Case Number : Case 818 - 6th August Posted By: Guest

scleredema of Buschke

[i]Agree[/i]
[i]Thickening of dermis with increased dermal mucin (appears to be more in deeper dermis), consistent with Scleredema.[/i]

Increased fibroblast, mucin and fibrosis ends in a diagnosis of Sclermexedema which is associated with paraproteinemia.Patients usually have cardiac , neurologic and hematological complications like this patient who has lymphoma.

[font="Palatino Linotype, serif"][size="4"]Non-inflammatory dermal thickening characterized by morpheaform entrapment of eccrine sweat glands and fenestrations between sclerotic collagen bundles filled with abundant mucin deposits, highlighted by colloidal iron, in the absence of fibroblastic proliferation.[/size][/font]
[font="Palatino Linotype, serif"][size="4"]Given the case history, I would call this monoclonal gammopathy-associated scleredema.[/size][/font]

Scleromyxedema

Scleromyxedema should appear with increased fibroblasts and It also does not affects the tongue or the esophageal musculature, which could explain the dysphagic symptom. I don´t see increased fibroblasts.
I think this is a Scleredema of Buschke with its increased dermal thickness and mucin, no or few fibroblast proliferation and no inflammatory cells. Scleredema can also appear with dysphagic symptoms and can be secondary to malignant lymphoid diseases.

[color=#1C2837][font=arial, verdana, tahoma, sans-serif][size=4]Scleredema[/size][/font][/color]

Scleroedema - Thickened dermis,replacement of fat by coarse collagen bundles and mucin.I couldn`t see any fibroblast proliferation - are you supposed to see this in scleroedema?

I do not perceive considerable fibroblast proliferation, so I also favor this to be scleredema.. Could be paraneoplastic.

[quote name='Dr. Mona Abdel Halim' timestamp='1375793544']
I do not perceive considerable fibroblast proliferation, so I also favor this to be scleredema.. Could be paraneoplastic.
[/quote]
agree

Scleredema.

The biopsy shows thickening of the reticular dermis (with illusion of an upwards displacement of eccrine coils from deep to mid-reticular dermis) and increased dermal mucin. No significant fibroblast increase is seen, which would be expected for the differential dx consideration of scleromyxedema.

I agree that the history of non-Hodgkin's lymphoma (with presumed absent history of infection or diabetes) and long history of sclerodermoid symptoms would favor monoclonal gammopathy as the likely cause of scleredema. Dysphagia can be seen in both scleredema and scleromyxedema.

Frequently, scleredema will present several years (median 2.5 years) before a monoclonal gammopathy can be detected by serum protein electrophoresis (SPEP) testing. A negative SPEP test therefore does not exclude gammopathy-related scleredema. (This delay may not apply to patients with previous lymphoma diagnosis).
Did this patient have a positive SPEP?

I agree with most of my colleagues: scleredema.