Presentation
Most present with subtle elevated indurated skin involving the upper back. Rarely, patients may present with more diffuse and widespread areas of involvement. [dermatologyadvisor.com]
Abstract We present a case of Scleredema Diabeticorum (SD) in a patient with diabetic neuropathy and restrictive respiratory disease with unusual skin lesion distribution. [ncbi.nlm.nih.gov]
Pyoderma gangrenosum is a neutrophilic dermatosis that presents as an ulcer with violaceous borders. [unboundmedicine.com]
Entire Body System
- Swelling
A diffuse, non-pitting swelling and induration of the skin define this disease. [pagepress.org]
Skin biopsy revealed the ballooning and swelling of collagen bundles with few scattered lymphocytes. Protein electrophoresis showed increased gamma globulin polyclonal bands. [ncbi.nlm.nih.gov]
[Case Reports] J Scleroderma Relat Disord. 2021 Oct; 6(3):311-315.Nakatsuji M, Ishimaru N, … Kinami S JS Scleredema is a rare cutaneous mucinosis characterized by diffuse swelling and non-pitting induration. [unboundmedicine.com]
Gastrointestinal
- Abdominal Distension
We report a case of a 56-year-old female with rapidly progressing skin thickening of limbs, face and trunk for 1 year and abdominal distension for 3 months. [unboundmedicine.com]
Skin
- Skin Disease
Most recently, ultraviolet A-1 phototherapy has become available for a variety of skin diseases. The first case of scleredema diabeticorum successfully treated with UVA-1 was published in 2004 [14]. [hindawi.com]
- Skin Thickening
Apart from SD, Case 2 also had thickening of the skin and fascia of her hands, with flexion contractures and extensive tenosynovitis. [jrheum.org]
Free article Abstract Scleredema adultorum of Buschke is a rare skin disease characterized by skin thickening and tightening typically at the neck and the upper part of the body. [pubmed.ncbi.nlm.nih.gov]
A 63-year-old man reported a 5-year history of skin thickening of the trunk and a 3-week history of dyspnea. Echocardiography revealed diffuse hypokinesis. Skin biopsies obtained from the waist showed thickened dermis with mucin. [unboundmedicine.com]
SAB is a rare skin disease characterized by skin thickening and tightening typically at the neck and the upper part of the body. [medicaljournals.se]
- Localized Scleroderma
Localized scleroderma and scleroderma-like syndromes. In: Hochberg MC, Gravallese EM, Silman AJ, Smolen JS, Weinblatt ME, Weisman MH, eds. Rheumatology. 7th ed. Philadelphia, PA: Elsevier; 2019:chap 155. [thnm.adam.com]
European Dermatology Forum S1-guideline on the diagnosis and treatment of sclerosing diseases of the skin, Part 1: localized scleroderma, systemic sclerosis and overlap syndromes. J Eur Acad Dermatol Venereol. 2017 Sep. 31 (9):1401-1424. [emedicine.medscape.com]
Currently, phototherapy, namely long-wave UV, such as UVA, is widely used in the treatment of localized scleroderma (morphea), but the exact mechanism of how it reduces the symptoms of scleroderma is unclear. [medicaljournals.se]
Workup
[Scleredema adultorum : Clinical presentation, diagnostic workup, differential diagnosis, treatment option]. Kreuter A. Kreuter A. Hautarzt. 2018 Nov;69(11):908-915. doi: 10.1007/s00105-018-4259-6. Hautarzt. 2018. PMID: 30128796 Review. German. [pubmed.ncbi.nlm.nih.gov]
Additional workup may be necessary that includes imaging studies and recruitment of subspecialists to treat affected organs. For example, scleredema involving the heart muscles may lead to cardiomyopathy resulting in congestive heart failure. [dermatologyadvisor.com]
There are no specific laboratory abnormalities, but the diagnostic workup of SAB should include analysis of anti-streptolysin O titre, haemoglobin A1c, sedimentation rate, blood count, and protein electrophoresis. [medicaljournals.se]
Treatment
At the end of the treatment (fiftieth treatment), the 17-MHz probe ultrasound was repeated at the same site. [ncbi.nlm.nih.gov]
No effective treatment is known for SD. [jrheum.org]
Treatment Options Treatment options for scleredema are summarized in Table I. Table I. [dermatologyadvisor.com]
Treatment may improve movement and breathing. [ucsfhealth.org]
Prognosis
[…] exposed to ultraviolet light Glucocorticoid medications (topical or oral) Electron beam therapy (a type of radiation therapy) Medicines that suppress the immune system Physical therapy, if you find it hard to move your torso or breathe deeply Outlook (Prognosis [ucsfhealth.org]
Amyloidosis: Recognition, confirmation, prognosis, and therapy. Mayo Clin Proc. 1999;74:490–4. [PubMed] [Google Scholar] 12. Banker BQ, Engel AG. Myology. New York: McGraw-Hill; 1986. The polymyositis and dermatomyositis syndrome; pp. 1385–422. [ncbi.nlm.nih.gov]
The majority of childhood cases are postinfectious and self-resolving. [21, 22] Prognosis The course of scleredema is unpredictable. [emedicine.medscape.com]
Etiology
Affiliations PMID: 33865279 Free article Case Reports Scleredema diabeticorum in a patient: un uncommon etiology of restrictive lung pattern Escleredema diabeticorum en un paciente: una etiología infrecuente de patrón restrictivo pulmonar Mónica García-Arpal [pubmed.ncbi.nlm.nih.gov]
ISSN 0304-4866, Vol. 78, Nº. 4 (Diciembre), 2017, págs. 165-167 Idioma: portugués Títulos paralelos: Buschke´s Scleredema - Case Report Enlaces Texto completo (pdf) Resumen English Scleredema of Buschke (EB) is a rare sclerotic disorder, of unknown etiology [dialnet.unirioja.es]
Other etiologic mechanism besides hyperglycemia probably takes place in some patients. There are different approaches for the treatment of scleredema adultorum. [hindawi.com]
Epidemiology
This review describes characteristic skin findings of diabetes, general skin findings related to diabetes, and findings related to diabetes treatment with a focus on clinical presentation, diagnosis, pathophysiology, epidemiology, and treatment. [unboundmedicine.com]
[…] due to mechanical stress. [16] Drug reaction: In 2005, scleredema was reported in association with the use of infliximab; although rare, treating physicians should consider the possibility of an adverse drug reaction as the underlying etiology. [17] Epidemiology [emedicine.medscape.com]
Pathophysiology
FREE Publisher Full TextPMC Free Full TextPMC Free PDF Cutaneous Manifestations of Nutritional Excess: Pathophysiologic Effects of Hyperglycemia and Hyperinsulinemia on the Skin. [unboundmedicine.com]
Etiology Pathophysiology The pathogenesis of scleredema is poorly understood. [dermatologyadvisor.com]
Pathophysiology Increased deposition of collagen and mucin (hyaluronic acid, an acid mucopolysaccharide) is seen in this condition; however, the causative mechanism is unknown. [emedicine.medscape.com]
Prevention
Keeping blood sugar levels within range helps prevent diabetes complications. [ucsfhealth.org]