Presentation
We present a case of a 56-year-old woman with non insulin-dependent diabetes mellitus who presented with a progressive history of thickening of the skin on her back. [endocrine-abstracts.org]
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]
We present a case of scleredema associated with hypergammaglobulinemia. The skin findings had been progressive for 2 years before he was diagnosed with scleredema. [ncbi.nlm.nih.gov]
If infection is present, antibiotics may be required. If diabetes is present, it should be controlled with diet, exercise, and medication. Physical therapy may help if movement of body parts is restricted. VeryWell. [web.archive.org]
Entire Body System
- Fatigue
However, the patient treated with mixed photon therapy developed sore throat, nausea, fatigue, and headache during treatment. [appliedradiationoncology.com]
CASE REPORTS Case 1 A 55-year-old white man (skin type IV) had a 10year history of general fatigue and progressive tightness and thickening of the skin of his upper and lower extremities and back. [kundoc.com]
The treatment was well tolerated with the exception of patient 2, who received a combination of penetrating photons and electron-beam therapy and who complained of a temporary sore throat, headache, and fatigue. [jamanetwork.com]
While the aggressive disease can be associated with constitutional symptoms such as profound fatigue and loss of weight, there is usually no visceral involvement. [indianjrheumatol.com]
Jaw & Teeth
- Trismus
Journal of College of Medical Sciences-Nepal 9(3): 60-62, 2014 Trismus and focal infection in a case of Buschke's scleredema adultorum. [eurekamag.com]
There was minimal trismus and the throat was congested. Joints were normal. Cardiovascular system examination revealed tachycardia, S3 gallop and a short systolic murmur at left sternal edge. There was no cardiomegaly. She had hepatomegaly. [pediatriconcall.com]
Cardiovascular
- S3 Gallop
Cardiovascular system examination revealed tachycardia, S3 gallop and a short systolic murmur at left sternal edge. There was no cardiomegaly. She had hepatomegaly. [pediatriconcall.com]
- Mitral Valve Prolapse
Echocardiography showed mitral valve prolapse without mitral regurgitation., Blood culture was sterile. Skin biopsy showed swollen collagen bundles with frozen section showing bluish material in between the collagen fibers. [pediatriconcall.com]
- Systolic Murmur
Cardiovascular system examination revealed tachycardia, S3 gallop and a short systolic murmur at left sternal edge. There was no cardiomegaly. She had hepatomegaly. [pediatriconcall.com]
Skin
- Insect Bite
She had no history of an insect bite. Cutaneous examination revealed a well-defined erythematous, shiny, firm, non-tender plaque (measuring 3 cm X 4 cm) on the extensor surface of the lower third of her left forearm. [cyberleninka.org]
Eyes
- Prolapse
Echocardiography showed mitral valve prolapse without mitral regurgitation., Blood culture was sterile. Skin biopsy showed swollen collagen bundles with frozen section showing bluish material in between the collagen fibers. [pediatriconcall.com]
Musculoskeletal
- Arthralgia
Up to 9% of these patients can have arthralgias. [6] Unlike SSc, however, digital ulceration, nail fold capillary changes, Raynaud's phenomenon, resorption of phalanges, and internal organ involvement is very rare. [indianjrheumatol.com]
- Joint Effusion
Systemic involvement, including pleural, pericardial and joint effusions, has recently been stressed. Twenty-seven per cent of the cases occurred within the first decade and females were involved more frequently than males. [amjmed.com]
Workup
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
Treatment Options Treatment options for scleredema are summarized in Table I. Table I. [dermatologyadvisor.com]
[…] as the first-line treatment of this disease. [ncbi.nlm.nih.gov]
Patient 1 received electron-beam treatment to a new area, the abdomen, 6 months after initial neck and shoulder treatment; patient 2 received additional photon treatment to his left forearm 16 months after initial treatment; and patient 3 received a repeat [jamanetwork.com]
(Also see Lichen Sclerosus) Treatments For UVA1 treatment, first contact your doctor to see if it they will recommend it for you. It is not used for lichen sclerosus that is on the genital area. [web.archive.org]
Treatment options include topical as well as systemic treatments, and physical modalities. There is a need for randomized controlled trials and studies on long-term outcomes after treatment. Key words: scleroedema adultorum Buschke; treatment. [medicaljournals.se]
Prognosis
Medical prognosis Spontaneous recovery is common, but the condition often lasts for several years. [bindevevssykdommer.no]
The practical importance of knowing scleredema adultorum rests on the fact that the disease is benign and self-limited but is easily mistaken for conditions which carry a poor prognosis and tend to be progressive. [jamanetwork.com]
Treatment and prognosis Phototherapy, physiotherapy, corticosteroids, antibiotics, intravenous immunoglobulin and tight glycemic control are the main treatment options. Partial remission of the disease is documented in most of the cases 6. [radiopaedia.org]
There are three forms of the condition which vary by disease course and long term outlook (prognosis). [rarediseases.org]
It may also be associated with monoclonal gammopathy, multiple myeloma, and thyroid dysregulation.[8] Prognosis The prognosis of the scleredema of Buschke highly depends on the subtype. [statpearls.com]
Etiology
NCI: A usually benign and self-limited skin disorder of unknown etiology, characterized by induration of the skin. [linkedlifedata.com]
A usually benign and self-limited skin disorder of unknown etiology, characterized by induration of the skin. It may be associated with infection, diabetes mellitus, and hematologic malignancies. [ebi.ac.uk]
A diffuse, non-pitting induration of the skin of unknown etiology that occurs most commonly in association with diabetes mellitus, predominantly in females. [profiles.uchicago.edu]
Engelsk definition A diffuse, non-pitting induration of the skin of unknown etiology that occurs most commonly in association with diabetes mellitus, predominantly in females. [mesh.kib.ki.se]
Epidemiology
Epidemiology of genital chlamydial infections in patients with chlamydial conjunctivitis: a retrospective study. Genitourinary medicine 1996; 72:203-205. Hammerschlag MR. Chlamydia trachomatis. In: Nelson textbook of paediatrics. 16th ed. [md-medicaldata.com]
[…] atrophicus and scleredema adultorum of Buschke had been reported, endorsing the theory that scleroderma-like disorders share common underlying pathogenesis.[5] Rare cases of scleredema of Buschke have carry associations with concomitant neoplasms.[6] Epidemiology [statpearls.com]
Pathophysiology
Scleredema, an uncommon condition with unknown pathophysiologic characteristics, is characterized by nonpitting induration of the skin with occasional erythema. Limited options are available to treat patients with scleredema diabeticorum. [jamanetwork.com]
Etiology Pathophysiology The pathogenesis of scleredema is poorly understood. [dermatologyadvisor.com]
Pathophysiology Scleredema of Buschke is a skin connective tissue disease of unknown pathogenesis. [statpearls.com]
Prevention
Keeping blood sugar levels within range helps prevent diabetes complications. [ucsfbenioffchildrens.org]
Patients with scleredema have to be screened for internal organ involvement to prevent mortality. Kavitha Mohanasundaram, Subramaniyan Kumarasamy, Ramesh Kumar, Chandran P. [cyberleninka.org]
Though the benign nature of the disease is the rule in most cases, the systemic involvement has to be looked for and appropriately treated to prevent morbidity and mortality in scleredema adultorum of Buschke. [ijdvl.com]
Rapidly find the answers you need with separate sections on diseases and disorders, differential diagnosis, clinical algorithms, laboratory results, and clinical preventive services, plus an at-a-glance format that uses cross-references, outlines, bullets [books.google.com]