Follicular Porokeratosis at alae Nasi; A Case Report and Short Review of Literature

Porokeratosis are disorders of keratinization, characterized histologically by a parakeratotic column, the cornoid lamellae, and clinically by a distinct peripheral ridge. Porokeratosis usually occurs on the trunk or extremities, and facial lesions are rare. Follicular involvement, identified by follicular localization of cornoid lamellae, is uncommon and described only in association with disseminated superficial actinic porokeratosis (DSAP) and porokeratosis Mibelli (PM). A 34-Year- old woman is presented with asymptomatic 1 cm plaque lesion with hyperkeratotic ridge and depressed center on her nose since three years. Histopathological examination of a biopsy revealed cornoid lamellae confined exclusively to the follicle.     

Facial and bilateral acral porokeratosis with nail dystrophy: A case report

Porokeratosis is a rare disorder of keratinization with unknown etiology. It exhibits both sporadic and autosomal dominant inheritance. The lesions are sharply demarcated, hyperkeratotic, and annular with distinct keratotic edges. The cornoid lamella is the histological hallmark of porokeratosis. Porokeratosis lesions on the face may have a superficial or a destructive nature. To our knowledge there are only a few cases of facial porokeratosis that have been reported. We report a sporadic form of facial and bilateral acral porokeratosis with nail dystrophy.     

Facial porokeratosis characterized by destructive lesions

Porokeratosis is a rare group of keratinizing diseases. It is inherited as an autosomal dominant disease with variable penetrance, although sporadic cases are often reported. Porokeratosis has as its histological hallmark the typical cornoid lamella. Porokeratosis lesions localized on the face can vary from superficial to destructive in nature. Only 12 cases have been reported in the literature to date. We report one more unusual case of destructive facial porokeratosis.     

Eruptive inflammatorische disseminierte Porokeratose bei einer 71-jährigen Patientin

The inflammatory stage of disseminated superficial porokeratosis is a rare disease, which has only been described in case reports, primarily affecting Japanese patients. After having asymptomatic disseminated superficial porokeratosis for years, a typical patient then experiences a sudden exacerbation with distinct pruritus. Histologically, the lesions may show eosinophilic spongiosis in addition to the characteristic cornoid lamella. We describe a 71-year old patient with clinically and histologically diagnosed inflammatory stage of disseminated superficial porokeratosis.     

Type 2 segmental manifestation of disseminated superficial porokeratosis showing a systematized pattern of involvement and pronounced cancer proneness

Nine tumors of squamous cell carcinoma developed in a 61-year-old Japanese woman with linear porokeratosis. She had disseminated superficial porokeratosis, but a linear arrangement of pronounced lesions was found only on the left side of the body, and all of the tumors arose on the linear lesions on the left side of the body. Some of her family members had disseminated superficial porokeratosis. This case may represent a type 2 segmental manifestation of disseminated superficial porokeratosis showing a systematized pattern of involvement and pronounced cancer proneness.     

Simultaneous development and parallel course of disseminated superficial porokeratosis and ovarian cancer: Coincidental association or true paraneoplastic syndrome?

Disseminated superficial porokeratosis has been described in the setting of immunosuppressive conditions, including organ transplantation, infections, and hematopoietic malignancies. The outbreak of disseminated superficial porokeratosis during the development of solid organ malignancies has been rarely reported in the literature in patients affected by hepatitis C virus-related hepatocellular carcinoma or by cholangiocarcinoma, which suggests a paraneoplastic nature of the cutaneous disease. We report an unusual case of disseminated superficial porokeratosis in a patient affected by ovarian cancer, characterized by simultaneous onset and a parallel course of the two pathologies; there was good clinical response to chemotherapy, accompanied by a successful stop of disseminated superficial porokeratosis progression and gradual clearing of the keratotic lesions.     

Q开关532nm激光治疗浅表播散性汗孔角化症一例

报告Q开关532 nm激光成功治疗1例浅表播散性汗孔角化症。患者,女,49岁。颜面部弥漫分布大小不等浅褐色斑疹,中心色淡略凹陷,边缘隆起,似黑圈状损害。皮损组织病理检查诊断为汗孔角化症。经Q开关532 nm激光治疗2次,皮损大部分消退,取得较好效果。     

Linear porokeratosis with multiple squamous cell carcinomas successfully treated by electrochemotherapy

Porokeratosis is a clonal epidermal disorder of keratinization characterized by annular lesions with an atrophic centre and a hyperkeratotic edge. The cornoid lamella is the histopathological hallmark. Six clinical variants are recognized: porokeratosis of Mibelli; disseminated superficial porokeratosis; disseminated superficial actinic porokeratosis (DSAP); porokeratosis plantaris et palmaris disseminata; punctate porokeratosis and linear porokeratosis. Linear porokeratosis is the type most frequently associated with malignant transformation into squamous cell carcinoma (SCC). It is thought to represent a mosaic form of DSAP and has an incidence of less than 1 in 200 000; treatment options are limited. We describe a patient with systematized linear porokeratosis and multiple SCCs who was successfully treated with bleomycin electrochemotherapy (ECT), a form of intralesional chemotherapy. In view of their large number, the individual SCCs were treated with bleomycin ECT. One year post‐treatment the patient remains tumour free. To our knowledge, this is the first case of multiple SCCs treated by ECT in the context of systematized linear porokeratosis. Our case highlights the challenges associated with diagnosing and managing this unusual form of porokeratosis.     

Porokeratosis in a patient with dermatomyositis

There are several reports of porokeratosis in the context of immmunosuppressive diseases. These mainly include organ transplant, HIV infection, lymphomas and some inflammatory and autoimmune diseases commonly treated with immunosuppresive drugs or chemotherapy. Disseminated superficial actinic porokeratosis is the clinical variant of porokeratosis that most frequently develops in immunosuppressive states. We report a case of porokeratosis in a woman with dermatomyositis.     

Linear Porokeratosis in Two Families with Disseminated Superficial Actinic Porokeratosis

Two case reports of linear porokeratosis occurring in individuals or families with disseminated, superficial, actinic porokeratosis (DSAP) are presented. Linear porokeratosis and DSAP may be different expressions of one dominantly inherited condition. We reviewed the clinical features of linear porokeratosis and its association with other forms of porokeratosis. The linear form has a potential for carcinomatous change.     

Eruptive disseminated superficial porokeratosis with rapid resolution: a drug‐induced phenomenon?

We report a case of eruptive disseminated superficial porokeratosis occurring in a 63‐year‐old man with no history of excessive sun exposure. Unlike disseminated superficial actinic porokeratosis, this condition resolved rapidly with minimal topical treatment. This is most likely to have represented a drug‐induced phenomenon, which is very rarely reported in the dermatological literature.     

Disseminated superficial actinic porokeratosis occurring in two members of an Asian family

Disseminated superficial actinic porokeratosis (DSAP) is a genodermatosis which usually affects fair-skinned individuals at sites of chronic sun exposure, although the face is usually spared. We now report the occurrence of this condition in a Pakistani mother and her 13-year-old daughter, both predominantly with facial lesions.     

Exclusive facial porokeratosis: histopathologically showing follicular cornoid lamellae

Porokeratosis is a heterogeneous group of disorders that are mostly inherited in an autosomal dominant fashion. It has a wide variety of clinical manifestations with a characteristic histological finding of cornoid lamella. Porokeratosis usually affects the trunk or the extremities. Exclusive facial lesions are rare, although 15% of patients with disseminated, superficial, actinic porokeratosis do have facial lesions. Herein, we discuss the case of a 25-year-old woman who noticed the appearance of well-defined, brownish, macular lesions located on both cheeks. She had no other symptoms. A skin examination showed well-demarcated, multiple, brownish macules with raised hyperkeratotic borders on both cheeks and a half-palm-sized, arcuate-shaped lesion formed by grouped macules on the right cheek. No other parts of the body were affected. Histopathological examination of a biopsy specimen obtained from the hyperkeratotic margin gave a diagnosis of porokeratosis with follicular involvement of the cornoid lamellae. Topical pimecrolimus was applied twice a day to the lesions, although little improvement was achieved after 2 months.     

Disseminated superficial actinic porokeratosis with Bowen's disease

While there have been several reports about the development of malignant neoplasms in porokeratosis of Mibelli and porokeratosis plantaris, palmaris et disseminata, only one case of squamous cell carcinoma in disseminated superficial actinic porokeratosis (DSAP) has been documented. A review is presented of the variants of porokeratosis that have so far been described in the literature as well as the differential diagnosis, genetic aspects and therapy of DSAP. The first case of DSAP with Bowen's disease is presented.     

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.     

A Case of Disseminated Superficial Actinic Porokeratosis Subsequent to Renal Transplantation

Disseminated superficial actinic porokeratosis is characterized by the development of numerous annular keratotic lesions on sun-exposed areas, accompanied by pathological evidence of cornoid lamellae. We examined a case of disseminated superficial actinic porokeratosis in a 40-year-old male who had undergone renal transplantation and was being treated with immunosuppressants. Five years after surgery, he began to develop numerous eruptions. Some of these eruptions enlarged and developed over a second period of five years until he finally required hospitalization.     

Disseminated superficial actinic porokeratosis: a clinical study

Disseminated superficial actinic porokeratosis is an autosomal dominant condition that requires sun exposure for full expression. It affects only sun-exposed areas, with relative sparing of the face. In sun-damaged skin of Australians disseminated superficial actinic porokeratosis is commonly mistaken for solar keratosis. Twenty-nine subjects with disseminated superficial actinic porokeratosis were involved in an extensive questionnaire and clinical study. The distribution of lesions was charted on all subjects, with a mean count of 268 lesions per subject. We found no evidence that skin cancer had arisen in disseminated superficial actinic porokeratosis lesions. A review of the etiologic and clinical features of disseminated superficial actinic porokeratosis also is presented.     

Porokeratosis on the scrotum: two cases

Porokeratosis is a group of hereditary or acquired disorders of epidermal keratinization, characterized by keratotic lesions with an atrophic center and a prominent peripheral ridge. At least seven clinical variants have been identified: the plaque type, disseminated superficial porokeratosis, disseminated superficial actinic porokeratosis, linear porokeratosis, giant porokeratosis, porokeratosis plantaris palmaris et disseminate, and punctuate porokeratosis. Genital porokeratosis is a rare condition even in disseminated forms. We described two male patients who had porokeratosis only affecting the scrotum. The patients were treated by surgical excision under local anesthesia. One‐year follow‐up revealed no sign of recurrences and malignant changes.     

Linear porokeratosis superimposed on disseminated superficial actinic porokeratosis: report of two cases exemplifying the concept of type 2 segmental manifestation of autosomal dominant skin disorders.

A concept of dichotomous types of segmental involvement of autosomal dominant skin disorders has recently been proposed. Among the different types of porokeratosis, disseminated superficial actinic porokeratosis is known to be an autosomal dominant skin disorder, and linear porokeratosis represents the segmental form of the disease. We intended to exemplify the type 2 segmental manifestation within this concept. Clinical and histopathologic aspects of porokeratotic lesions of 2 patients were investigated. The family history was studied in both cases. Linear porokeratosis superimposed on disseminated superficial actinic porokeratosis was observed in both patients. These 2 cases of linear porokeratosis associated with disseminated superficial actinic porokeratosis can be taken as further examples of a type 2 segmental involvement occurring in an autosomal dominant skin disorder.     

Topical photodynamic therapy in disseminated superficial actinic porokeratosis

Photodynamic therapy (PDT) has been reported to be useful in treating certain nonmelanoma skin cancers and a variety of benign skin conditions. We examined whether PDT might be effective in the treatment of disseminated superficial actinic porokeratosis. Three patients with classical disseminated superficial actinic porokeratosis had a small test area treated with topical aminolaevulinic acid PDT. Following the first treatment, the disorder cleared in the test area in one patient, but failed to respond in the other two patients. After subsequent treatment, the initial benefit seen in the first patient was not sustained, and no response was seen in the second patient, while the third patient was not retreated due to hyperpigmentation induced by the procedure. The results obtained in this small case series suggest that topical PDT is not a promising treatment for disseminated superficial actinic porokeratosis.