) may be associated with scars Inheritance Autosomal dominant single-gene disorder Etiology [en.wikibooks.org]
However, this concept is still not widely accepted in the medical literature. 9 As these are very rare syndromes, all sharing an aspect of ageing skin similar to progeria, they are also at times called progeroid syndromes. 10 The etiology of acrogeria [scielo.br]
Epidemiology
[…] data: Class of prevalence: 1-9 / 100 000 Average age onset: neonatal/infancy Average age of death: no data available Type of inheritance: autosomal dominant Epidemiological data: (no data available) External references: 1 OMIM reference - No MeSH references [csbg.cnb.csic.es]
[citation needed] Epidemiology[edit] Acrogeria is extremely rare, with only about 40 cases having been reported in the medical literature, since 1941. [theinfolist.com]
Epidemiology Acrogeria is extremely rare, with only about 40 cases having been reported in the medical literature, since 1941. [medbox.iiab.me]
With this in mind, what is most important when making a differential diagnosis with them is based on the prognosis, which appears to be far better in acrogeria. [ citation needed ] Epidemiology [ edit ] Acrogeria is extremely rare, with only about 40 [en.wikipedia.org]
Pathophysiology
Ascending aortic dilatation associated with bicuspid aortic valve: pathophysiology, molecular biology, and clinical implications. Circulation 2009; 119 : 880–890. 17. Janssens K, Vanhoenacker F, Bonduelle M, et al. [nature.com]
Prevention
How can Acrogeria, Gottron type be Prevented? Currently, Acrogeria, Gottron type may not be preventable, since it is a genetic disorder. [dovemed.com]
Preventing Acrogeria While there are no known ways to prevent the occurrence of this very rare disease, genetic counseling can be seen to help prevent voluntary transmissions of the disease to the future generations. [epainassist.com]
Each mutation is private for the affected family or individual concerned having the potential for early prenatal diagnosis and prevention. The full text of this article hosted at iucr.org is unavailable due to technical difficulties. [onlinelibrary.wiley.com]
[…] about every moisturizer I can find, and I was a human nutrition major in college, so I take all kinds of supplements (omega-3 fatty acids, D-3, multi-vitamin, extra magnesium, CoQ10, hawthorn, vitamin C complex - most of these are for the heart, to prevent [inspire.com]
The medical management for patients with EDS IV is primarily preventative and symptomatic. [crd.york.ac.uk]