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a skin disease marked by hard edema of the tissue usually beginning in the face

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出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2015/06/05 10:24:49」(JST)

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Scleredema, also known as Buschke disease, scleredema of Buschke, and scleredema adultorum,^[1]^[2] is a rare, self-limiting skin condition defined by progressive thickening and hardening of the skin, usually on the areas of the upper back, neck, shoulders and face.^[3] The skin may also change color to red or orange. The disease was discovered by Abraham Buschke. Although the cause of scleredema is unknown, it is usually associated with a disease, usually diabetes,^[4] a viral illness or strep throat.^[5] It is usually not fatal, but it may cause death if the disease spreads to the internal organs.^[3] It may also cause an infection.^[5]

Diagnosis

The scleredema is usually proposed as a diagnosis based on the appearance of the skin and the patient's medical history. To confirm the diagnosis, the doctor performs a skin biopsy, in which hematoxylin and eosin staining will show a thick reticular dermis with thick collagen bundles separated by clear spaces.^[6] The patient's blood may be examined for diseases that may appear after the onset of symptoms, such as multiple myeloma.^[5]

Treatment

Although many types of medications have been tried as treatments, none of them have been proven effective in treating scleredema. Those treatments, such as corticosteroids, may benefit the patient, but will not cure their condition. If the affected area is infected, it is usually treated immediately. The symptoms of the condition usually resolve within six months to two years after onset. However, patients whose condition was associated to diabetes may suffer for longer periods of time.^[5]

Myocarditis resulting as a complication from the disease has been successfully treated with penicillin and steroids.^[7]

References

^ Turchin I, Adams SP, Enta T (September 2003). "Dermacase. Scleredema adultorum, or Bushke disease". Can Fam Physician 49: 1089, 1093. PMC 2214291. PMID 14526859.
^ Pegum JS (June 1972). "Scleredema of Buschke". Proc. R. Soc. Med. 65 (6): 528. PMC 1643957. PMID 5044976.
^ ^a ^b "Scleredema". Retrieved 2009-05-18.
^ Meguerditchian, C; Jacquet P, Béliard S et al. (November 2006). "Scleredema adultorum of Buschke: an under recognized skin complication of diabetes". Diabetes and Metabolism 32 (5): 481–484. doi:10.1016/S1262-3636(07)70307-5. PMID 17110904.
^ ^a ^b ^c ^d "Scleredema". Retrieved 2009-05-18.
^ Schmults CA (October 2003). "Scleredema". Dermatol. Online J. 9 (4): 11. PMID 14594584.
^ "Buschke's Sclerema with right-sided heart failure.". Retrieved 2009-05-18.

UpToDate Contents

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3. 好酸球性筋膜炎 eosinophilic fasciitis
4. 若年性全身性硬化症（強皮症） juvenile systemic sclerosis scleroderma
5. 血管浮腫の概要：臨床的特徴、診断、およびマネージメント an overview of angioedema clinical features diagnosis and management

English Journal

Scleredema. A multicentre study of characteristics, comorbidities, course and therapy in 44 patients.

Rongioletti F1, Kaiser F1, Cinotti E1,2, Metze D3, Battistella M4, Calzavara-Pinton PG5, Damevska K6, Girolomoni G7, André J8, Perrot JL2, Kempf W9, Cavelier-Balloy B10.
Journal of the European Academy of Dermatology and Venereology : JEADV.J Eur Acad Dermatol Venereol.2015 Aug 24. doi: 10.1111/jdv.13272. [Epub ahead of print]
BACKGROUND: The prognostic and therapeutic features of scleredema are poorly documented.OBJECTIVES: To describe the characteristics of patients with scleredema regarding demographics, clinical characteristics, comorbidities, therapeutic interventions and course.METHODS: We conducted a retrospective
PMID 26304054

Scleredema of Buschke Following Streptococcal Infection.

Jung SE1, Kim YC1.
Annals of dermatology.Ann Dermatol.2015 Aug;27(4):478-80. doi: 10.5021/ad.2015.27.4.478. Epub 2015 Jul 29.
PMID 26273177

Clinical correlates of human leucocyte antigen (HLA)-G in systemic sclerosis.

Favoino E1, Favia IE1, Vettori S2, Vicenti C1, Prete M1, Valentini G2, Perosa F1.
Clinical and experimental immunology.Clin Exp Immunol.2015 Jul;181(1):100-9. doi: 10.1111/cei.12633. Epub 2015 May 10.
Human leucocyte antigen (HLA)-G has a tolerogenic function and could play a role in the pathogenesis of immune-mediated diseases, including systemic sclerosis (SSc). The aim of this study was to evaluate HLA-G serum expression (sHLA-G) and the HLA-G gene 14 base pairs (bp) insertion/deletion (del(-)
PMID 25847615

Japanese Journal

Scleredema diabeticorum successfully treated with ultraviolet A1 phototherapy

THUMPIMUKVATANA Narumol,WONGPRAPARUT Chanisada,LIM Henry W.
Journal of dermatology 37(12), 1036-1039, 2010-12-01
NAID 10028275952

糖尿病性浮腫性硬化症(<特集>皮膚疾患とアフェレシス)

小林愛,福成健一,古賀伸彦
日本アフェレシス学会雑誌 27(2), 145-151, 2008-05-31
… Diabetic scleredema (DS) has been reported to occur in 2.5-14% of diabetic patients. …
NAID 110006664589