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Scleredema Adultorum


Presentation

  • 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]
  • TY - JOUR T1 - Scleredema adultorum of Buschke presenting as periorbital edema: a diagnostic challenge.[wwww.unboundmedicine.com]
  • 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.[sclero.org]
Fatigue
  • 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]
  • However, the patient treated with mixed photon therapy developed sore throat, nausea, fatigue, and headache during treatment.[appliedradiationoncology.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]
Odynophagia
  • No dysphasia or odynophagia nor constipation. No chest pain, palpitation or dyspnea.[shmabstracts.com]
Gagging
  • Cell culture studies revealed that fibroblasts from the involved skin produced more glycosaminoglycans (GAG) than uninvolved skin and most of the GAG produced was hyaluronic acid.[ncbi.nlm.nih.gov]
Palpitations
  • No chest pain, palpitation or dyspnea. Examination revealed right hemiparesis non pitting, thickened, indurated, hard skin over the posterior neck, upper back and shoulder, chest and over the legs bilaterally with limited range of motion.[shmabstracts.com]
Chest Pain
  • No chest pain, palpitation or dyspnea. Examination revealed right hemiparesis non pitting, thickened, indurated, hard skin over the posterior neck, upper back and shoulder, chest and over the legs bilaterally with limited range of motion.[shmabstracts.com]
Delayed Wound Healing
  • Although it may be a benign, self-limited disease, its complications may require drainage of abscesses or soft-tissue coverage, which may result in delayed wound healing.[ncbi.nlm.nih.gov]
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]
Proximal Muscle Weakness
  • No clinical evidence of proximal muscle weakness. With this clinical presentation, a provisional diagnosis of scleredema was made.[ijdvl.com]
Frozen Shoulder
  • This specialist diagnosed a diabetes-related frozen shoulder and prescribed local corticosteroid injection followed by physiotherapy.[em-consulte.com]
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]
Neglect
  • It is worthy to note that most of the cases in this series were previously unrecognised or neglected by MP or diabetologists.[em-consulte.com]
Dysphasia
  • No dysphasia or odynophagia nor constipation. No chest pain, palpitation or dyspnea.[shmabstracts.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.[clinicaladvisor.com]
Microalbuminuria
  • Renal function was normal (microalbuminuria: 5 mg/l, plasma creatinine: 85 µmol/l). Blood pressure fluctuated and was found elevated (150/90 mm Hg) in some occasions. Echocardiography, ECG and chest X-ray were normal.[em-consulte.com]
Normal Electrocardiogram
  • Serum protein electrophoresis was normal. Electrocardiogram, echocardiogram, chest X-Ray and HRCT of chest were normal. Anti nuclear antibodies, anti-topoisomerase-1, anticentromere antibodies and anti U 1 RNP were negative.[ijdvl.com]
  • Serum protein electrophoresis was normal. Electrocardiogram, echocardiogram, chest X-ray and HRCT of chest were normal.[cyberleninka.org]

Treatment

  • […] 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.[sclero.org]

Prognosis

  • Conclusions: Awareness of this disease is very important as its treatment, prognosis differs significantly with scleroderma, a more common but similar medical condition. To cite this abstract: 2Ugbarugba E, Aninyei M.[shmabstracts.com]
  • […] steroids, cyclosporine, colchicine, low-dose methotrexate, high-dose penicillin, electron beam therapy, extracorporeal photopheresis, psoralen followed by ultraviolet A Psoralen followed by ultra violet A (PUVA) and intravenous immunoglobulins[ 5 ] Prognosis[ncbi.nlm.nih.gov]
  • […] intralesional steroids, cyclosporine, colchicine, low-dose methotrexate, high-dose penicillin, electron beam therapy, extracorporeal photopheresis, psoralen followed by ultraviolet A Psoralen followed by ultra violet A (PUVA) and intravenous immunoglobulins [5] Prognosis[idoj.in]
  • The possibility of systemic involvement makes early diagnosis critical for proper treatment and better prognosis. REFERENCES 1 Gervini RL, Lecompte SM, Pineda RCB, Ruthner FG, Magnabosco EM, Silva LMC. Escleredema de Buschke: relato de dois casos.[scielo.br]

Etiology

  • Its etiology is unknown, but there may be a preceding history of infection and there is a known association with diabetes mellitus. Women are more frequently affected. Usually, the disease is self-limiting but some patients show progressive disease.[ncbi.nlm.nih.gov]
  • Scleredema adultorum of Buschke characterized by thickening and hardening of the skin is an uncommon sclerotic disorder of unknown etiology. Scleredema adultorum of Buschke is an uncommon disorder of unknown origin.[freethesaurus.com]

Epidemiology

  • Their role in epidemiological studies of trachoma and its control. Br J Ophthalmol 1974; 58:438-454. Band D, Regan L, Greub G. Comparsion of five commercial serological tests for the detection of anti- Chlamydia trachomatis antibodies.[md-medicaldata.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]
  • Based on the epidemiological data, Ballantyne, a British physician, argued erroneously that SN and ‘oedema neonatorum’ were the same disease.[nature.com]
  • […] skin and subcutaneous tissues leading to sclerosis. [4] The absence of features such as Raynaud's phenomenon, sclerodactyly, nail bed capillary abnormalities and involvement of the internal organs differentiates localized from systemic scleroderma. [4] Epidemiology[indianjrheumatol.com]
Sex distribution
Age distribution

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.[clinicaladvisor.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

  • 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]
  • Patients with scleredema have to be screened for internal organ involvement to prevent mortality. Kavitha Mohanasundaram, Subramaniyan Kumarasamy, Ramesh Kumar, Chandran P.[cyberleninka.org]
  • Physical therapy is recommended to prevent limitations in range of motion. However, no trials have been performed to evaluate the benefit of physical therapy. References 1. Venencie PY, et al. Scleredema: review of 33 cases.[dermatology.cdlib.org]
  • The goals of the Registry are to promote the search for basic defects, improve methods of diagnosis, and develop effective methods of treatment and/or prevention of these disorders.[kundoc.com]

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