Request PDF on ResearchGate | On Jul 7, , Helvecio Leal Santos Júnior and others published AMILOIDOSE SISTÊMICA HEREDITÁRIA EM UM CÃO DA . Title: AMILOIDOSE SISTÊMICA EM AVES AQUÁTICAS CRIADAS EM CATIVEIRO. (Portuguese); Alternate Title: Systemic amyloidosis in captive waterfowls. Os autores apresentam um caso de amiloidose sistémica com envolvimento cardíaco e discutem a importância de um elevado índice de suspeição para o.

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This case report is about a year-old female patient with systemic amyloidosis and multiple myeloma simultaneously. Amyloid cutaneous infiltrative lesions like papules, nodules, or plaques with a sisteemica aspect were found in the eyelids, neck and retroauricular region, among others. She had presented intermittent papular lesions on the upper eyelids one year before, which worsened following local trauma.

A local skin biopsy showed amorphous and eosinophilic substance in the dermis. Congo red staining confirmed the amyloid deposits.

Primary systemic amyloidosis associated with multiple myeloma

Following the diagnosis of multiple myeloma based on amyloid skin lesions, the patient was referred to the Hematology service and died 5 months after the diagnosis. Amyloidosis; Sistemics Multiple myeloma; Skin. Amyloidosis is the term used to describe abnormal deposition of a protein-rich substance insoluble and proteolysis resistant in the skin or other organs. The composition of such amyloid substances, in addition to fibrils, although formed by different types of proteins and carbohydrates presents some common aspects: Amilidose also bind to thioflavin, becoming strongly fluorescent and conferring a amjloidose aspect to histological sections.

Tissue infiltration by these amyloid deposits occurs in a localized or systemic manner. When localized, there is only cutaneous involvement and when systemic, it may be primary or secondary. The primary systemic form is related to hidden dyscrasia or multiple myeloma, while the secondary form arises from complications of chronic inflammatory processes like rheumatoid arthritis, osteomyelitis, sclerodermia, Hansen’s disease and tuberculosis, among others.


We report a case of systemic amyloidosis associated with multiple myeloma, which was diagnosed based on the cutaneous symptom complex. A white, year-old female patient presented at the dermatology service complaining of papulous lesions with bloody content in the upper eyelids for one year. The first lesions appeared following emotional stress and regressed spontaneously within 15 days. Since then she started presenting new lesions in outbreaks, always in the same places.

Such lesions worsened when there was local trauma. Concerning associated pathologies, arrhythmia she was using a pacemakerhypertension and fibromyalgia were informed. During the physical examination confluent papules of varied sizes were observed in the internal half of upper eyelids and more discreet in the internal corner of the lower eyelids Figure 1. After local trauma with a blunt object, there was onset of local purpuric papules – a Sign of Barth Figure 2.

Biopsies of the eyelid, gums and rectal mucosa lesions were performed, with the following results: Congo red staining confirmed it was an amyloid substance Figures 4 and 5. Absence of amyloid material. The hemogram showed that creatinine, protein electrophoresis, VHS, iron and ferritin were within normal values.

Cranium and long bones Amiloivose did not reveal lytic lesions. The sistemcia was normal, from an anatomic viewpoint.

Amiloidose sistêmica do tipo AA em um canino Shar-pei Chinês | Sonne | Acta Scientiae Veterinariae

Among the abnormal tests, we found: Thus it was possible to make the diagnosis of multiple myeloma in the initial phase in a patient that presented only cutaneous manifestations of amyloidosis. The patient was referred to a hematology service for multiple myeloma treatment. She died 5 months after the diagnosis. The sequential analysis of aminoacids demonstrated that in systemic amyloidosis associated with multiple myeloma, fibrils are composed of an AL protein light chain amyloidosis proteinwhich in turn is composed of variable immunoglobulin light chain aminoterminalintact light chain, or both.


The most common cutaneous signs include: Such signs occur spontaneously following trauma and are due to amyloid infiltration in the vascular walls.


They affect mainly the eyelids, lips, tongue, oral mucosa, neck, axillae, umbilical scar and retroauricular, inguinal and anogenital regions. Some less ssistemica cutaneous alterations are: In primary systemic amyloidosis, the deposit occurs predominantly in tissues such as in the gastrointestinal tract, smooth and skeletal muscles, carpal connection, nerves and skin.

In contrast, in the secondary systemic form the amyloid deposition takes place above all in parenchymatous organs: The amyloid deposits in the gastrointestinal tract may result in a variety of manifestations: The prognosis of amyloidosis associated with multiple myeloma is unfavorable, with between 12 and 15 months mean sistemixa rate.

It is very important to make the earliest possible diagnosis, and always investigate multiple myeloma, especially in a,iloidose of systemic amyloidosis in patients considered suspect after the anatomic-pathological test used in this case. Multiple myeloma and systemic amyloidosis.

Primary, systemic amyloidosis and the dermatologist where classic skin lesions may provide sistemiva clue for early diagnosis. Diffuse haemorrhagic bullous amyloidosis with multiple myeloma.

Acta Scientiae Veterinariae

A case of systemic amyloidosis associated with multiple myeloma presented as macroglossia and purpura. J Eur Acad Dermatol Venereol.

Ederson Valei Lopes de Sistemicq Av. Murchid Homsi, – Vila Elvira. Approved by the Advisory Board and accepted for publication on All the contents of this journal, except where otherwise noted, is licensed under a Creative Commons Attribution License.

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