Early Dermoscopic Signs Leading to Primary Systemic Amyloidosis’ Diagnosis: A Case Report

Hassanandani, Trashita; Singh, Bratati; Kar, Bikash Ranjan

doi:10.5826/dpc.1103a23

Cited by 2 publications

(1 citation statement)

References 2 publications

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“…The cutaneous manifestations in PSA depend on the site of amyloid deposition [11]. The most common clinical cutaneous presentations of PSA are hemorrhagic lesions, which are caused by the deposition of amyloid in the walls of dermal blood vessels, leading to vessel fragility [12]. Similarly, amyloid deposits in cutaneous appendages such as hair follicles can result in the destruction of their structures and functions, ultimately leading to the formation of comedo-like openings and milia-like cysts, which are manifested as keratinous cysts in histopathology.…”

Section: Discussionmentioning

confidence: 99%

Primary Systemic Amyloidosis Characterized by Multiple Keratinous Cysts: A Case Report

Xie

et al. 2023

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Primary systemic amyloidosis (PSA) is a systemic disease caused by amyloid deposition in various tissues and organs. In the early stages of the disease, approximately 40% of patients have skin damage, which may be the only clinical manifestation. This report described the case of a 67-year-old male with PSA who presented with characteristic cutaneous manifestations. Physical examination showed seborrheic keratotic-like plaques around the orbit, purpura and ecchymosis on the neck and dorsum manus, as well as nail dystrophy. An auxiliary examination indicated a 24-h urine protein content of 2.89 g, and serum immunoelectrophoresis showed a monoclonal λ light chain. Echocardiography showed decreased left ventricular diastolic function and mild pulmonary hypertension. The dermoscopic features were multiple comedo-like openings and milia-like cysts. Histopathology showed multiple keratinous cysts in the dermis and eosinophilic, acellular, homogenous material in the dermis that was Congo red-positive. The diagnosis was confirmed through biopsy of skin lesions and kidney.Skin lesions of this case are rare.Skin lesions around the orbit need to arouse the vigilance of dermatologists. PSA should be considered if the patient with multisystemic symptoms.Skin biopsy and Congo red staining were very important for early diagnosis of PSA.

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Section: Discussionmentioning

confidence: 99%

Primary Systemic Amyloidosis Characterized by Multiple Keratinous Cysts: A Case Report

Xie

et al. 2023

Preprint

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Primary Systemic Amyloidosis Characterized by Multiple Keratinous Cysts: A Case Report

Lu,

Fu,

Xie

et al. 2024

J Cutan Pathol

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A 67‐year‐old male presented with plaques around the orbit and ecchymosis on the neck and back of hands for 2 years. Physical examination showed seborrheic‐keratosis‐like plaques around the orbit, ecchymosis on the neck and back of hands, as well as nail dystrophy. Serum λ light chain was positive. Histopathological examination demonstrated multiple keratinous cysts in the dermis and eosinophilic homogeneous material in the dermis, which was positive for Congo red with apple‐green birefringence on polarized light. Kidney biopsy showed deposition of acellular eosinophilic material in the arteriole wall. Congo red staining was positive. A bone marrow biopsy revealed multiple myeloma. The diagnosis of primary systemic amyloidosis with multiple myeloma was made.

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Early Dermoscopic Signs Leading to Primary Systemic Amyloidosis’ Diagnosis: A Case Report

Cited by 2 publications

References 2 publications

Primary Systemic Amyloidosis Characterized by Multiple Keratinous Cysts: A Case Report

Primary Systemic Amyloidosis Characterized by Multiple Keratinous Cysts: A Case Report

Primary Systemic Amyloidosis Characterized by Multiple Keratinous Cysts: A Case Report

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