Generalized linear porokeratosis

A 23-year-old woman was seen for widespread skin lesions present since the age of 2.5 years. Twenty years ago, she developed a brown macular lesion on her right buttock. The lesion became hyperkeratotic and subsequently spread through the posterior aspect of her right leg. It later spread to the right side of the trunk and to the right arm. When she was 9 years old, she developed similar lesions on her left arm and leg. After she was 13 years old, no new skin lesions appeared. There was no family history of similar lesions. On examination, there were numerous linear and whorled, reddish-brown, hyperkeratotic plaques, with central atrophy and raised borders, following Blaschko's lines on all of the extremities. These lesions on the extremities extended to the dorsum of the hands and feet (Fig. 1). She had hyperkeratotic lesions on the pressure points of both of the soles, but no palm involvement. The number of lesions on the right side was greater than that on the left. Reddish-brown annular plaques with central atrophy and raised borders, appearing in zosteriform configuration, and numerous individual 2-3-mm erythematous lichenoid papules were observed on the right side of the thorax and the right inguinal region (Fig. 2). No face, scalp, or mucous membrane involvement was seen. The nails of the second and fifth fingers of the right hand and the nail of the third finger of the left hand showed nail dystrophy with longitudinal ridges and pterygium. All the nails of the right foot and the nails of the first and fifth toes of the left foot showed dystrophic changes with subungual keratosis. The patient was otherwise in good health. Two biopsy specimens taken from a hyperkeratotic plaque and a lichenoid papule showed an epidermal invagination with angulated parakeratotic tier, denoting cornoid lamella. The epidermis just underneath the cornoid lamella displayed vacuolization and the granular layer was absent. The adjacent epidermis was atrophic, and hydropic degeneration within the basal cell layer was seen. In the dermis, a nonspecific, mild, chronic, inflammatory cell infiltrate, telangiectatic vessels, and pigment-laden macrophages were present. These findings were consistent with linear porokeratosis (Fig. 3). Microscopic examinations and mycologic cultures of the nails were negative. We decided to treat our case systemically with retinoids, but the patient refused this therapy. So, topical tretinoin 0.05% was started once a day. A marked improvement was observed in hyperkeratosis through the first 4 weeks of treatment and plateaued at 8 weeks. After 10 weeks, the lesions had almost disappeared. We planned to continue the applications every other day. One year later, she remains stable with application of topical tretinoin 0.05% twice a week and is satisfied with the final appearance. She is under regular follow-up.     

Dermoscopy assisting the diagnosis of mycetoma: case report and literature review

Mycetomas are a chronic skin infection characterized by perilesional edema, formation of sinus tracts, and discharge of purulent or seropurulent exudate containing grains. This report aims to demonstrate the clinical diagnosis (by dermoscopy) of a skin lesion that initially bared no clinical features of a mycetoma.     

Coexistence of congenital linear porokeratosis and disseminated superficial porokeratosis

The coexistence of two or more forms of porokeratosis in a single individual is rarely reported. We report here on a patient exhibiting the coexistence of congenital linear porokeratosis and disseminated superficial porokeratosis. To our knowledge, this entity has been previously reported only once.     

Congenital facial linear porokeratosis

We report a unique case of congenital linear porokeratosis with exclusive facial involvement in a 27-year-old Chinese man. No other family member was affected. To our knowledge, this is the first report in the English literature of congenital linear porokeratosis confined to the face.     

Coexistence of disseminated superficial porokeratosis in childhood with congenital linear porokeratosis

Porokeratosis is a genodermatosis characterized by abnormal epidermal keratinization with the histologic finding of cornoid lamella. To date, five clinical variants have been identified. However, the coexistence of these variants in a single patient has been described only rarely. We report a 5-year-old girl with the simultaneous occurrence of porokeratotic lesions in linear and disseminated patterns. Linear lesions were detected at birth and the disseminated lesions developed at the age of 3 years. Histologically the lesions had cornoid lamella, which is typical of this condition.     

Multicentric Bowen disease in linear porokeratosis

Linear porokeratosis is a genodermatosis, caused by a clonal proliferation of keratinocytes along the lines of Blaschko. This type of porokeratosis is particularly susceptible to malignant degeneration e.g. to Bowen disease, a squamous cell carcinoma (SCC) in situ with the potential for significant lateral spread. Here we report a case of linear porokeratosis on the left leg of a 56-year-old man, complicated with two lesions of multicentric Bowen disease.     

Dermoscopy in the diagnosis of juvenile xanthogranuloma

Juvenile xanthogranuloma is the most common form of non-Langerhans cell histiocytosis. It manifests clinically as a red-yellow papule, usually showing spontaneous remission. The diagnosis is based on clinical and histological findings. We report the use of dermoscopy (“setting sun” pattern) as an adjuvant tool in the diagnosis of juvenile xanthogranuloma in a female patient presenting with a 2-month history of a pre-auricular papule.     

单侧线状型汗孔角化症一例

患儿,男,7月,单卵双胎之A,出生后其母即发现患儿右上臂至腋下并延续至右乳头处线状波纹样深褐色斑。出生一个月开始右腋下反复出现数个芝麻大小水疱,3-5天自然消退,于2008年11月就诊于我科。体格检查：生长发育正常,皮肤科检查：右上臂中部至右腋下并延续至右胸部线状深褐色波纹样斑,右腋下数个绿豆至黄豆大小丘疱疹,毛发、牙齿、甲均正常,神经系统正常。 患儿父母体键,非近亲结婚,患儿为单卵双胎A,出生体重2.2kg,单卵双胎B,出生体重2.05kg,两者出生时均无窒息史。 皮损组织病理活检水疱部位皮损特征：表皮内海绵水肿及表皮内多发水疱,疱内多数嗜酸粒细胞浸润,表皮内散在角化不良细胞。 患儿同胞弟弟生长发育正常,既无皮肤损害,也无皮肤外异常表现。 讨论：色素失禁症是一种x性联显性遗传的皮肤病。在男性几乎 是致死的疾病,而女性由于带有镶嵌的杂合子可以生存[1]。本病例的特点系男性病例,并且是单卵双胎中之一发病,国内尚未见到报道。     

单侧线状型汗孔角化症一例

患者,男,31岁。左侧腰腹部丘疹、斑块31年,破溃、结痂1年。既往以“银屑病”治疗多年效果欠佳,行组织病理学确诊为线状型汗孔角化症。给予阿维A 40mg/d治疗2个月效果欠佳。     

Multiple squamous cell carcinomas complicating linear porokeratosis

Linear porokeratosis is a distinctive variant of porokeratosis with malignant transformation, a known but infrequently reported complication. We report a case of linear porokeratosis on the right leg of a 57-year-old man present since infancy that, over a 2-year period, developed two squamous cell carcinomas within the lesion. Concurrent right inguinal lymphadenopathy was demonstrated on biopsy to be reactive in nature.     

Porokeratosis linearis unilateralis (linear porokeratosis)

A case of linear porokeratosis is reported. This rare disease is described and the different entities that can be grouped under the term porokeratosis are discussed.