"I forgot to shave my hands": A case of spiny keratoderma

A 57-year-old Caucasian man presented with multiple asymptomatic spiny papules on the palms and soles that he had been shaving off with a razor for many years. He was otherwise healthy with no personal or family history of skin disease or malignancy. A diagnosis of spiny keratoderma of the palms and soles or "music box spine dermatosis" was made. The clinical, histologic, and electron-microscopic features of spiny keratoderma are distinct. This entity previously had multiple classifications and it is important to distinguish it from other keratodermas as some keratodermas can be linked to cutaneous and internal malignancies and conditions: polycystic kidney disease, liver cysts, Darier's disease, and hyperlipoproteinemia among others. Spiny keratodermas can have systemic associations and do not resolve spontaneously. Treatment is generally ineffective.     

Microbiology of the interdigital web

The microbiology of the interdigital toe areas is a complex subject that has been partially elucidated by the results of many investigations. Stable resident populations must be maintained in these moist and warm skin surfaces in order to prevent colonization of potential pathogens and overgrowth of innocuous residents. This balanced population of normal flora can be upset by conditions of increased hydration or prolonged occlusion. Population densities invariably increase as the moisture content of skin increases. ^2,4,16 Fluorescent diphtheroids, nonlipophilic diphtheroids, and gram-negative bacilli preferentially flourish under such conditions. Concomitantly, hydrated skin also is macerated easily, and this predisposes individuals to develop superficial mycotic infections of the foot. ^1,5 Stable resident populations thereby become affected by the diffusible antibiotic-like substances produced by dermatophytic fungi, ^28–30 Resistant populations of aerobic cocci, nonlipophilic diphtheroids, and gram-negative bacilli become dominant over sensitive cocci and diphtheroids in such dermatophyte infections. Whereas the keratinolytic activity of dermatophytes was initially responsible for the pruritic, scaling lesions of mild athlete's foot, it is the proteolytic activity of brevibacteria and gram-negative bacilli that is responsible for the development of severe lesions. ^5,32 Coincidently, large populations of such microorganisms generate copious amounts of metabolic by-products such as carbon dioxide and methanethiol, that are toxic to fungi.6'30 Dermatophytes thus become quite difficult to isolate in many cases of severe athlete's foot. ^13,14,32 In such exacerbated intertriginous infections, treatment with drying agents and antibacterial substances is quite effective in alleviating symptomatology.^16,32 However, dermatophytes may persist or reinfect the interdigital web and thus maintain an asymptomatic form of chronic athlete's foot. In order to prevent further exacerbations, the interdigital web surfaces must be kept dry, and antimycotic therapy must be instituted to ultimately free the individual of the dermatophytic agent. Only under these circumstances will stable populations of resident normal flora and healthy interdigital web surfaces be maintained.     

棘状角皮症

报告5例多发性棘状角皮症。患者的皮损直径均为0．5～2．0mm,突出皮面1．0～4．0mm的似棘状角化性突起主要分布于手掌。组织病理检查示表皮角化过度和界限清楚的角化不全柱,其下表皮凹陷,颗粒层变薄或正常,棘层肥厚,真皮无明显异常。超微结构显示角质层细胞内角质透明蛋白颗粒正常,角质微丝排列稍疏松。     

棘状角皮症

患者男,59岁. 主诉:双手多发对称性刺状突起15年. 现病史:患者15年前无诱因双手掌及手指侧缘出现针状或刺状角化性突起,皮损逐渐增多、变大.触物时感轻微刺痛不适.当地医院未予明确诊断,给予糖皮质激素药膏外用疗效不佳.遂于2009年6月来我科就诊.既往史:患原发性高血压8年.一直口服降压药治疗.     

Aquagenic keratoderma

Aquagenic keratoderma has been described as a transitory condition involving young females and defined clinically by the appearance of palmar lesions accentuated after immersion in water. According to previous case reports, these lesions are characterized histologically by hyperkeratosis and dilated eccrine ducts. Some aberration in the eccrine ducts has been suggested as a possible pathogenic mechanism. We report a new case in a female adolescent. With regard to the normal aspect of the epidermis in our case, the clinical changes should be ascribed to a structural alteration of the horny layer without any visible microscopy change or functional alteration of the stratum corneum involving the organism in the adolescence period.     

Atypical aquagenic keratoderma

Aquagenic keratoderma is an infrequent condition characterized by the appearance of lesions on the palms of the hands a few minutes after contact with water; these lesions vanish a short time after they dry. Published cases were primarily in adolescent females. We present the case of a male patient with lesions that clinically corresponded to aquagenic keratoderma, except that they were distributed on the backs of both hands and on the anterior face of the wrist. These characteristics had not been described in the literature prior to this case. Previously described cases were reviewed and several differential diagnoses were established, such as rofecoxib-induced palmar lesions, or those appearing in cystic fibrosis.     

表皮松解性掌跖角化病研究进展

表皮松解性掌跖角化病是一种常染色体显性遗传性单基因病,以掌跖部对称性弥漫性角化过度为主要特征,其组织学特点为表皮松解性角化过度.目前已从分子水平上阐明表皮松解性掌跖角化病由角蛋白9及角蛋白1的基因突变引起.此外,环境及药物卡培他滨也可能为其致病因素.表皮松解性掌跖角化病主要以对症治疗为主,小干扰RNA的研究逐步成为热点,为表皮松解性掌跖角化病的基因治疗提供一定的理论基础.随着对此病分子基础的研究,产前诊断的技术正不断发展.     

Mutilating palmo-plantar keratoderma

A 36-year-old male, with no history of hereditary palmo-plantar keratoderma, showed at the age of 2-3 years a typical keratoderma of the Unna Thost variety on palms and soles. At the age of 16 years he developed mutilating symptoms localized to the fingers. Radiologic examination showed abnormally pointed end phalanges.     

Mutilating acral keratoderma

A 15-year-old male, born of non-consanguineous marriage started developing thickening of skin of sole of the both feet at the age of 7 which progressed to involve whole of both feet by 11th year. Patient later developed painful autoamputation of little toes of both feet due to pseudoainhum. Involvement of the palms was limited to a localised area over palmar aspect of right middle finger and was non-progressive. No other family member was affected. This may represent a variant of Vohwinkel's syndrome or the rarer form of acral keratoderma.