结节型淀粉样变

患者女,48岁. 主诉:下肢黄色结节14年. 现病史:14年前无明显诱因右下肢出现棕黄色斑块,逐渐增大形成结节,无自觉症状.4年前左下肢亦出现类似皮损.2008年8月来我院就诊.发病以来一般情况良好.     

Nodular amyloidosis

We present a 38-year-old man with nodular amyloidosis and diabetes mellitus. Skin biopsy showed massive amyloid deposition in the dermis. Immunohistochemical study revealed that amyloid deposits contained amyloid of Kappa light-chain origin and P component. Under electron microscope, vascular changes were noted in addition to amyloid deposits. The origin of nodular amyloidosis and its relationship to diabetes mellitus is discussed in this report.     

Nodular cutaneous amyloidosis

A nodular cutaneous amyloidosis biopsy specimen from a solitary nodule of a 75-year-old patient was characterized by amino terminal sequence analysis and was proved to be derived from immunoglobulin kIII light chain. Five years after the diagnosis of amyloidosis in the skin was made, a rectal biopsy demonstrated amyloid deposits in a blood vessel. It is suggested that nodular cutaneous amyloidosis is a slowly progressive systemic disease of the AL type, that manifest itself mainly in the skin.     

Xanthomas in a patient with Langerhans cell histiocytosis and liver cirrhosis

Skin involvement in acute forms of Langerhans cell histiocytosis (LCH) is in the form of erythematous papules, although rare forms of xanthomatous lesions have been described. We present the case of a boy with acute disseminated LCH who, at the age of 16 months, began to experience outbreaks of seborrheic dermatitis-like skin lesions and progressive hepatic dysfunction. The symptoms were complicated by partial central diabetes insipidus and specific pulmonary infiltration by Langerhans cells, which led to fibrosis. During the course of the disease, the patient developed liver cirrhosis, alterations in the lipid profile and disseminated xanthomatous skin lesions, concomitant with the lesions specific to the LCH. Despite successive cycles of chemotherapy, the outcome was the death of the patient after five years, due to his liver disease. Xanthomatous lesions in LCH are typical of the late stages of chronic progressive forms, such as Hand-Schüller-Christian disease. When they appear in acute disseminated forms, there is some controversy over whether they correspond to a progression of the disease towards more chronic forms, or whether they are associated independent lesions, such as in this case.     

结节性皮肤淀粉样变一例

患者,男,46岁。左侧鼻唇沟、鼻翼及下颌结节1年。皮肤组织病理：真皮内可见大量嗜酸性、无定形物质沉积,甲紫染色和刚果红染色均阳性。诊断：结节性皮肤淀粉样变。给予复方倍他米松局封后结节缩小。     

Nodular localized primary cutaneous amyloidosis

The most common forms of localized primary cutaneous amyloidosis are lichen (papular) and macular amyloidosis. Nodular or tumefactive amyloidosis is rare and demonstrates important clinical, historical, pathogenic and prognostic differences from the lichen and macular variants. We report two cases of nodular localized primary cutaneous amyloidosis.     

Nodular localized primary cutaneous amyloidosis: a bullous variant

Primary cutaneous amyloidosis describes a group of disorders in which amyloid is deposited in the skin without evidence of systemic involvement. Nodular localized primary cutaneous amyloidosis (NLPCA) is a rare form of these skin‐restricted amyloidoses. We present an unusual case of NLPCA in a 51‐year‐old man, who had clinical and histopathological evidence of subepidermal bullous formation, a unique feature in NLPCA. The possible pathogenesis of this change is discussed.     

Nodular cystic fat necrosis in a patient with erythema nodosum

Nodular cystic fat necrosis shows a distinctive spectrum of clinical and histological features. Most of the lesions arc mobile subcutaneous nodules in regions vulnerable to trauma, such as the elbows, knees, and shins. There have been no reports of an association with other conditions. The histology is characterized by encapsulated fat necrosis in which multiple, non-viable adipocytes are surrounded by condensed fibrous tissue. We report a case of early stage nodular cystic fat necrosis associated with erythema nodosum.     

Systemic amyloidosis manifestation in a patient with psoriatic arthritis

Systemic amyloidosis secondary to psoriatic arthritis is rare, and published data are based mainly on case reports and are associated with increased mortality. This is the report of a patient with long-term psoriatic arthritis and chronic sialadenitis, who showed an inadequate response to therapy. The diagnosis of secondary amyloidosis was attained through biopsies of genital skin lesions. Although very rare, it is important that dermatologists and general practitioners consider the possibility of amyloidosis in patients with chronic inflammatory diseases, since an early intervention can be implemented, and thus, the prognosis of this condition can be improved.     

Nodular localized primary cutaneous amyloidosis: a long-term follow-up study

We present long-term follow-up data on patients with nodular localized primary cutaneous amyloidosis (NLPCA) seen at the St John's Institute of Dermatology between 1968 and 1999. This is the largest clinical follow-up study of this type of amyloid to date. Based on these cases we estimate the rate of progression of NLPCA to systemic amyloidosis to be only 7%, much lower than the 50% rate currently quoted in the literature.     

Managing the patient with amyloidosis

Amyloidosis is a rare disease characterized by extracellular protein deposits in various tissues and vital organs of the body, which often leads to severe and debilitating chronic health problems and death. Early recognition of symptoms, prompt diagnosis and treatment, and nursing care are essential in decreasing the morbidity and improving the quality of life of the patient with this disease.