Primary Cutaneous Localized Amyloid Elastosis

Santos‐Briz, Ángel; Cañueto, Javier; Antunez, Patricia; Bravo, Javier; Unamuno, P.

doi:10.1097/dad.0b013e3181b1c62f

Cited by 9 publications

(8 citation statements)

References 18 publications

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“…2,3,6 The previously reported patient with localized amyloidosis remained without evidence of systemic or residual involvement 1 year after diagnosis. 7 Our patient, the only other reported patient with localized involvement, remains well without evidence of systemic involvement 9 months after diagnosis.…”

Section: Discussionmentioning

confidence: 51%

“…5,6 However, in a single previously reported case, additional workup failed to identify systemic involvement, and this case was reported as primary cutaneous localized amyloidosis. 7 Of note, in situ hybridization (ISH) studies for kappa and lambda light chains performed on the biopsy material demonstrated a kapparestricted plasma cell population, indirectly suggesting that the amyloid protein may have been light chain-derived. Cutaneous deposits of AL-amyloid without systemic involvement, which have been termed AL-amyloidomas, are considered by some authors to represent an unusual manifestation of primary cutaneous marginal zone lymphoma (pcMZL) when accompanied by a variably dense lymphoplasmacytic infiltrate demonstrating evidence of light-chain restriction.…”

Section: Introductionmentioning

confidence: 99%

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A Second Case of Localized Cutaneous Amyloid Elastosis: Expanding the Discussion of Associations With a Unique Histopathologic Entity

Roberts

Dittmer

Vidal

et al. 2021

The American Journal of Dermatopathology

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Amyloid elastosis is an exceedingly rare form of amyloidosis characterized by amyloid material deposited on dermal elastic fibers. Most reported cases have been associated with systemic amyloid light-chain amyloidosis. A single previously reported case of amyloid elastosis showed evidence that the amyloid material was derived from light-chain proteins and was associated with a monoclonal plasma cell infiltrate but failed to demonstrate systemic involvement. As a result, the case was felt to represent localized cutaneous amyloid elastosis. We present a case of localized cutaneous amyloid elastosis that is not associated with a definitive monotypic plasma cell population or with systemic amyloidosis. We also review the clinical and histopathologic features of reported cases of amyloid elastosis and discuss possible etiologic considerations. Because amyloid elastosis can be either localized to the skin or associated with systemic involvement, additional workup to exclude an underlying plasma cell dyscrasia or hematologic malignancy is warranted.

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Section: Discussionmentioning

confidence: 51%

Section: Introductionmentioning

confidence: 99%

A Second Case of Localized Cutaneous Amyloid Elastosis: Expanding the Discussion of Associations With a Unique Histopathologic Entity

Roberts

Dittmer

Vidal

et al. 2021

The American Journal of Dermatopathology

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“…4 The clinical course is sometimes dramatic requiring an aggressive approach: 3 cases ended in death by multiorgan involvement [1][2][3] and one case was successfully treated with hematopoietic stem cell transplantation. 10 In 2010, Santos-Briz et al 11 reported the first case of primary cutaneous localized amyloid elastosis and, to the best of our knowledge, ours is the second to be reported. In contrast to amyloid elastosis associated with systemic disease, in both Santos-Briz's case and in ours, lesions were nonitchy, localized, and showed anetodermal features; furthermore, the clinical course was remarkably indolent, with no associated systemic symptoms.…”

Section: Discussionmentioning

confidence: 57%

Cutaneous Nodules and Violaceous Patches on the Legs: Answer

Ortins-Pina

Zumdick²,

Weiss

et al. 2019

The American Journal of Dermatopathology

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“…All of these cases suggest that the interaction between immunoglobulin light chains and elastic fibres is unique, but the molecular explanation remains to be determined (7). The reason why amyloid elastosis is restricted to a small subpopulation of patients with gammopathy of any severity also remains to be determined.…”

Section: Discussionmentioning

confidence: 99%

Cutaneous Amyloid Elastosis Revealing Multiple Myeloma with Systemic Amyloidosis

Marchand¹,

Levaltier²,

Croué³

et al. 2013

Acta Derm Venerol

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Cutaneous amyloid elastosis is a rare presentation of amyloidosis. Only 5 cases have been reported since 1985; 4 in the setting of various presentations of systemic amyloidosis and 1 primary cutaneous localized amyloid elastosis. We report here an additional case, initially misdiagnosed as pseudoxanthoma elasticum (PXE). CASE REPORTA 69-year-old woman was referred for fatigue, dyspnoea on exertion and skin changes. Five years earlier, papules on several aspects of the neck had appeared suddenly (over a single night according to the patient), and had quickly extended following oedematous episodes. The patient's medical history included myocardial infarction, hypertension and concentric hypertrophic cardiomyopathy, osteoporosis, hypothyroidism and depression. Dermatological examination revealed diffuse but subtle, whitish or skin-coloured papules, located mainly in cervical, flexural, periumbilical, abdominal and lumbar areas, but also on the gingiva and the inner aspect of the lower lip. Debilitating pruritus was present, as well as hand paraesthesia and vague aches. There was no macroglossia. Cardiac auscultation revealed a systolic murmur (3/6) related to aortic valve stenosis. On echocardiography, septal hypertrophy with a small pericardial effusion was present. Laboratory findings showed normochromic, normocytic, aregenerative anaemia (haemoglobin 10.9 g/dl), but no leucopaenia or thrombopaenia. Renal and hepatic functions and serum calcium level were normal. There was moderate inflammatory syndrome. The beta-2-microglobulin level was high (5.25 g/l). Massive nonselective proteinuria (6-8 g/24 h) was also present. Serum protein electrophoresis revealed a high peak in gammaglobulins (15.7 g/l) with monoclonal immunoglobulin (Ig)G-λ gammopathy. Residual IgA, IgM and albumin levels were decreased. Free λ light chains were present in the urine. Brain natriuretic peptide was high (971 pg/ml -n < 100). The diagnosis of multiple myeloma was established after demonstration of 20% bone marrow infiltration with plasma cells displaying a hyperdiploid karyotype. There were no lytic bone lesions.A skin biopsy was performed, and haematoxylin and eosin (H&E) and orcein staining revealed an accumulation of dystrophic basophilic elastic fibres in the dermis. Some elastic fibres were fragmented, showing a striking "fishbone" appearance at high magnification (Fig. 1). Congo red staining was negative. Electron microscopy of a skin sample revealed an abundant microfibrillar material highly suggestive of amyloid fibrils that coated the oxytalan and elaunin fibres in the papillar dermis and the mature elastic fibres in the mid-dermis (Fig. 2). Renal biopsy demonstrated amyloidosis with amorphous deposits within tubules and arterioles. Congo red staining was positive in the kidney. The patient was treated with melphalan, dexamethasone and bortezomib. After the third injection of bortezomib, the patient developed left-side heart failure, presumably due to cardiac amyloidosis. Treatment was suspended until the heart function improved ...

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Primary Cutaneous Localized Amyloid Elastosis

Cited by 9 publications

References 18 publications

A Second Case of Localized Cutaneous Amyloid Elastosis: Expanding the Discussion of Associations With a Unique Histopathologic Entity

A Second Case of Localized Cutaneous Amyloid Elastosis: Expanding the Discussion of Associations With a Unique Histopathologic Entity

Cutaneous Nodules and Violaceous Patches on the Legs: Answer

Cutaneous Amyloid Elastosis Revealing Multiple Myeloma with Systemic Amyloidosis

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