Unusual primary cutaneous localized amyloidosis

The case of a 41-year-old female patient with an unusual type of primary localized cutaneous amyloidosis is reported. Clinical examination revealed lesions typical for macular cutaneous amyloidosis, while histology and histochemistry, in contrast, revealed the presence of nodular amyloidosis of the skin with deposition of lambda light chain amyloid.     

特殊部位的原发性皮肤淀粉样变性3例

报告3例部位特殊的原发性皮肤淀粉样变性。3例患者皮损分别发生于外耳、乳房及额部,由小而密集的褐色斑疹或丘疹组成,或呈苔藓样改变,不伴或伴有瘙痒。皮损组织病理检查示:真皮乳头层淀粉样蛋白沉积,刚果红和结晶紫染色阳性。诊断:原发性皮肤淀粉样变性。     

Unusual cutaneous manifestation of angiocentric T-cell lymphoma: a case report

Angiocentric T-cell lymphoma may clinically present with a broad variety of cutaneous manifestations, including papules, nodules, and ulcerated tumors, and may mimic cutaneous vasculitis and Wegener granulomatosis. Histologic diagnosis of angiocentric T-cell lymphoma also may present a challenge because of variations in the degree of cellular infiltrate and cellular atypia. The correct diagnosis is critical because of the importance of choosing the correct therapeutic modality-chemotherapy or x-ray radiation. We report a case of angiocentric T-cell lymphoma with extensive necrotic facial ulcers that responded to treatment with fludarabine, cyclophosphamide, and prednisone.     

The molecular skin pathology of familial primary localized cutaneous amyloidosis

Please cite this paper as: The molecular skin pathology of familial primary localized cutaneous amyloidosis. Experimental Dermatology 2010; 19: 416–423. Abstract: Familial primary localized cutaneous amyloidosis (FPLCA) is an autosomal dominant disorder associated with chronic itching and skin lichenification. In lesional skin, there are apoptotic basal keratinocytes and deposits of amyloid material on degenerate keratin filaments in the upper dermis. The genetic basis of FPLCA involves mutations in the OSMR and IL31RA genes but the disease pathophysiology is not fully understood. In this study, we identified new pathogenic heterozygous missense mutations in the OSMR gene (p.Val631Leu and p.Asp647Tyr) in two Dutch FPLCA families. We then compared gene expression profiles between FPLCA lesional skin (n = 4) and site‐matched control skin (n = 6). There was twofold or greater upregulation of 34 genes and downregulation of 43 genes. Most changes in gene expression (verified by quantitative RT‐PCR) reflected alterations in epidermal differentiation and proliferation consistent with lichenification, but we also noted a reduction in several interfollicular keratinocyte stem cell markers in FPLCA skin. Differences in gene expression were also noted for proteins involved in apoptosis and nerve conduction. Collectively, this study expands the molecular basis of FPLCA and provides new insight into the skin pathology of this condition.     

Unusual cutaneous findings in primary systemic amyloidosis. Report of a case.

A case of primary, systemic amyloidosis is reported because of the unique location of cutaneous lesions. Amyloid deposits had occluded both external auditory canals. Both the cutaneous biopsies and postmortem examination established the diagnosis.     

Primary cutaneous amyloidosis

Three cases of primary cutaneous amyloidosis are reported. Family history was negative. Systemic involvement was ruled out. Histopathology was confirmed by congored stain. Patients responded to oral colchicine.     

皮肤异色病样淀粉样变病

皮肤异色病样淀粉样变是原发性皮肤淀粉样变的少见类型,国内外关于本病的报道较少;多种因素参与皮肤异色病样淀粉样变发病,明确诊断需组织病理的支持,必要时需行电镜观察,且需与有皮肤异色样变表现的其他疾病相鉴别.基于已报道的病例,就该病病因、临床特点、组织病理、诊断及鉴别诊断等方面进行探讨.     

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.     

皮肤异色病样淀粉样变病

皮肤异色病样淀粉样变是原发性皮肤淀粉样变的少见类型,国内外关于本病的报道较少;多种因素参与皮肤异色病样淀粉样变发病,明确诊断需组织病理的支持,必要时需行电镜观察,且需与有皮肤异色样变表现的其他疾病相鉴别.基于已报道的病例,就该病病因、临床特点、组织病理、诊断及鉴别诊断等方面进行探讨.     

Unusual manifestations of primary cutaneous amyloidosis in association with Raynaud''s phenomenon and livedo reticularis

A patient with unusual manifestations of primary cutaneous amyloidosis, including macules, papules, oedematous plaques and urticarial lesions is described. Raynaud's phenomenon and livedo reticularis were an associated finding. During the acute phase, single doses of systemic corticosteroids resulted in an impressive, long-lasting improvement in cutaneous manifestations.     

Late cutaneous schistosomiasis representing an isolated skin manifestation of schistosoma mansoni infection

Ectopic late cutaneous schistosomiasis is usually preceded or accompanied by visceral schistosomiasis infection. Our patient presented the very rare case of late cutaneous schistosomiasis as an isolated skin manifestation. Perigenital lesions occurred 1 year after contact with infested water. Identification of the few eggs remaining in the late lesion among the dense cellular infiltrate was difficult. Electron-microscopic studies clearly demonstrated the characteristic eggshell ultrastructure.