Scleromyxoedema-like changes in four renal dialysis patients

Abstract

We describe four renal dialysis patients from our hospital who, over a 6-month period, developed erythematous, thickened, indurated dermal plaques. The plaques were limited to the limbs and in three patients there were associated flexion contractures. The clinical features most resembled scleromyxoedema. All patients had previously received at least one renal transplant. Histopathology of the plaques showed features of scleromyxoedema in two patients, whereas the other two showed a different picture, more suggestive of a morphoea-like process. There are important differences between our patients and classical scleromyxoedema. All four patients had normal immunoglobulins and no paraprotein was detected. Almost all cases of classical scleromyxoedema are associated with an IgGlambda paraproteinaemia. We have not yet identified an underlying cause for this cluster of cases in our hospital. It is possible that the skin changes seen may have been precipitated by an environmental agent, such as in 'toxic oil syndrome' and vinyl chloride-induced scleroderma. We discuss the differences between our patients and those with scleromyxoedema, localized or generalized morphoea and environmentally induced scleroderma. We feel that our patients show a constellation of features similar, but not identical, to scleromyxoedema. There has been only one previous report of similar patients. We believe this to be a new and distinct phenomenon.

Nephrogenic systemic fibrosis: Clinical spectrum of disease

“…1a,b) (2,(34)(35)(36)(37)(38)(39). In our experience, underscored by many published reports, contracture of the joints may become severe enough to make ambulation impossible (2,(34)(35)(36)(37)(38). The patients become not only dependent on a wheelchair but also disabled and unable to perform normal daily activities.…”

“…Initially, the lesions tend to affect the distal parts and progress to the proximal parts of the extremities and the trunk during the course of the disease. In general, the trunk is less commonly involved, the face and neck normally spared (2,(34)(35)(36)(37)(38). However, there are sporadic reports of patients with involvement of the face (5,38).…”

“…In contrast, a report of four patients with assumed NSF published in 2002 were reassessed 2007. All four patients, including a young female with cardiac involvement (discussed above), had received Gd-CA prior to disease onset (35,49).…”

Dermopatía fibrosante nefrogénica: Reporte de dos casos

“…Los inhibidores de la calcineurina han sido implicados en la iniciación de la fibrosis de los tejidos, dado su capacidad de inducir altos niveles de una citoquina profibrótica, el TGFß 1 (transforming growth factor ß 1 ) 21,22 .…”

“…Es importante hacer notar que aquellos pacientes que tuvieron una mejoría moderada o importante, son pacientes que discontinuaron la diálisis, dado que su función renal había mejorado 10,21,23 . En algunos pacientes, el uso de interferón o de inmunoglobulinas endovenosas ha mostrado ser parcialmente efectivo, sin embargo, en la mayoría de los casos los efectos son sólo momentá-neos 21,32 .…”