Primary localized cutaneous amyloidosis: a sign of immune dysregulation?

Dahdah, Maurice J.; Kurban, Mazen; Kibbi, Abdul‐Ghani; Ghosn, Samer

doi:10.1111/j.1365-4632.2009.03799.x

Cited by 32 publications

(24 citation statements)

References 14 publications

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“…If amyloid deposits are identified, it is important to determine whether the amyloidosis is localized or systemic. Typical sites for localized amyloidosis include the skin , larynx , or urinary tract , which can include the renal pelvis, ureter, bladder, and urethra. Pulmonary nodules demonstrating amyloid are frequently localized deposits composed of light chains or transthyretin .…”

Section: Disease Overviewmentioning

confidence: 99%

Immunoglobulin light chain amyloidosis: 2014 update on diagnosis, prognosis, and treatment

Gertz

2014

American J Hematol

129

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Disease Overview: Immunoglobulin light chain amyloidosis is a clonal, nonproliferative plasma cell disorder in which fragments of immunoglobulin light chain are deposited in tissues. Clinical features depend on organs involved but can include restrictive cardiomyopathy, nephrotic syndrome, hepatic failure, peripheral/autonomic neuropathy, and atypical multiple myeloma. Diagnosis: Tissue biopsy stained with Congo red demonstrating amyloid deposits with apple‐green birefringence is required for diagnosis. Invasive organ biopsy is not required because amyloid deposits can be found in bone marrow biopsy or subcutaneous fat aspirate in 85% of patients. Verification that amyloid is composed of immunoglobulin light chains is mandatory. Prognosis: N‐terminal pro‐brain natriuretic peptide (NT‐proBNP), serum troponin T, and difference between involved and uninvolved immunoglobulin free light chain values are used to classify patients into four groups of similar size; median survivals are 94.1, 40.3, 14.0, and 5.8 months. Therapy: All patients with a systemic amyloid syndrome require therapy to prevent deposition of amyloid in other organs and prevent progressive organ failure of involved sites. Stem cell transplant (SCT) is preferred, but only 20% of patients are eligible. Requirements for safe SCT include NT‐proBNP <5,000 ng/mL, troponin T <0.06 ng/mL, age <70 years, <3 organs involved, and serum creatinine ≤1.7 mg/dL. Nontransplant candidates can be offered melphalan–dexamethasone or cyclophosphamide–bortezomib–dexamethasone. Other combinations of chemotherapy with agents such as cyclophosphamide–thalidomide (or lenalidomide)–dexamethasone, bortezomib–dexamethasone, and melphalan–prednisone–lenalidomide have documented activity. Future Challenges: Late diagnosis remains a major obstacle to initiating effective therapy. Recognizing the presenting syndromes is necessary for improving survival.Am. J. Hematol. 89:1133–1140, 2014. © 2014 Wiley Periodicals, Inc.

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Section: Disease Overviewmentioning

confidence: 99%

Immunoglobulin light chain amyloidosis: 2014 update on diagnosis, prognosis, and treatment

Gertz

2014

American J Hematol

129

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“…On the basis of a patient of PLCA having sarcoidosis and IgA nephropathy and increasing number of reports suggesting immune mediated factors, the possibility of autoimmune basis in a group of patients with extensive disease was also proposed. [18] Yet, its etiology still remains an enigma.…”

Section: Introductionmentioning

confidence: 99%

A clinico-epidemiological study of macular amyloidosis from North India

2012

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Background:Macular amyloidosis (MA) is the most subtle form of cutaneous amyloidosis, characterized by brownish macules in a rippled pattern, distributed predominantly over the trunk and extremities. MA has a high incidence in Asia, Middle East, and South America. Its etiology has yet to be fully elucidated though various risk factors such as sex, race, genetic predisposition, exposure to sunlight, atopy and friction and even auto-immunity have been implicated.Aim:This study attempts to evaluate the epidemiology and risk factors in the etiology of MA.Materials and: Methods:Clinical history and risk factors of 50 patients with a clinical diagnosis of MA were evaluated. Skin biopsies of 26 randomly selected patients were studied for the deposition of amyloid.Results:We observed a characteristic female preponderance (88%) with a female to male ratio of 7.3:1, with a mean age of onset of MA being earlier in females. Upper back was involved in 80% of patients and sun-exposed sites were involved in 64% cases. Incidence of MA was high in patients with skin phototype III. Role of friction was inconclusiveConclusion:Lack of clear-cut etiological factors makes it difficult to suggest a reasonable therapeutic modality. Histopathology is not specific and amyloid deposits can be demonstrated only in a small number of patients. For want of the requisite information on the natural course and definitive etiology, the disease MA remains an enigma and a source of concern for the suffering patients.

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“…[4] Dahdah et al . [5] also reported a case of co-existing macular form of PLCA and sarcoidosis. However, to our knowledge, this report is the first description of concomitant appearance of NPLCA and PS.…”

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confidence: 99%

“…We cannot state with certainty whether the occurrence of these two diseases was associated or independent, but Dahdah et al . [5] suggested that a subset of PLCA patients, especially those with extensive or atypical involvement, may have associated immune disorders. In our case, NPLCA and PS have been stable without subjective symptoms.…”

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confidence: 99%