Porokeratosis of Mibelli following bone marrow transplantation

BACKGROUND: Porokeratosis is a rare disorder of keratinization with both autosomal dominant and acquired forms. Immunosuppression has been associated with the development of porokeratosis in numerous case reports and series. To our knowledge, however, only five cases of porokeratosis have been reported following bone marrow transplantation. RESULTS: We report five cases of porokeratosis of Mibelli following bone marrow transplantation. The diagnosis of porokeratosis was made between 1 and 13 years post-transplantation. The underlying malignancy in four of the five cases was leukemia, while the fifth patient had non-Hodgkin's lymphoma. Porokeratosis developed during remission in the four leukemia patients, whereas, in the fifth patient, it occurred during a relapse of lymphoma. CONCLUSIONS: Porokeratosis may develop following bone marrow transplantation. Our five cases double the number reported in the medical literature, and the incidence of porokeratosis following bone marrow transplantation may be significantly higher than previously recognized. As cutaneous carcinomas have been reported in association with porokeratosis, careful surveillance for porokeratosis in bone marrow transplant recipients is warranted.     

Mibelli's porokeratosis after bone marrow transplantation]

A 24-year old male patient developed, on both legs, lesions typical of Mibelli's porokeratosis 22 months after bone marrow transplantation, under treatment with cyclosporin A. He denied any family history. Mibelli's porokeratosis seldom develops after an immunosuppressive treatment, and to our knowledge it has exceptionally been described after bone marrow transplantation. A possible complication of Mibelli's porokeratosis is the development of Bowen's disease, basal or squamous cell carcinomas. Immunosuppressive treatment might facilitate the degeneration. For this reason, these subjects should be periodically and carefully examined.     

POROKERATOSIS IN IMMUNOSUPPRESSED AND NONIMMUNOSUPPRESSED PATIENTS

Porokeratosis is an uncommon, inherited, autosomally dominant disorder. In the last decade association of porokeratosis and immunosuppression has been observed. In this study we carried out a comparative study between immunosuppressed and nonimmunosuppressed porokeratosis cases. We found that 9 out of 20 cases of porokeratosis were associated with organ transplantation/immunosuppression. Clinicopathologic study revealed that the pattern of disease is alike both in immunosuppressed and nonimmunosuppressed patients. Our observations indicate that immune modulation could be a factor in the genesis of porokeratosis.     

Porokeratosis of Mibelli after renal transplantation

A 50-year-old woman, who probably had pre-existing quiescent porokeratosis of Mibelli, suffered an abrupt and extensive eruption of porokeratosis in a systematized pattern 2 weeks after renal transplantation, while being treated with prednisolone and azathioprine.     

Disseminated Superficial Porokeratosis in a Patient with Chronic Liver Disease

A 55-year-old male suffering from liver cirrhosis presented with diffuse annular hyper-keratotic papules of abrupt onset on the trunk and extremities. Histopathologic examination revealed cornoid lamella and eosinophilic spongiosis. He did not receive any medications other than cephalosporin for spontaneous bacterial peritonitis. A review of the literature revealed that three cases developed porokeratosis when their liver function declined and that, in one case, the porokeratosis disappeared spontaneously with liver transplantation. Although the precise mechanism is unclear, there is evidence demonstrating immunoincompetence in cirrhosis. Even though we did not perform immunologic studies or exclude the possibility of drug-induced porokeratosis in our case, it is conceivable that porokeratosis can be triggered by immunosuppression due to liver cirrhosis per se.     

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.     

Efficacy of Acitretin for Porokeratosis in a Child with Chronic Cutaneous Graft Versus Host Disease

Abstract: Porokeratosis is a rare disorder of epidermal keratinization that is regarded as a precancerous. Recipients of hematopoietic stem cell transplantation (HSCT) have a greater risk of skin cancer; chronic graft versus host disease (GVHD) is an additional risk factor. A 16‐year‐old boy who had received HSCT for acute myelogenous leukemia was referred to us for sclerodermoid chronic cutaneous GVHD. Two years later, he developed disseminated porokeratosis with a few atypical lesions. Despite cryotherapy, numerous lesions of porokeratosis recurred rapidly. Acitretin resulted in good clinical response and reduced the rate of onset of new lesions.     

Immunosuppression-induced porokeratosis of Mibelli: complete regression of lesions upon cessation of immunosuppressive therapy

Porokeratosis developing subsequent to immunosuppressive therapy is a common and well recognized phenomenon. A previously reported case of porokeratosis showed complete remission of the lesions following discontinuation of immunosuppression. A second example of porokeratosis is presented, in which lesions appeared 2 years after bone marrow transplantation, and completely regressed after immunosuppressive therapy was stopped.     

Facial superficial porokeratosis

A case of exclusively facial superficial porokeratosis occurring in a young man is reported. These lesions were asymptomatic and remarkable because of their location. Histopathology revealed a typical superficial porokeratosis. Exclusively facial porokeratosis is an unusual clinical presentation. To our knowledge, it is the second report of a case with exclusively facial involvement.     

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.     

伴炎症反应的汗孔角化病三例

汗管角化症是一种少见的慢性角化性皮肤病,以中央轻度萎缩边缘堤状角质嵴围绕的皮损为特征。组织病理学特征为角样板层。本病一般无临床症状,但伴明显瘙痒的汗孔角化症已经有文献报告,并被命名为“发疹性瘙痒性丘疹型汗管角化症”或“处于炎症反应期的DSP”。笔者近期诊断三例这样的病患并推荐使用“发疹性瘙痒性丘疹型汗管角化症”来命名这一特殊类型的汗孔角化症。     

Giant porokeratosis

Porokeratosis is a disorder of keratinization characterized by annular plaques with an atrophic center surrounded by a raised, keratotic wall. It has several clinical forms including a porokeratosis of Mibelli, giant porokeratosis, linear porokeratosis, disseminated superficial actinic porokeratosis, palmoplantar porokeratosis and punctate porokeratosis. We report a patient with the rare condition of giant porokeratosis. Several variants of porokeratosis coexist in our patient.     

Squamous cell carcinoma in a renal transplant recipient with linear porokeratosis.

A 40-year-old man developed squamous cell carcinoma on a perianal lesion of linear porokeratosis after renal transplantation. The tumor metastasized to the left inguinal lymph node 25 months after the primary tumor was excised. p53 overexpression was observed in the tumor cells, but not in the porokeratotic lesion. Interestingly, continuous subcutaneous infusion of peplomycin for the lymph node metastasis significantly improved the warty lesions of porokeratosis. In this patient, immunosuppressive agents might have accelerated the development of carcinoma on a skin area with malignant potential.     

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.     

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.     

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.     

Disseminated Epidermolytic Acanthoma with Disseminated Superficial Porokeratosis and Verruca Vulgaris in an Immunosuppressed Patient

A 40-year-old man who had received long term immunosuppressive treatment for 14 years following kidney transplantation developed multiple skin lesions on both antecubital fossae, scalp, and both lower extremities. Histopathologic findings from three skin regions revealed characteristic features of epidermolytic hyperkeratosis, verruca vulgaris, and disseminated superficial porokeratosis, respectively. Although immunocompromised individuals may demonstrate verruca vulgaris or porokeratosis, disseminated epidermolytic acanthoma (DEA) has not been reported to be associated with immunosuppressed status. We suggest that immunosuppression may play a role in the pathogenesis of DEA, as shown in our case.     

Disseminated superficial actinic porokeratosis*

A family is analysed in which disseminated superficial actinic porokeratosis (DSAP) occurred in five members. All three children of one generation are affected. Clinical, histopathological and genetic aspects of DSAP are discussed. Disseminated superficial actinic porokeratosis appears to be a clinical variant of porokeratosis of Mibelli. Since DSAP is a genodermatosis inherited as an autosomal dominant it can occur in any geographical location; however excess sunlight can definitely exacerbate the condition.     

Giant verrucous porokeratosis of Mibelli mimicking psoriasis in a patient with psoriasis

Porokeratosis of Mibelli is a disorder of epidermal keratinization characterized by annular plaques with an atrophic center surrounded by a keratotic wall. We report a case of a giant verrucous porokeratosis of Mibelli mimicking psoriasis that developed in a patient with psoriasis and therefore went unrecognized for a long time. Histologically the lesion combined features of porokeratosis at the periphery and of psoriasis at its center, a picture recently described as "psoriasis encircled by porokeratosis." The possible pathogenetic relationship between psoriasis and the development of porokeratosis is also discussed.     

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.