Scleredema Buschke, scleredema adultorum 3.75/5 (4)

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Definition

Sclerotic edema is a rare condition of unknown cause, but often associated with it d, Streptococcal infection (in the throat) or changes in the blood in the form of Monoclonal gammopathy. Huden blir hard og stram i typiske områder på overkropp og nakke, men ikke i hender. Sykdomsmekanismene er ikke godt klarlagt. Sklerødem klassifiseres som en scleroderma-like disease, but is not counted among them inflammatory systemic connective tissue diseases. However, the symptoms mean that the rheumatologist should know the condition and the closest differential diagnoses. The disease was first described by Curizo in 1752 and further defined by Abraham Buschke in 1902.

Occurrence

Scleral edema is defined as one rare disease, but the exact occurrence is unknown. The disease most commonly occurs in middle-aged, overweight people with diabetes.

Symptoms

Gradually the skin and subcutaneous tissue become hard, especially over the neck and back. The front of the chest can also be attacked, but usually not arms and legs.

Based on classification according to Graff in 1968, scleroedema can be divided into three groups

  • Type 1 is the largest group (55%). Children and young adults are most commonly attacked. The onset of the disease is relatively quick 2-3 weeks after a streptococcal throat infection, less often other bacterial or viral infections (reference: Shrestha B, 2014).
  • Type 2 (25%) has a gradual and slow onset and progression and correlates with blood disease occurring in the course. Haematological disease includes "monoclonal gammopathy of uncertain significance" (MUGS) with high IgG or IgA, multiple myeloma or amyloidosis (reference: Rongioletti F, 2015).
  • Type 3 is also called diabetic scleroedema, since it is patients with diabetes mellitus who are attacked. Type 2 accounts for 20% of scleroedema cases. Predisposing factors are long-term, poorly regulated diabetes and diabetic vasculopathy. A possible explanation for the disease is that high glucose levels cause non-enzymatic glycolysis of collagen in the skin (reference: Murphy-Chutorian B, 2013).

Examinations

Medical history dekker symptomer og etterspør disponerende tilstander (se ovenfor). Raynauds fenomen er uvanlig.

Clinical skin changes are assessed, especially on the upper body and neck. Changes in hands are not expected.

Blood tests: Ingen spesielle utslag forventes, men CK (kreatin kinase fra muskler) er pfte forhøyet. Fastende blodsukker, anti-streptolysin (AST) og elektroforese for å påvise eventuelle assosierte sykdommer. ANA (antistoff) og leukocytter med differensial-telling (eosinofile) undersøkes for å vurdere differensialdiagnoser (Systemic sclerosis, Eosinophil fasciitis).

Tissue sample is not needed to confirm the diagnosis, but can rule out other scleroderma conditions. Marked thickening of collagen bundles separated by prominent clear, mucin-filled spaces is typical. Subcutaneous fat shows invasion of thickened collagen fibers. No pathology in the epidermis, sweat or sebaceous glands is expected. Mucin depots that can be stained with Alcian blue or toluidine are most often available (reference: Naschel j, 2012).

Diagnosis

The disease picture with hard skin, medical history with a predisposition to disease (diabetes, infection or Monoclonal gammopathy).

Similar conditions, differential diagnoses

  • Systemic sclerosis: Raynaud's phenomenon, harde fingre, ANA with subgroups (CENP, Scl-70) i blodet.
  • Eosinophilic fasciitis: Arms and legs are attacked, affected skin often warm and reddish (in active phase). High eosinophilic white blood cells (in blood sample and in tissue sample)
  • Generalized morfea. Like morphea with plaques, but spread (generalized) over different skin areas.
  • Sclero- myxoedema. (Papulær mucinose); 2-4mm store papler, til dels med flat overflate. Inneholder mucin, ikke puss. Grupper og lineær utbredelse over dorsalside av hender, ansikt, albuer og ekstensor-sider er typisk. Vevsprøve viser fibroblast proliferasjon i huden.
  • “Stiff skin syndrom”: Starter ofte i barne- og ungdomsårene. Ingen organaffeksjon. Histologi viser ingen inflammasjon. (Guiducci  S Rheumatology 2009)
  • Nephrogenic systemic fibrosis. After an MRI examination with contrast agent when kidney failure is present

Treatment

If predisposing diseases are present, these must be treated as best as possible. If significant stiffness, physiotherapy may be useful to counteract more stiffness (reference: Mickel M, 2017). Light therapy with UVA1 can be tried (reference: Knobler R, 2017), methotrexate or immunoglobulins (IVIG) are tested in some individuals (reference: Eastham AB, 2014).

Prognosis

Spontaneous recovery is common, but the condition often lasts for several years. It is unusual for internal organs to be attacked. Infection-related (streptococcal) type 1 has the best prognosis (reference: Kreuter A, 2018).

 Literature


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