肛周生殖器部位丘疹性棘层松解性角化不良1例

患者女,41岁。外阴、肛周出现丘疹伴瘙痒2年,无糜烂、疼痛不适。病理检查示:表皮角化过度,棘层肥厚,棘层松解,可见散在角化不良细胞,真皮浅层血管增生,少许淋巴细胞浸润。诊断为:肛周生殖器部位丘疹性棘层松解性角化不良。     

肛周生殖器部位丘疹性棘层松解性角化不良6例临床分析

目的分析肛周生殖器部位丘疹性棘层松解性角化不良的临床及病理特点。方法回顾性分析2013年1月至2022年12月在北京大学第三医院确诊的6例肛周生殖器部位丘疹性棘层松解性角化不良患者的临床及组织病理学资料。结果 6例患者中, 女3例, 男3例, 发病年龄22 ～ 58岁, 发病至确诊时间1 ～ 18个月,均无类似皮损家族史。6例患者临床表现均为肛周或生殖器部位密集分布多发肤色或灰白色、粟粒大小丘疹, 部分表面糜烂、结痂, 可伴瘙痒。组织病理表现类似:角化过度, 灶性角化不全, 表皮内出现棘层松解及角化不良细胞。6例患者外用糖皮质激素或他克莫司软膏等治疗, 疗效不一, 部分皮损可持续存在, 反复发作。结论丘疹性棘层松解性角化不良罕见, 好发于肛周生殖器部位, 诊断时需注意结合临床特征及组织病理。     

外生殖器部位棘层松解性皮病

报告1例外生殖部位棘层松解性皮病。患者女,54岁。外阴灰白色、肤色丘疹伴瘙痒8个月。皮肤科检查：双侧大阴唇可见暗红色水肿性红斑,边界清楚,红斑上散在线状排列的米粒至黄豆大肤色、苍白色圆形扁平丘疹,表面光滑,质地较硬;部分丘疹融合形成斑块,呈苔藓样变,散在抓痕、糜烂。皮损组织病理检查：表皮灶性角化过度、角化不全,棘层肥厚,棘细胞松解,角化不良,表皮下血管增生,淋巴细胞浸润。直接免疫荧光检查示阴性。诊断：外生殖器部位棘层松解性皮病。     

多发性疣状角化不良瘤一例

46岁男性患者。额部、左颞部角化性丘疹1年,缓慢增大。皮损组织病理示:表皮角化过度伴角化不全,不规则增生,棘层松解,基底层上方形成裂隙,可见谷粒及圆体。诊断:疣状角化不良瘤。肿物切除术后愈合良好,无复发。     

棘层松解角化不良性表皮痣

报告1例棘层松解角化不良性表皮痣.患者女,29岁.右腿内侧线状皮损伴瘙痒20年.皮损组织病理检查可见类似于毛囊角化病的改变:表皮乳头瘤样增生、基底层上方裂隙与棘层松解,局部可见谷粒和圆体.诊断为棘层松解角化不良性表皮痣.     

疣状角化不良瘤1例

患者男,66岁,面部丘疹偶伴瘙痒1年。体检:左侧眼睑下可见一黄豆大小褐色丘疹,疣状外观,中央角化略凹陷,表面粗糙,无明显渗出。皮损组织病理示:表皮内见两处杯状凹陷,内充满大量角化物,伴角化不全,基底层可见棘层松解性裂隙及绒毛结构,细胞分化良好。诊断:疣状角化不良瘤。治疗:手术切除。随访2个月无复发。     

棘层松解性角化不良表皮痣 1例

报告1例左足底条状角化性损害为主要临床表现的棘层松解性角化不良表皮痣。病理显示乳头瘤样增生伴棘层松解性角化不良细胞。     

Grover病1例并文献复习

患者男,51岁。躯干及四肢丘疹、水疱及脓疱反复发生30余年,腹股沟角化性丘疹10余天。皮损组织病理检查见表皮局灶性棘层松解,直接免疫荧光阴性。诊断:暂时性棘层松解性皮肤病(Grover病)。并结合本例临床特点进行文献复习。     

棘层松解型光线性角化病1例

报告1例棘层松解型光线性角化病.患者男,80岁,因左颞部皮肤斑块伴疼痛1年余就诊.皮肤组织病理检查示:表皮增生,角化过度,棘层松解,基底层不典型细胞向真皮上部生长.免疫组化检查示:CK(+)、P63(+),ki67约20%(+).诊断为棘层松解型光线性角化病.     

毛囊角化病伴抑郁障碍1例

患者女,28岁,头面角化性丘疹13年,泛发全身8年。皮肤科情况:面部、颈部、腹部可见大量角化性丘疹,多个指(趾)甲有纵行条纹及V形缺损。皮损组织病理示:局灶性基底细胞层上裂隙,棘层上部表皮松解,可见谷粒和圆体细胞。PHQ-9评分为18分。诊断:毛囊角化病伴抑郁障碍。     

Two Cases of Aquagenic Wrinkling of the Palms in Korean Healthy Women: Easily Overlooked Disease

Aquagenic wrinkling of the palms (AWP) is a rare condition, which is characterized by appearance of whitish papules and plaques and an excessive wrinkling and swelling of the palmar skin after exposure to water. Herein, we report two cases of AWP. A 17-year-old woman presented prickling focal various sized whitish papules, plaques with wrinkles on both palms after water contact for 6 months ago. Histologic findings were consistent with AWP. A 26-year-old woman presented asymptomatic multiple various sized whitish papules, plaques with wrinkles on both palms after water contact for 1 year ago. Both patients underwent a cystic fibrosis transmembrane conduct receptor test but were negative and improved without any treatment.     

Acantholytic dyskeratotic epidermal naevus localized unilaterally in the cutaneous and genital areas

A 38-year-old woman presented with unilateral lesions on the left side of the body and in the genital area. Clinically, the lesions showed a polymorphic pattern: brownish papules in the axilla, keratotic comedo-like papules on the hand and foot, and whitish papular plaques on the labia majora and anal canal. There was no family history of skin diseases. Histologically, cutaneous and mucosal specimens were characterized by acantholytic and dyskeratotic cells, corps ronds and grains in the parakeratotic zone, and by hyperkeratosis and parakeratosis. A diagnosis of epidermal naevus with acantholytic dyskeratosis was made.     

So-called multicentric pigmented Bowen's disease. Report of a case and a possible etiologic role of human papilloma virus.

Multicentric pigmented Bowen's disease (MPBD) is a bowenoid atypia in the genitocrural region with peculiar clinical appearances. A 36-year-old Japanese female patient showed a variety of lesions. Clinically the lesions on the external genitalia consisted of brown-black papillomatous eruptions, black discrete or confluent papules, and whitish macerated papules. Histologically only black papules showed bowenoid atypia, but whitish papules also showed transient bowenoid atypia. Electron microscopically, in all three kinds of the lesions, spherical particles with a diameter of about 50 nm were scattered or gathered together in the nuclei of the keratinocytes beneath the horny layer. These particles were morphologically similar to human papilloma virus. Based upon clinical, histologic and electron microscopic observations, MPBD may be regarded as a new entity, and a term such as multicentric pigmented viral papulosis may be rather preferable than MPBD.     

水源性肢端角化症

患者男,28岁。半年前开始双手接触水数分后钟局部出现丘疹和斑块,伴肿胀,脱离水源约15min后,皮损及不适感消失。临床表现为:双手掌可见细小、白色的扁平丘疹及斑块。皮损组织病理示:表皮角质层明显增厚,棘层肥厚。诊断:水源性肢端角化症。     

Darier disease: the use of dermoscopy in monitoring acitretin treatment

Darier disease is an uncommon autosomal dominant inherited disease, caused by a mutation in the ATP2A2 gene. The clinical findings are hyperkeratotic papules on the trunk, scalp, face, and neck, maceration of intertriginous areas, palmar pits, whitish papules on the oral mucosa and nail abnormalities. The main histopathologic findings are acantholysis and dyskeratotic keratinocytes. Dermatoscopic features are comedo-like openings with a central polygonal yellowish/brownish structure, surrounded by a whitish halo. First-line treatment includes acitretin. Five reports have been published describing Darier disease dermatoscopic findings. Herein, we report for the first time a patient under acitretin treatment and dermatoscopic follow-up.     

Síndrome de Birt-Hogg-Dubé, en un paciente con clínica cutánea y mutación en el exón 12 del gen BHD: c.1429 C > T;p.R477X

Birt-Hogg-Dubé syndrome is an autosomal dominant genodermatosis characterized by the presence of fibrofolliculomas, renal cancer, pulmonary cysts, and spontaneous pneumothorax. Recently, the folliculin gene responsible for this process has been identified, located on the short arm of chromosome 17. We present the case of a 49-year-old man with multiple whitish papules on the face, neck, and retroauricular area. Histology was compatible with fibrofolliculoma and genetic study showed a pathogenic mutation of the folliculin gene.     

Rediscovering Perifollicular Elastolysis: A Hitherto Undocumented Entity in India

Perifollicular elastolysis, also known as papular acne scars is a common but taken for granted entity. It appears as asymptomatic whitish yellow papules on the trunk and proximal arms. Histologically there is loss of elastin around the pilosebaceous follicles. The lesions are the result of a scarring process that appear as papules and are often mistaken for papules of acne and are treated. A knowledge of this entity which has never been described before in India is being reiterated.     

Papular elastorrhexis in childhood improved by intralesional injections of triamcinolone.

Papular elastorrhexis is a rare disease developing asymptomatic skin-colored small papules in adolescence with histopathological loss of elastic fibers. There has been no established treatment for this disease. A 4-year-old Korean boy had multiple, hard, whitish papules on his chest and back for one year. Histopathologic examination revealed focal loss of elastic fibers in the dermis, and X-ray examination showed no bony abnormalities. His skin lesions were improved by intralesional injections of triamcinolone but recurred after four months.     

MALIGNANT ATROPHIC PAPULOSIS (DEGOS'' SYNDROME)

A 29-year-old Japanese man with malignant atrophic papulosis had a 1-year history of scattered, asymptomatic, whitish or skin-colored papules, as well as erythematous papules with central atrophy showing a porcelain-like appearance on the trunk and extremities. Histologic examination revealed lymphocyte-mediated necrotizing vasculitis with considerable deposition of mucin in the dermis. The wedge-shaped necrosis of the dermis was not detected. Based on a review of the literature we think that this case represents the histologic features of the early skin lesions of malignant atrophic papulosis.     

Milialike idiopathic calcinosis cutis and syringoma in Down's syndrome

A 6-year-old girl with Down's syndrome presented milialike whitish small papules on her hands and feet and periorbital syringoma. Histopathological examination of the hand lesion revealed small localized calcium deposits and syringoma in the adjacent upper dermis. This is a very rare but typical case of calcinosis cutis with syringoma in a patient with Down's syndrome.