匐行性穿通性弹性纤维病

报告1例匐行性穿通行弹性纤维病.患者男,20岁.面部淡红色环形排列丘疹2年,无痒痛感,缓慢进展.皮损组织病理检查:局部表皮增生,可见穿通通道及内含嗜碱性碎片.Verhoeff-Van Gieson弹性纤维染色示真皮浅层弹性纤维增多,穿通的通道内可见弹性纤维.结合临床表现及组织病理检查结果,诊断为匐行性穿通行弹性纤维病.     

弹性纤维性假黄瘤1例

患者女,35岁。颈部、双侧腋下密集淡黄色扁平丘疹10余年。组织病理学检查示真皮大量波纹状、扭曲、断裂的深嗜碱性纤维成分。弹性纤维染色可见变性弹性纤维。诊断:弹性纤维性假黄瘤。     

局灶性肢端角化过度

报告1例局灶性肢端角化过度患者。患者女,37岁。左侧拇指、大鱼际扁平丘疹7个月,无自觉症状。皮损组织病理检查:表皮局限性角化过度,颗粒层及棘层增厚,基底细胞灶状液化变性,真皮浅层血管周围稀疏炎性细胞浸润。弹性纤维染色真皮弹性纤维大致正常,未见明显弹性纤维断裂及减少,弹性纤维染色阴性。诊断:局灶性肢端角化过度。     

弹性纤维性假黄瘤1例

报道发生于颈部的弹性纤维性假黄瘤1例。患者女,40岁,颈部淡黄色丘疹、斑块伴皮肤松弛10余年。皮肤科检查:颈部可见较密集分布的淡黄色丘疹,米粒至绿豆大小,部分密集融合成斑块,皮疹大致沿皮纹分布,颈部皮肤松弛形成皱褶。行组织病理检查示表皮角化过度,真皮中下部弹性纤维变性、断裂,呈不规则碎片状、颗粒状,符合弹性纤维性假黄瘤组织学改变。弹性纤维染色阳性。     

弹性纤维性假黄瘤1例

报告1例颈部弹性纤维性假黄瘤,患者女,41岁,颈部多发性肤色或淡黄色扁平丘疹40余年,偶觉瘙痒。组织病理学检查示真皮网状层中部有多量波纹状,扭曲,断裂或不规则形状的深嗜碱性纤维成分,诊断为弹性纤维性假黄瘤。     

局灶性肢端角化过度

报告1例局灶性肢端角化过度.患者女,45岁.双手淡黄色扁平丘疹10余年,无自觉症状.组织病理检查可见表皮明显角化过度,颗粒层及棘层增厚,未见典型空泡细胞.弹性纤维染色示真皮弹性纤维大致正常.     

获得性反应性穿通性胶原病合并糖尿病

报告1例获得性反应性穿通性胶原病合并糖尿病。患者女,53岁。四肢及躯干丘疹、结节及溃疡伴伴剧烈瘙痒半年。皮肤科检查：背部正中可见一浸润性紫红色肿块,全身散在分布大小不等的淡红色坚实丘疹、结节,中央凹陷,表面结痂。皮损组织病理检查：表皮呈杯形下陷,其内可见角化不全性碎屑、胶原变性及炎性细胞浸润。皮损两边棘层增厚,角栓底部菲薄,局部可见垂直穿通表皮的胶原蛋白。Masson三色染色及van Gieson染色示胶原组织向表皮穿通。诊断：获得性反应性穿通性胶原病合并2型糖尿病,背部痈。     

肢端角化弹性组织变性1例

报告1例肢端角化弹性组织变性。患者女,37岁。双手淡黄色扁平丘疹渐增多30余年,无明显自觉症有关方面,组织病理学检查见表皮角质层增厚,部分棘层轻度肥厚,真皮上部有呈水平方向的带状结缔组织区,轻度嗜碱性变,弹性纤维染色示真皮弹性纤维减少,可见断裂的弹性纤维。     

泛发性扁平红色丘疹——播散型丘疹性环状肉芽肿

诊断:播散型丘疹性环状肉芽肿. 皮损组织病理特点:表皮轻度角化过度,表皮突变平.真皮浅层可见局灶性胶原纤维变性区,周围较多的组织细胞和淋巴细胞浸润,呈栅栏状排列.     

角化性皮肤病

20100529局灶性肢端角化过度/周城(北京大学人民医院皮肤科),杜娟,张建中∥临床皮肤科杂志.-2009,38(11).-693～694女,45岁。双手淡黄色扁平丘疹10余年,无自觉症状。皮肤科检查:双手背、手指背、指间关节伸侧、大小鱼际及手腕屈侧见密集的圆或卵圆形淡黄色至肤色半透明角化性丘疹,栗粒至黄豆大,质较硬,表面光滑,部分皮损融合,少数丘疹呈脐状。组织病理示:表皮角化过度,颗粒层及棘层增厚,真皮浅层血管周围稀疏性细胞浸润;真皮弹性纤维及胶原纤维大致正常,未见明显弹性纤维断裂及减少。图2参7(刘昕)20100530线状汗孔角化病1例/黄莉宁(贵阳中医学院一附院皮肤科),贾敏,廖跃…∥     

A Case of Eruptive Collagenoma on the Left Calf

Eruptive collagenoma is an acquired connective tissue nevus without family history. It is typically described as numerous small papules or nodules on the trunk and arms with histopathological features of decreased or degenerated elastic fibers. We report a case of a 16-year-old male who presented with multiple asymptomatic 2 to 5 mm sized yellowish grouped papules on the left calf. Histopathologically, the lesion showed thickened homogenized collagen fibers highlighted by Masson trichrome stain and decreased and fragmented elastic fibers stained by Verhoeff-van Gieson stain. The skin lesion was diagnosed as eruptive collagenoma and no treatment was provided.     

Elastosis perforans serpiginosa in a patient with pseudoxanthoma elasticum

A 34-year-old female previously diagnosed of pseudoxanthoma elasticum developed an annular plaque with serpiginous borders of 42 by 30 mm in diameter on the inner left arm. A similar lesion later appeared on the inner right arm. Histopathological examination of a papule showed short, fragmented, granular, basophilic and calcified elastic fibers in the mid-reticular dermis. The epidermis showed hyperplasia surrounding degenerated and normal elastic fibers. Transepidermal elimination channels of these elastic fibers were also observed. These findings were consistent with the diagnosis of elastosis perforans serpiginosa. Abundant multinucleated giant cells were observed surrounding the area of epidermal hyperplasia and in the reticular dermis. The patient was treated with tazarotene, and the plaques disappeared in 9 months.     

Elastosis Perforans Serpiginosa

Elastosis perforans serpiginosa is a rare skin disease characterized by transepidermal elimination of abnormal elastic fibers. This condition classically presents as small papules arranged in serpiginous or annular patterns on the neck, face, arms, or other flexural areas. While these lesions may spontaneously resolve, they often persist for longer periods of time. Though numerous treatment modalities have been described in the literature, most are not very effective. A 35-year-old man presented to Gangnam Severance Hospital with a 2-year history of skin eruptions on his neck, which were refractory to topical antifungal or steroid treatment. On examination, the patient showed multiple crusted and erythematous papules arranged in an annular pattern with central clearing. A biopsy specimen revealed acanthosis with notable transepidermal elimination of nuclear debris and eosinophilic degenerated elastic fibers from the dermis via an epidermal channel. Verhoeff-van Gieson staining showed dense clumps of altered elastic fibers in the papillary dermis. Based on these findings, a diagnosis of elastosis perforans serpisinosa was made. Treatment with topical 0.05% tretinoin application for 6 months resulted in no improvement.     

Acquired perforating folliculitis in chronic renal failure: report of two cases

Background Patients with chronic renal failure, regardless of its cause, with/without hemodialysis/peritoneal dialysis, showed cutaneous alterations. Two patients had a follicular hyperkeratotic papular eruption, with clinical and histological features of perforating folliculitis. Methods A dermatological examination and multiple skin biopsies were done. Results We diagnosed acquired perforating folliculitis in two chronic renal failure patients on hemodialysis. The lesions were located predominantly on the lower extremities with hyperkeratotic papules and small nodules. Microscopically, the papule always affected the follicular structure. The follicle was widely dilated and packed with keratotic material, necrotic debris and degenerated basophilic coarse connective fibers passing which passed through follicular perforations. Conclusion Acquired perforating folliculitis is a cutaneous manifestation in chronic renal failure, with/without hemodialysis treatment, that can mimic essential primary perforating folliculitis. Transepidermal elimination mechanisms and the distinction between primary and acquired perforating dermatoses arc discussed.     

Digital papular calcific elastosis: a histopathological, histochemical and ultrastructural study of 20 patients

In 1960, 5 patients were reported with a condition termed "degenerative collagenous plaques of the hands". Ultraviolet light and chronic pressure seem to be the inciting factors. Clinically, it is characterized by keratotic, translucent papules, in linear array, on the radial border of the hands. Histology shows hyperkeratosis and dermal elastosis. The current study documented the histopathological, histochemical and ultrastructural features of 20 patients with this condition and considered the differential diagnoses. The findings were in accordance with those of previous studies, but peculiar, basophilic elastotic masses (BEMs), were a prominent finding. Ultrastructurally, elastic tissue appeared in the form of degenerated non-fragmented and fragmented elastic fibres, degenerated fibres showing foci of increased density, with or without a fibrillar component and as amorphous, structureless material. BEMs arose almost exclusively from degenerated elastic tissue and contained calcium. Disintegrated collagen may be incorporated in the amorphous material and, subsequently, form a minor part of BEMs. Solar elastosis, and its variants, show some similarity to degenerative collagenous plaques of the hands, but lack the constant presence of these calcified angular elastotic masses. BEMs show resemblance to elastic globes, and elastotic bodies found in elastotic nodules of the anthelix and cartilage-hair hypoplasia. In the clinical setting of "degenerative collagenous plaques of the hands", BEMs seem to be unique and due to their extensive presence serve as a non-specific marker for this condition. Digital papular calcific elastosis therefore seems to be more appropriate nomenclature.     

Buschke-Ollendorff syndrome: three generations in a Japanese family

Buschke-Ollendorff syndrome is an autosomal dominant disease characterized by disseminated connective tissue nevi of elastic type and osteopoikilosis. We report a 6-year-old Japanese boy with connective tissue nevi that showed slightly grouped yellowish or skin-colored papules and nodules, distributed from birth over his right thigh, right buttock, and back. Radiologic skeletal survey revealed osteopoikilosis. A skin biopsy specimen obtained from a papule showed that collagen bundles in the dermis were thickened and homogenized. The elastic fibers were not increased but were coarse and clumped. The boy's father, at age 34, has had osteopoikilosis and similar papules and nodules on his left buttock and back for the preceding 18 years. We studied the paternal grandfather, aged 65. He had osteopoikilosis and similar skin lesions on his lumbar region. None of the three had a history of hearing loss or malignant tumor. To our knowledge, this is the first report of three generations of Buschke-Ollendorf syndrome in a Japanese family.     

Disseminated Nevus Anelasticus

A 23-year-old man presented with a diffuse and fine wrinkling of the skin of his back and trunk. Skin lesions had first appeared about 5 years earlier and resulted from coalescing of multiple, flat, pinkish-red papules, a few millimeters in diameter. A diagnosis of widespread cutaneous elastic hamartoma was made. Histology revealed a focal disappearance of elastic fibers within circumscribed areas of the dermis. The pattern of the dermal collagen and reticulin was normal. No concomitant changes in the bone density were recognized by x-ray, excluding the Buschke-Ollendorff syndrome. Therefore, we call this case "disseminated nevus anelasticus."     

Cutaneous extravascular necrotizing granuloma (Churg Strauss granuloma)

Churg Strauss granuloma (cutaneous extravascular necrotizing granuloma) is a distinct entity which is associated with systemic immunoreactive or autoimmune diseases in a majority of cases. Typically, Churg Strauss granuloma presents as symmetrical papules or nodules on the extremities. There are two histological patterns: the classic pattern reveals palisading granuloma with central degenerated collagen, interspersed polymorphonuclear leukocytes, and leukocytoclastic debris; the focal basophilic necrosis pattern does not show palisading granuloma. We report two cases of Churg Strauss granuloma with different histopathological patterns.     

Elastic nevus with normal expression of elastin and elastin-related proteins mRNAs.

A 3-year-old Japanese girl presented with disseminated white papules on the trunk. A biopsied specimen of the lesional skin revealed a focal accumulation of elastic fibers in the mid to deep dermis. No skeletal involvement was detected. This case was considered to be elastic nevus without systemic involvement. Since an elevated elastin expression in the fibroblasts of typical Buschke-Ollendorff syndrome (BOS) has been reported, elastin and elastin-related protein mRNA levels in the cultured patient fibroblasts were determined. There were no significant differences of steady-state levels of elastin, fibrillin 1 and microfibril-associated glycoprotein 1 mRNAs between patient and matched control fibroblasts. Elastic nevus without skeletal involvement may be etiologically different from typical BOS.     

泛发型环状肉芽肿2例

环状肉芽肿是以环状丘疹或结节性损害为特征的慢性皮肤病,其中泛发型环状肉芽肿临床较少见。本文报道2例女性泛发型环状肉芽肿病例,皮损表现为躯干四肢弥漫的丘疹、环状斑块。甲状腺功能、EB病毒及巨细胞病毒抗体、血糖血脂检查均未见异常。组织病理表现为真皮浅中层栅栏样肉芽肿形成,中央结缔组织变性,周围上皮样细胞,多核巨细胞及淋巴组织细胞浸润。