Primary systemic amyloidosis with unusual dermatological manifestations: A rare case report

Vyas, Kapil; Morgaonkar, Manjaree; Gupta, Savera; Jain, Suresh

doi:10.4103/0019-5154.177767

Cited by 6 publications

(3 citation statements)

References 4 publications

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“…We presume that the patient’s pleural effusion and back sensory disturbance are both caused by this disease. 2…”

Section: Discussionmentioning

confidence: 99%

“…We presume that the patient's pleural effusion and back sensory disturbance are both caused by this disease. 2 The dermatological manifestations are seen in approximately one-third of patients with systemic amyloidosis, the most common ones are petechiae, purpura, and ecchymosis. 3 Macroglossia, plaques, papules and nodules are always reported.…”

Section: Discussionmentioning

confidence: 99%

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Primary Systemic Amyloidosis Presenting as Skin Vegetations: A Case Report

Li¹,

Wu²

2021

International Journal of Dermatology and Venereology

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Introduction: Primary systemic amyloidosis is characterized by clonal plasma cell disorder, and its signs and symptoms are various and complex, damage to the skin and mucous membrane is often more likely to attract attention.Here we reported a case of a 61-year-old male patient who presented with topical mucocutaneous lesion, as well unusual skin vegetations. Case presentation: A 61-year-old man was hospitalized due to repeated burning sensation on his back, multiple ecchymosis, and skin vegetations. Through a series of examinations (mainly including skin histopathology, bone marrow cytology, bone marrow flow cytometry, immunofixation electrophoresis), Primary systemic amyloidosis was diagnosed, but multiple myeloma could not be diagnosed. Subsequently, he received chemotherapy. In the half-year follow-up, there was no significant change in his symptoms and signs. Discussion: In this case, in addition to the typical skin damage of primary amyloidosis, the multiple skin vegetations in the buttocks, abdomen, and arms are particularly noteworthy. According to the histopathology and Immunohistochemistry of the skin vegetation, we infer that the formation mechanism of these skin vegetation is lymphatic obstruction caused by amyloid, which leads to lymphatic dilatation, lymph leakage, and dermal edema. Conclusion: Primary systemic amyloidosis is a rare disease, which is often difficult to diagnose. We should be alert to those atypical skin features so as not to delay diagnosis.

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“…We presume that the patient’s pleural effusion and back sensory disturbance are both caused by this disease. 2…”

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Primary Systemic Amyloidosis Presenting as Skin Vegetations: A Case Report

Li¹,

Wu²

2021

International Journal of Dermatology and Venereology

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“…Amyloidosis is a disease caused by the extracellular deposition of insoluble polymeric protein brils in tissues and organs [1]. Primary systemic amyloidosis (PSA) is caused by the abnormal proliferation of plasma cells.…”

Section: Introductionmentioning

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 (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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Periorbital Dermatology and Oculoplasty

Hoshy

El‐Kalioby

2020

Oculoplastic Surgery

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Primary systemic amyloidosis with unusual dermatological manifestations: A rare case report

Cited by 6 publications

References 4 publications

Primary Systemic Amyloidosis Presenting as Skin Vegetations: A Case Report

Primary Systemic Amyloidosis Presenting as Skin Vegetations: A Case Report

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

Periorbital Dermatology and Oculoplasty

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