进行性对称性红斑角化症1例

患者女,13岁。双下肢皮损伴痒痛5年余。查体:一般情况良好;臀部及双侧股内侧、膝部、小腿下段及足背对称分布红色角化性斑块,双手背散在淡红色丘疹及斑块。皮损组织病理示:表皮显著网篮状角化过度,局灶性角化不全,颗粒层增厚,棘层肥厚,真皮浅层血管周围轻度淋巴细胞浸润。诊断为进行性对称性红斑角化症。     

可变性红斑角化病1例

患者男,2岁。面部及腋下红斑1.5年。皮肤科情况:面部、颈部、双腋下及躯干部淡褐色角化过度性斑片,双手足角化过度,轻度脱屑。皮损组织病理示:表皮角化过度,棘层轻度肥厚,真皮浅层毛细血管周围少量以淋巴细胞为主的细胞浸润。诊断:可变性红斑角化症。     

进行性对称性红斑角化症1例并文献复习

一个常染色体显性遗传病家系,所有患者均表现为手足背侧及腔口周围固定性红斑角化皮损,随年龄增长逐渐自愈,无其它系统症状。皮损病理显示:表皮过度角化、棘层肥厚,伴有局部角化不全及真皮浅层轻微血管周围淋巴细胞浸润。外周血基因组DNA检测结果:携带TRPM4基因c. 3119T C杂合错义突变。诊断:进行性对称性红斑角化症。     

可变性红斑角化症1例

患者男,39岁,出生1月后躯干部及四肢出现风团样红斑,后双膝、手足及臀部相继出现角化性斑块,天冷时皮损加重,无明显痒痛感。皮肤组织病理:表皮角化过度,轻度棘层增厚,真皮乳头层血管周围见稀疏单一核细胞浸润。诊断:可变性红斑角化症。     

进行性对称性红斑角皮症1例

报告进行性对称性红斑角皮症1例。患者男,27岁,因四肢末端红斑角化27年。皮损组织病理检查示：表皮角化过度,棘层肥厚,真皮见少许炎症细胞浸润。结合临床表现诊断为进行性对称性红斑角皮症。     

儿童腋窝颗粒状角化不全

报告1例儿童腋窝颗粒状角化不全.患儿女,12岁.左侧腋窝发生红斑、丘疹伴瘙痒10年.组织病理检查示角化过度伴角化不全,并可见透明角质颗粒,颗粒层轻度增厚,表皮突延长.诊断:腋窝颗粒状角化不全.     

幼年局限型毛发红糠疹1例

患儿女,9岁,四肢关节鳞屑性红斑伴掌跖角化痒1个月。组织病理示:表皮部分区域角化过度与角化不全大致交替出现,部分区域水平与柱状角化不全交替出现,毛囊角栓,其周围灶性角化不全。棘层增厚,轻度棘细胞间水肿,部分表皮突和真皮乳头相应增宽。真皮浅层毛细血管扩张、充血,管周轻度淋巴细胞、组织细胞浸润。诊断为毛发红糠疹。     

可变性红斑角化症1家系

可变性红斑角化症是以可变性红斑和皮肤角化过度为主要特征的罕见遗传性皮肤病。目前的研究认为突变基因主要是GJB3和GJB4。本患者女,22岁,四肢红斑、脱屑18年。皮肤科情况:四肢伸侧及双足背可见大片红色对称性角化性斑片,以双下肢为重,边缘清晰,形状不规则。皮损组织病理示:表皮角化过度,颗粒层及棘层增厚,真皮浅层有淋巴细胞浸润。诊断:可变性红斑角化症。家族中可追溯到有5人有相类似症状。针对目前热点突变基因GJB3和GJB4进行直接测序,未发现突变。     

可变性红斑角化症1例

患者女,24岁。四肢臀部斑片23年。查体:四肢及臀部可见边界清楚的棕红色角化过度性斑片,双手、足角化过度,轻度脱屑。皮损组织病理示:表皮角化过度,棘层肥厚,真皮非特异性炎症细胞浸润。诊断:可变性红斑角化症。     

可变性红斑角化症1例

患者男,39岁,出生1月后躯下部及四肢出现风团样红斑,后双膝、手足及臀部相继出现角化性斑块,天冷时皮损加重,无明显痒痛感。皮肤组织病理：表皮角化过度,轻度棘层增厚,真皮乳头层血管周围见稀疏单一核细胞浸润。诊断：可变性红斑角化症。     

先天梅毒1例

患儿女,1个月。面部及四肢红斑水疱,伴脱屑20天。体检:一般情况良好。皮肤科情况:面部、双手、臀部及双足弥漫性潮红肿胀,部分表面呈鳞屑性或片状黄色痂皮。TRUST1:32(+),TPPA(+)。诊断:先天梅毒。予苄星青霉素G5万u/kg肌注,1次/w,治疗3次后,皮疹消退,复查血清TRUST(-)。     

Linear and whorled nevoid hypermelanosis complicated with inflammatory linear verrucous epidermal nevus and ichthyosis vulgaris

We report a 17-year-old girl who presented with linear and whorled melanosis following Blaschko lines mainly on her trunk. Ichthyosiform lesions and linear scaling erythemas were observed respectively on her lower limbs and the dorsa of her hands, left knee, ankle and foot. No abnormality was found in systemic examination and blood tests. A biopsy specimen of pigmentation of her back showed there was increased pigmentation within the basal keratinocytes, with focal incontinentia pigmenti. Notable lymphangiectasis could also be seen in the mid part of dermis, which had never previously been described. The biopsy of scaling erythemas of her hand showed dominant hyperkeratosis with focal parakeratosis, acanthosis and papillomatous hyperplasia in the epidermis. The diagnosis of linear and whorled nevoid hypermelanosis (LWNH) complicated with inflammatory linear verrucous epidermal nevus and ichthyosis vulgaris was made in accordance with clinical and pathological manifestations. LWNH ought to be differentiated from incontinentia pigmenti and hypomelanosis of Ito.     

Acrokeratoelastoidosis

A 45-year-old white woman presented with several years' history of firm, shiny papules on the lateral hands with slight extension to the dorsal fingers. The lesions first appeared between the index fingers and thumbs on both hands. They gradually increased in number, coalescing into plaques and affecting the junction between the palmar and dorsal skin. The patient did not have involvement of her feet. She had been diagnosed previously with chronic eczema that had failed to respond to multiple topical medications. In addition, the patient's sister had similar lesions on both hands. The patient denied any symptoms of hyperhidrosis, excessive sun exposure, or trauma. The plaques were asymptomatic, but were cosmetically unappealing to the patient. On physical examination, small, firm, skin-colored, hyperkeratotic papules, coalescing into plaques, were located on the junction between the palmar and dorsal skin on both lateral margins of the thumb and on the radial side of the index finger (Fig. 1). There were no lesions on the feet. A biopsy taken from a papule on the patient's left hand was consistent histologically with acrokeratoelastoidosis. The biopsy showed marked degeneration of collagen in the dermis with solar elastosis and some smudging of the papillary dermal collagen (Fig. 2). She was treated with clobetasone cream to the affected areas on the hands. After 6 weeks of treatment, she reported no significant improvement.     

A unique variant of Darier's disease.

A 45-year-old black woman presented with a chief complaint of an increasing number of "light spots" on her face, upper trunk, and legs. She had a 4-year history of a pruritic eruption on the dorsum of her hands. The eruption was particularly pruritic in the summer months. Other family members, including her sister and her daughters, reportedly had a similar dermatologic problem. The patient had been previously evaluated and biopsied by another dermatologist. The earlier biopsy was nondiagnostic, however, and she presented for further evaluation of this problem. On physical examination, the patient had hypopigmented macules along her jawline (Fig. 1), lateral neck, and upper chest. She had similar hypopigmented macules on her thighs. She had hyperkeratosis of the palmoplantar surface of her hands and feet. The dorsum of her hands had numerous coalescing, shiny, flat-topped, hypopigmented papules (Fig. 2), and several of her fingernails had distal, V-shaped notching. A punch biopsy from a papule on the dorsum of her hand was obtained. The epidermis had corps ronds present with focal areas of acantholysis above the basal layer (Fig. 3). The dermis had sparse, superficial, perivascular infiltrates composed of lymphocytes and histiocytes. These changes were consistent with our clinical diagnosis of Darier's disease (keratosis follicularis).     

Bullous Pemphigoid in Infancy: Case Report and Literature Review

Abstract: We report a 2-month-old girl who developed bullous pemphigoid on her hands and feet shortly after receiving her routine immunizations. Infantile bullous pemphigoid has a clinical presentation distinct from bullous pemphigoid seen in older children and should be included in the differential diagnosis of blisters involving the hands and feet. Our patient responded well to topical corticosteroid therapy.     

Erythemas Associated with Essential Thrombocythemia

Two cases of erythemas associated with essential thrombocythemia (ET) are reported. Case 1: A 64-year-old woman, whose ET had been treated with myebronitol for about 10 years, developed erythematous plaques on her trunk after she stopped taking the medicine. Case 2: A 58-year-old man, who had had gallstones and ET, developed annular erythemas on his thighs, arms, and trunk. Operation for the gallstones had no effect on the eruption. In both cases, remission of thrombocythemia induced by myebronitol was associated with the disappearance of these erythemas.     

妊娠性类天疱疮1例

患者女,23岁,孕33周。躯干及四肢出现红色皮疹,伴瘙痒2周。皮肤科情况:躯干及四肢广泛分布轻度水肿性红色斑疹和斑丘疹,双手及双足有较多张力性水疱。右前臂皮损组织病理示:真皮乳头高度水肿,水疱形成,疱液及真皮浅层少量嗜酸粒细胞和淋巴细胞浸润。直接免疫荧光检查示:皮肤基底膜带处C3呈线状沉积。诊断:妊娠性类天疱疮。予小剂量强的松口服9d后好转。     

多发性硬下疳合并二期梅毒1例

患者男,34岁。阴茎、阴囊反复起溃疡6个月,躯干、大腿起红斑5d。查体:阴茎及阴囊可见多处浅溃疡,直径0.5～1.0cm,界清,边缘整齐,呈软骨样硬度,无触痛。躯干、大腿见广泛分布类圆形淡红斑,大小不一;双手足未见红斑和脱屑。实验室检查:TPPA(+),RPR1:128(+),HIV抗体阴性。HSV-IIgG(+),HSV-IIIgG(+),HSV-IIgM(-),HSV-IIIgM(-)。诊断:多发性硬下疳;二期梅毒。予苄星青霉素240万U分两侧臀部肌注,1次/周,连用3次后皮损消退。     

Necrolytic acral erythema without hepatitis C infection

Necrolytic acral erythema is a newly described entity characterized by sharply demarcated scaly plaques on the dorsum of the hands and feet. More than 30 patients have been reported since 1996, all of whom had anti-hepatitis C virus antibody. A 32-year-old Taiwanese woman had been diagnosed with and treated for systemic lupus erythematosus with lupus nephritis about 10 years earlier. Soon thereafter, she noted several well-demarcated keratotic plaques with erythematous borders on her feet, with sparing of the soles. Histopathology showed diffuse parakeratosis with a neutrophil infiltrate, hypogranulosis, pale upper keratinocytes, scattered and grouped dyskeratotic cells, psoriasiform hyperplasia and a mild lymphocytic infiltrate in the upper dermis. The diagnosis was made after three biopsies. The lesions regularly worsened just before and during menstruation, but patch and intradermal tests for progesterone and estrogen were negative. There was no evidence of either hepatitis B or hepatitis C infection. The lesions did not respond to treatment with zinc. The rash regressed spontaneously when corticosteroids were stopped and recurred when they were restarted, finally resolving completely after she was treated with high-dose pulse steroids for her lupus.     

Pemphigus vulgaris with nail involvement presenting with vegetating and verrucous lesions

We report the case of a 68-year-old female with longstanding insulin-treated diabetes mellitus, observed for the first time in our department in August 1999 with multiple painful erosive lesions of the oral cavity and many bullous or erosive lesions on the abdominal wall, back, and thigh. She also had vegetating and verrucous lesions, similar to common warts, involving the hands and feet, mainly on the palms, palmar surface of the fingers, and nail folds. Her lesions were present for 1 year. Skin and mucous biopsies showed the characteristic histopathologic findings of pemphigus vulgaris, with an epidermal intercellular IgG deposition on direct immunofluorescence. Histology of a warty lesion of the finger also showed suprabasal acantholysis. After partial improvement with low doses of oral steroids and azathioprine, her disease progressed to involve the oral cavity, trunk, hands, feet, and scalp. Control of her disease required successive treatments of mycophenolate mofetil and cyclophosphamide, as well as corticosteroids. A partial response was obtained with all these treatments. After being controlled by cyclophosphamide that was slowly tapered, she is now well controlled with azathioprine and oral steroids, showing only discrete lesions of the oral mucosa after 1 year of followup. We report this case of pemphigus vulgaris with unusual clinical aspects, namely vegetating and verrucous lesions as well as nail involvement, rarely described in this disease.