Presentation
Worldwide, there are fewer than 100 reported cases and we present the second Indian patient with a CRLF1 genetic mutation [1-4]. [indianpediatrics.net]
A–D, Facial expression at different ages, from birth to present. E, Front and back views, at the present time. Note scoliosis and limited extension of elbows. F, Subjects' hands. Note consistent camptodactyly. 5, Subject CS37, aged 14 mo. [pubmed.ncbi.nlm.nih.gov]
The patient we are presenting had small ectopic nonfunctioning right kidney in the association with CISS. [hindawi.com]
This condition rarely occurs in infancy, and DOES NOT present with fevers or arthralgias. [autoinflammatory.org]
Symptoms present at birth, and continuous. [102] Symptoms may be present at birth or early infancy. [102] Purpuric rash presenting at birth or in infancy. Vasculitis noted on skin biopsy. [autoinflammatory-search.org]
Workup
Growth delay, developmental delay, decrease pain sensation, and hyperintense lesions in the subcortical white matter on the brain MRI were reported with some of the patients, basic hematologic and biochemical workup usually does not show any abnormalities [hindawi.com]
If the workup is negative for symptoms of other diseases, and the ANA is negative, it is likely that the Raynaud’s is primary. [raynauds.org]
Workup was done and he was referred to genetics. [jcnonweb.com]
Treatment
The authors also discussed pathogenesis, differential diagnosis, and recommended management and treatment. [malacards.org]
The most important way to combat excessive sweating from anxiety, however, is to make sure you receive treatment for anxiety and follow your treatment plan. [healthcentral.com]
Primary hyperhidrosis controlled with oxybutynin after unsuccessful surgical treatment. Clin Exp Dermatol. 2009 Dec. 34(8):e990-1. [Medline]. Bouman HD, Lentzer EM. The treatment of hyperhidrosis of hands and feet with constant current. [emedicine.medscape.com]
PMID: 24613578 Crisponi/cold-induced sweating syndrome: Differential diagnosis, pathogenesis and treatment concepts. [pubmed.ncbi.nlm.nih.gov]
[…] or other antihidrotic treatment. [patient.info]
Prognosis
Prognosis Primary focal hyperhidrosis usually runs a chronic course, although a small number of people spontaneously improve after the age of about 25 years. [ 1 ] [patient.info]
Prognosis While spontaneous remission can occur in certain individuals, most experience unremitting, crippling, and irreversible pain. [cme.dannemiller.com]
The prognosis for complete healing is virtually 100%. Second-degree frostbite involves blister formation. Blisters filled with clear fluid have a better prognosis than blood-tinged blisters. [wwwnc.cdc.gov]
The causes and mechanisms of hive formation are different in each instance, as are the prognosis and approaches to treatment. [worldallergy.org]
There is no clear pattern for either future prognosis.[ return to top ] What research is being done? Unfortunately, most research to date has focused on treatment, as opposed to cause and cure. [raynauds.org]
Etiology
Applicable To Excessive sweating Night sweats Secondary hyperhidrosis Code First Code First Help Certain conditions have both an underlying etiology and multiple body system manifestations due to the underlying etiology. [icd10data.com]
Etiology CISS is caused by mutations in the CRLF1 gene on chromosome 19p12 (CISS type 1). In one Australian man with no family history, the syndrome was associated with mutations in the CLCF1 gene on chromosome 11q13.3 (CISS type 2). [orpha.net]
Stüve-Wiedemann syndrome: LIFR and associated cytokines in clinical course and etiology. Orphanet J Rare Dis. 2014;9:34. [0-www-ncbi-nlm-nih-gov.brum.beds.ac.uk]
Stüve-Wiedemann syndrome: LIFR and associated cytokines in clinical course and etiology. Orphanet Journal of Rare Diseases. 2014;9:34. https://doi.org/10.1186/1750-1172-9-34 Accessed Feb. 5, 2018. Crisponi Syndrome. Orphanet. [rarediseases.org]
While traction injuries (as in whiplash) to the posterior cervical chain are the most common etiology, other causes include local trauma, chronic infection and coagulopathy. [cme.dannemiller.com]
Epidemiology
Summary Epidemiology So far, six cases have been reported of Norwegian, Israeli and Canadian origin. [orpha.net]
Epidemiology of hyperhidrosis in 2 population-based health care databases. J Am Acad Dermatol. 2018 Feb. 78 (2):358-362. [Medline]. Adar R, Kurchin A, Zweig A, Mozes M. Palmar hyperhidrosis and its surgical treatment: a report of 100 cases. [emedicine.medscape.com]
Allen G, Galer BS, Schwartz L: Epidemiology of complex regional pain syndrome: A retrospective chart review of 134 patients. Pain. 1999;80:539-544. [cme.dannemiller.com]
Epidemiology of injuries in northern areas. Arctic Med Res 51:5–14. Mills, WJ. 1991. Cold injury. Alaska Med 35:1. Morton, WE and JWS Hearle. 1975. Physical Properties of Textile Fibres. London: Heinemann. Newburgh, LH (ed.). 1949. [ilocis.org]
CRPS is currently viewed as involving interactions between the immune system, the ANS and the Central Nervous System (Rooij, A.M., (2010) in ‘Genetic and Epidemiological Aspects of Complex Regional Pain Syndrome’, Doctoral Thesis, Leiden University (ignore [princessinthetower.org]
Pathophysiology
According to Bruehl and Chung (2006), CRPS may be understood as a biopsychosocial disorder, whereby psychological, behavioural and pathophysiological factors interact in a highly complex manner. [princessinthetower.org]
Janig W: Pathophysiology of complex regional pain syndrome. Pain. 2005 an updated review. Seattle, ISAP Press. 2005;307-316. Janig W, Baron R: Complex regional pain syndrome is a disease of the central nervous system Clin Auton Res. 2002;12:150-164. [cme.dannemiller.com]
The pathophysiology of hypothermia. In International Reviews of Ergonomics, edited by DJ Oborne. New York:Taylor & Francis. 201–218. Middaugh, J. 1992. Epidemiology of injuries in northern areas. Arctic Med Res 51:5–14. Mills, WJ. 1991. Cold injury. [ilocis.org]
Prevention
For treatment, avoid further mechanical trauma to the area and prevent infection. [wwwnc.cdc.gov]
Treatment and prevention A doctor may prescribe the following to reduce or control sweating with diabetes: Clinical strength or prescription antiperspirants : Products that contain high doses of aluminum chloride can prevent sweating by blocking sweat [medicalnewstoday.com]
Shower daily, but avoid saunas and hot showers to prevent body temperatures from rising too rapidly. Keep your feet dry after a bath or shower to prevent the growth of micro-organisms. Foot odour is caused by bacterial growth. [healthxchange.sg]
It may not be possible to prevent hypohidrosis, but you can take steps to avoid serious illnesses related to overheating. Wear loose, light-colored clothing, and don’t overdress when it’s hot. [healthline.com]
It can be prevented by addition of salt to the diet (drinking beer was a suggested preventive measure in the UK in the 1920s!). Similarly, physiological mechanisms control the electrolyte composition of the body fluids via processes in the kidneys. [ilocis.org]