Dexamethasone Pulse Therapy for Scleredema

Dogra, Sunil; Handa, Sanjeev; Kanwar, Amrinder J.

doi:10.1111/j.0736-8046.2004.21325.x

Cited by 9 publications

(2 citation statements)

References 12 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Our study confirms that there is no single universally effective therapy for scleredema [8][9][10][11][12][13] . Physical therapies such as UVA1, bath PUVA, cream PUVA, low-dose broad-band UVA and narrow-band UVB [14][15][16][17][18] , localized electron beam therapy and photopheresis 19,20 , intravenous immunoglobulin (IVIg) [21][22][23] have been reported to improve the disease in anecdotal reports or small case series.…”

Section: Discussionsupporting

confidence: 83%

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

Rongioletti

Merlo

Cinotti

et al. 2015

Acad Dermatol Venereol

115

View full text Add to dashboard Cite

show abstract

Section: Discussionsupporting

confidence: 83%

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

Rongioletti

Merlo

Cinotti

et al. 2015

Acad Dermatol Venereol

115

View full text Add to dashboard Cite

show abstract

“…24 Joint restriction, although common in SSS, is rare in scleredema and is more often due to a "bulk" effect of the thick skin usually affecting the eyelids, neck, mouth, and shoulder. 18,[26][27][28][29] The skin stiffness in SSS, in our experience, is often well demarcated, uneven, or lumpy and may not involve an entire anatomic unit, while the stiffness in scleredema is more uniform, typically encompassing an entire involved anatomic unit. The abrupt onset often seen in scleredema is not a feature of SSS.…”

Section: Differential Diagnosismentioning

confidence: 73%

The Stiff Skin Syndrome

Liu¹,

McCalmont²,

Frieden³

et al. 2008

Arch Dermatol

View full text Add to dashboard Cite

Background: Stiff skin syndrome is a sclerodermalike disorder that presents in infancy or early childhood with rock-hard skin, limited joint mobility, and mild hypertrichosis in the absence of visceral or muscle involvement, immunologic abnormalities, or vascular hyperreactivity.Observations: We describe 6 children who fit criteria for stiff skin syndrome. A review of the clinical range of this disorder and discussion of the differential diagnosis is presented. The age at onset in our cases ranged from infancy to 6 years of age. Stony-hard skin was noted mostly on the thighs, buttocks, and lower back with shoulder and arm involvement in 2 cases. There was associated hypertrichosis in 3 of 6 cases. Extracutaneous manifestations consisted primarily of joint restriction, and several patients had resulting postural and thoracic wall ir-regularities. Histopathologically, our cases showed areas of fascial sclerosis or showed increased fibroblast cellularity with thickened, sclerotic, horizontally oriented collagen bundles in the deep reticular dermis and/or subcutaneous septa without associated inflammation.Conclusions: Stiff skin syndrome is characterized by an early, insidious onset of stony-hard skin, often with associated contracturelike joint restriction, hypertrichosis, and postural and thoracic wall abnormalities. Supportive histopathologic findings consisting of either fascial sclerosis or increased fibroblast cellularity with sclerotic collagen bundles in the deep reticular dermis and/or subcutaneous septa may help to confirm this diagnosis.

show abstract

Clinical Case Vignettes: Connective Tissue Diseases in Children

Gupta

Aggarwal

2016

Pediatric Rheumatology

View full text Add to dashboard Cite

Dexamethasone Pulse Therapy for Scleredema

Cited by 9 publications

References 12 publications

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

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

The Stiff Skin Syndrome

Clinical Case Vignettes: Connective Tissue Diseases in Children

Contact Info

Product

Resources

About