Primary Systemic Amyloidosis Presenting as Extensive Cutaneous Ulceration

Al-Haddab, Mohammed; Srolovitz, Herbert; Rosen, Nathan

doi:10.2310/7750.2006.00046

Cited by 10 publications

(8 citation statements)

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“…Infiltrated, waxy papules and plaques with hemorrhage, macroglossia, blistering lesions resembling those of porphyria cutanea tarda or epidermolysis bullosa acquisita are also common signs 18,19 . Alopecia, nail dystrophy, scleroderma‐like thickening, acquired digital cutis laxa‐like changes and extensive cutaneous ulceration may also occur 20–22 . Average survival after diagnosis of primary and myeloma‐associated amyloidosis is poor (13.2 months) 23…”

Section: Disorders Because Of Deposition Of M‐proteinmentioning

confidence: 99%

“…18,19 Alopecia, nail dystrophy, scleroderma-like thickening, acquired digital cutis laxa-like changes and extensive cutaneous ulceration may also occur. [20][21][22] Average survival after diagnosis of primary and myeloma-associated amyloidosis is poor (13.2 months). 23 Histologically, amyloid deposits consisting of immunoglobulin light chains (AL-fibril type) are present in the dermis and surrounding blood vessels walls, adnexal structures, and individual lipocytes (amyloid rings).…”

Section: Disorders Because Of Deposition Of M-proteinmentioning

confidence: 99%

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The histological and pathogenetic spectrum of cutaneous disease in monoclonal gammopathies

2008

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The dermatological disorders associated with monoclonal gammopathies are clinically heterogeneous and may be divided into four groups with distinctive pathogenetic mechanisms (a) specific (infiltrative) disorders including primary and secondary cutaneous plasmacytoma and cutaneous lymphoplasmacytic infiltration of Waldenström's disease (b) skin disorders because of the deposition of monoclonal immunoglobulin (M protein), including amyloidoisis macroglobulinemia cutis, light chain deposition of Randall's disease, follicular spicules of the nose, and cryoglobulinemia (c) skin disorders associated with monoclonal gammopathies, including highly associated (>50%), weakly associated (<50%) or anecdotal and (d) miscellaneous (non specific). In most cases, histopathology is crucial to confirm or to diagnose those skin conditions and is also very useful to understand their pathogenetic mechanisms.

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Section: Disorders Because Of Deposition Of M‐proteinmentioning

confidence: 99%

Section: Disorders Because Of Deposition Of M-proteinmentioning

confidence: 99%

The histological and pathogenetic spectrum of cutaneous disease in monoclonal gammopathies

2008

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“…Skin involvement takes the form of papules, nodules, and plaques. Cutaneous amyloidosis can be a risk factor for non-healing ulcer in elderly persons [4].…”

Section: Introductionmentioning

confidence: 99%

New advances in renal amyloidosis

et al. 2008

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Renal amyloidosis is a rare and intractable disease that accounts for 0.2% of the original kidney diseases of dialysis patients in Japan. However, the number of patients with renal amyloidosis seems to be increasing in recent years. There have been some new concepts focusing on the mechanism of amyloidogenesis, such as molecular chaperones, seeding mechanism, and genetic polymorphisms of precursor protein. Clinical and histological features of renal amyloidosis vary according to the type. Significantly higher levels of urinary protein excretion are seen in the AL type, whereas microscopic haematuria is more prominent in the AA type. Histologically, amyloid deposition of AL type has stronger predilection for GBM than mesangium, and spicule formation is more frequently observed. In contrast, AA type has a higher affinity to TBM and interstitial area. For the histological diagnosis of renal amyloidosis, plural staining methods including Congo-red, Daylon and thioflavin-T stains are available. Combinations of these staining methods are necessary for establishing the precise diagnosis. The more recent and intensive treatments for renal amyloidosis are expected to improve patient outcome. For AL amyloidosis, high-dose melphalan plus high-dose dexamethasone or VAD, in conjunction with bone marrow stem cells transplantation, have shown a definitive effect on reducing urinary protein excretion. The biological agent, tumor necrosis factor (TNF alpha) blocker, improves the renal function in AA-type renal amyloidosis, as well as suppresses the inflammatory reactions in patients with rheumatoid arthritis. Clinical advances have been made in various aspects of renal amyloidosis.

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“…Skin involvement takes the form of papules, nodules, and plaques. Cutaneous amyloidosis can be a risk factor for non-healing ulcer in elderly persons [6]. The kidney is a major target organ for systemic amyloidosis that often affects the kidney including proteinura, and elevated serum creatinine (Cr).…”

Section: Introductionmentioning

confidence: 99%

Significant association between renal function and amyloid-positive area in renal biopsy specimens in AL amyloidosis

et al. 2012

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BackgroundThe kidney is a major target organ for systemic amyloidosis that often affects the kidney including proteinura, and elevated serum creatinine (Cr). The correlation between amount of amyloid deposits and clinical parameters is not known. The aim of this study was to clarify correlation the amyloid area in all renal biopsy specimen and clinical parameters.MethodsFifty-eight patients with an established diagnosis of AL amyloidosis participated in the study. All patients showed amyloid deposits in renal biopsies. We retrospectively investigated the correlation between clinical data and amyloid occupied area in whole renal biopsy specimens.ResultsThe area occupied by amyloid was less than 10% in 57 of the 58 patients, and was under 2% in 40. For statistical analyses, %amyloid-positive areas were transformed to common logarithmic values (Log10%amyloid). Cr showed significant correlation with Log10%amyloid and estimated glomerular filtration rate (eGFR) showed the significant negative correlation. Patient age, cleatinine clearance (Ccr), blood urea nitorogen, and urinary protein was not significantly correlated with Log10%amyloid. The correlation with other clinical factors such as sex, and serum concentrations of total protein, albumin, immunoglobulins, compliments was evaluated. None of these factors significantly correlated with Log10%amyloid. According to sex- and age- adjusted multiple linear regression analysis, Log10%amyloid had significant positive association with Cr and significant negative association with eGFR.ConclusionThere is significant association between amyloid-positive area in renal tissue and renal function, especially Cr and eGFR. The level of Cr and eGFR may be a marker of amount of amyloid in renal tissue.

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Primary Systemic Amyloidosis Presenting as Extensive Cutaneous Ulceration

Abstract: This presentation of the disease may signify an advanced stage with a grave prognosis as our patient passed away 3 months after development of the cutaneous ulceration.

Cited by 10 publications

References 10 publications

The histological and pathogenetic spectrum of cutaneous disease in monoclonal gammopathies

The histological and pathogenetic spectrum of cutaneous disease in monoclonal gammopathies

New advances in renal amyloidosis

Significant association between renal function and amyloid-positive area in renal biopsy specimens in AL amyloidosis

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