Pseudoxanthoma-like late-onset focal dermal elastosis

A 73-year-old woman presented with a 10-year history of asymptomatic coalescent flat pale yellow lesions forming a cobblestone pattern that resembled pseudoxanthoma elasticum over her neck, upper trunk and axillae. Skin biopsies from both the neck and axilla showed focal upper dermal elastosis with increase in structurally normal elastic fibres. There was no evidence of either pseudoxanthoma elasticum or solar elastosis in either biopsy. This under-recognized presentation has previously been identified as a distinct clinicopathological entity and termed late-onset focal dermal elastosis.     

Analysis of elastin metabolism in patients with late-onset focal dermal elastosis.

Late-onset focal dermal elastosis has recently been described as new clinical entity characterized by pseudoxanthoma elasticum-like eruptions and an accumulation of normal-appearing elastic fibres in the dermis. Elastin and collagen contents of the skin of 2 patients were 2- and 1.4-fold higher than in the skin of controls, respectively. A focal accumulation of elastin but not of fibrillin-1 was observed by immunohistochemical staining. The levels of type I and III collagen and elastin mRNAs isolated from cultured patient fibroblasts were elevated 2-3-fold compared with control fibroblasts. There was no significant change in the excretion of elastin peptides in the urine of patients and controls. These results suggest that the focal accumulation of elastic fibres in the patient skin may be related to overexpression of elastin rather than to altered degradation of elastin.     

Late-onset focal dermal elastosis: clinical and histological features

We describe two patients with lesions clinically resembling pseudoxanthoma elasticum and histologically exhibiting focal elastosis. with normal-appearing elastic fibres in the mid- and deep dermis. We consider that these skin lesions represent a previously undescribed entity, whose pathogenesis appears to be related to the ageing process.     

Papillary dermal elastosis

There are numerous acquired disorders of elastic tissue that are distinguished by a combination of clinical appearance, location, gender, age of onset, and characteristic histopathologic findings. We present a case of a 36-year-old man with multiple confluent, hypopigmented papules that coalesced into plaques with prominent follicular ostia over the dorsal aspects of the forearms, shoulders, upper chest, and upper back. Histologically there was selective loss of papillary dermal elastic fibers. The clinical and histopathologic findings in this case are consistent with an acquired disorder of elastic tissue which we believe represents the second reported case of papillary dermal elastosis.     

Late-onset focal dermal elastosis: an uncommon mimicker of pseudoxanthoma elasticum

Late-onset focal dermal elastosis is a rare disorder that presents clinically with the development of small white-to-yellow papules simulating pseudoxanthoma elasticum (PXE) in otherwise healthy adults in the seventh through ninth decades. It is characterized histopathologically by foci of increased normal-appearing elastic tissue in the reticular dermis. The disorder lacks any of the systemic complications of PXE and clinically resembles several other elastic tissue disorders that mimic PXE. We report two cases of late-onset focal dermal elastosis. The first is of a 75-year-old female who presented with symmetrically distributed, 2-5 mm white-to-yellow, discrete and coalescing, non-follicular papules on the posterolateral neck, anterior chest and axillae. The second case involves a 39-year-old female who presented with asymptomatic flesh-colored lesions on the posterior neck, back, antecubital and popliteal fossae, thighs, forearms and wrists. Skin biopsies in each case revealed aggregates of elastic fibers in the reticular dermis without calcification. The differential diagnosis of clinical and histopathologic imitators of PXE is discussed.     

Linear focal elastosis

Aims   Merkel cell carcinoma (MCC) is said to be the most aggressive of all skin cancers, with frequent metastases and a significant mortality rate. This study examined the role of mast cells and vascular density in the prognosis of Merkel cell carcinoma.
Methods   Mast cells and vessels were stained immunohistochemically in 36 MCC with antibodies to tryptase and CD34 respectively. The number of stainable mast cells and vessels were quantified within the tumours and surrounding stroma. Other clinical and histological parameters were also examined, statistically analysed and compared to subsequent clinical course and prognosis.
Results   Prognosis was poorer with higher mast cell numbers (p < 0.002) and with increased vascular density (p = 0.005). A one unit increase in mast cells predicted a 1.7 times increased risk of death. Prognosis was also adversely affected by the presence of lymphovascular invasion (p = 0.03) and larger tumour size (p = 0.05).
Discussion   Although factors predicting the behaviour of MCC are largely unknown, various parameters have been proposed as potential prognostic markers [ Llombart, 2005 ].
Mast cells are pivotal in immunity, but a prognostic role for mast cells in malignancies has also been suggested, possibly through mitogenesis and angiogenesis [ Ch'ng, 2006 ]. Vascular density, as a surrogate for angiogenesis, may have prognostic value in certain cancers, e.g. melanoma [ Depasquale, 2005 ].
In this study, increased mast cells counts and vascular density were associated with an adverse prognosis in MCC. Evaluation of mast cell infiltrates and vascular density can provide prognostic data and may ultimately help to guide appropriate treatment selection for this aggressive form of skin cancer.     

Linear focal elastosis

A case of linear focal elastosis is reported. A 19-year old male presented with asymptomatic, palpable yellow lines over back along with striae distensae over axilla. Light microscopic examination demonstrated dermal thickening but no change in the epidermis. The elastic tissue stain revealed fragmented elastic fibres throughout the dermis.     

儿童线状局限性弹性组织变性

报告1例儿童线状局限性弹性组织变性.患儿男,4岁.因出生时即发现左股部淡黄色水平条纹而就诊.皮损组织病理检查:真皮中层胶原纤维束间隙增宽.Verhoeff-van Gieson染色见真皮中层簇集大量弹性纤维碎片.该文作者对线状局限性弹性组织变性的临床和组织病理进行了研究,并对近30年的文献作一复习.     

Case study on linear focal elastosis

Linear focal elastosis is an uncommon dermal elastic disorder, characterized by palpable, linear bands clinically and an increase in abnormal elastic tissue histologically. Two similar cases are presented and discussed.     

线状局限性弹性组织变性一例

患者女,51岁。因发现下背部黄白色条纹2个月,于2014年4月来我科门诊就诊。2个月前患者家人突然发现其下背部出现数条可触及的黄白色条纹,微隆起于皮肤,无自觉症状,就诊于当地医院,未明确诊断和治疗,后自行观察,发现条纹渐增长、增多,遂来我院。否认明显的体重增加或减少病史,否认皮损处有外伤史,否认系统应用糖皮质激素治疗史,否认家族遗传病史,家族中无类似病史……     

Secondary localized cutaneous amyloidosis in solar elastosis

A case of secondary localized cutaneous amyloidosis in solar elastosis was studied by light and electron microscopy. Amyloid deposition was restricted to areas with elastotic changes, and in some areas both changes were intermingled with each other. The amyloid was permanganate-sensitive, and proved to be protein AA. Ultrastructurally it was composed of fine tubular filaments. It is suggested that the amyloid deposition in this case was related to sunlight damage of the skin.     

Linear focal elastosis. An ultrastructural study

We studied an 86-year-old Japanese man with linear focal elastosis. The lesions were asymptomatic yellow striae in the lumbar region, histologically composed of massive, well-demarcated basophilic fibers that stained positively with elastic tissue stains. Electron microscopy revealed fine, reticular or granular electron-dense materials, and elastic fiber microfibril-like materials in the matrix, in addition to numerous mature and immature elastic fibers. These findings suggest that active elastogenesis was occurring in the lesions. The four cases reported so far have the three common features of age, sex, and lesion location.