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.     

Eruptive superficial porokeratosis in a patient with nephrotic syndrome

A 34-year-old female was referred to our department, complaining of multiple asymptomatic lesions that appeared two weeks previously. The patient had active nephritis with nephrotic syndrome and was treated with immunosuppressive therapies. Physical examination revealed multiple well-circumscribed rounds of flat brownish plaques with slightly elevated borders, some of which were covered by scales. The number of lesions was nine in total. Skin biopsy specimens showed dyskeratotic cells in the thinned epidermis with cornoid lamella, and the absence of a granular cell layer. The development of porokeratosis was considered to be related to immunosuppressive therapy or the activity of nephritis.     

Disseminated superficial porokeratosis and immunosuppression

We present a patient who developed skin lesions typical of disseminated superficial porokeratosis (DSP) while on immunosuppressive therapy for pemphigus foliaceus. Phototesting with artificial light sources did not have any effect on the DSP lesions. The literature describing occurrence of DSP on immunosuppression is reviewed and possible pathogenetic mechanisms are discussed.     

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.     

Disseminated Superficial Porokeratosisin a Patient with Gastric Cancer

Disseminated superficial porokeratosis (DSP) is a rare variant of porokeratosis, which is characterized histologically by cornoid lamella and clinically by central atrophy with elevated borders. DSP is usually associated with immunosuppressive states and hematopoietic malignancies, but rarely with malignancies of visceral organs. A 65-year-old male presented with numerous brownish macules with elevated borders on the trunk and limbs that had been present for 1 year. Before the visit to our clinic, gastric cancer was diagnosed at about the same time the skin lesions suddenly increased in size and number. Clinical and histopathological examination revealed that the lesions were consistent with DSP. We herein report a rare case of disseminated superficial porokeratosis that occurred in association with gastric cancer.     

Porokeratosis of Mibelli induced by topical corticosteroid

BACKGROUND: [corrected] Prorokeratosis of Mibelli is a chronic disorder characterised by slightly atrophic plaques surrounded by keratotic border. METHODS AND RESULTS: A 45-year-old with clobetazole propionate ointment for psoriasis over 15 years developed characteristic lesions of porokeratosis Mibelli on the elbows. Histopathological examination revealed the cornoid lamellae located on the edges of the specimen and psoriasiform acanthosis and a spongiotic pustule formation in the center of the specimen; thus, it was suggested as 'psoriasis encircled by porokeratosis'. CONCLUSIONS: Development of porokeratosis may be explained by the local immunosuppressive effect of the prolonged application of the topical steroid.     

Porokeratosis in Singapore: an Asian perspective

Porokeratosis is a rare disorder of skin keratinisation characterised by a cornoid lamella. We reviewed its associations with immunosuppression and phototherapy, as well as the risks of malignant progression. This is a retrospective review on all cases of porokeratosis seen at the National Skin Centre, Singapore, between 2000 and 2010. A total of 94 patients were reviewed. Clinical and histological diagnoses were confirmed in 63% patients. Most patients were Chinese (89%) with a mean age of 51.6 years. The male to female ratio was 1.4:1. The four main clinical variants were classical porokeratosis of Mibelli (56%), disseminated superficial actinic porokeratosis (DSAP) (18%), disseminated superficial porokeratosis (DSP) (11%), and linear porokeratosis (13%). Phototherapy-induced porokeratosis, seen in three patients, is rare. Seven cases of porokeratosis occurred in patients who were immunosuppressed. Progression of porokeratosis to malignancy is uncommon and was observed in three patients. The most common treatments included cryotherapy (26.5%) as well as topical steroids or retinoids (38.1%). A good response, defined as clear or almost clear lesions, occurred in 16% patients. The most common presentation of porokeratosis in our review was a middle-aged male patient with an asymptomatic lesion of porokeratosis of Mibelli over the extremities. No particular immunosuppressive drug was implicated. Porokeratosis associated with ultraviolet phototherapy or malignancy is rare. Progression of porokeratosis to malignancy arose in the disseminated variants, with a possible correlation with age. This is the largest institutional retrospective review of porokeratosis to date and highlights the major epidemiological characteristics of this condition.     

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.     

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 porokeratosis developing after electron-beam total skin irradiation for mycosis fungoides

A 74-year-old man with stage IB cutaneous T-cell lymphoma was treated with electron-beam total skin irradiation in 1988. Seven years later, multiple disseminated lesions of porokeratosis developed on the chest, extremities and abdomen. There was no family histon of porokeratosis, nor history of treatment with PUVA or of excessive sun exposure. Development of disseminated porokeratosis on nonexposed sites suggests a direct role for the previous ionizing radiation. Electron-beam total skin irradiation therapy should therefore he added to the list of possible causative factors in the development of disseminated porokeratosis.     

Porokeratosis: immunosuppression and exposure to sunlight

Four cases of disseminated superficial actinic porokeratosis are described, all in patients who had been given immunosuppressive treatment, which might have been a contributing factor in the development of the condition. The possible relationship of immunosuppressive actinic porokeratosis to viral infection is also discussed.     

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.     

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.     

A Case of Disseminated Superficial Porokeratosis Associated with Giant Porokeratosis in Pregnancy

A 23-year-old pregnant lady presented with dark raised lesions over face, axillae, and upper limbs of 15-day duration. She was 35 weeks pregnant at the time of onset of the lesions. Dermatological examination revealed hyperpigmented plaques on the face and papules with raised borders in the axillae and proximal arms. Skin biopsy from both the lesions revealed a diagnosis of porokeratosis. She was treated with emollients alone and the lesions regressed four weeks following delivery. This case is being reported for the rare occurrence of the combination of disseminated superficial porokeratosis with giant porokeratosis in pregnancy.     

Porokeratosis and immunosuppression

Immunosuppression may favour the development of disseminated superficial porokeratosis (DSP). We report the clinical features and the outcome of DSP in 24 patients receiving immunosuppressive treatment (group A), and compare the characteristics of the disease with those of 13 immuno-competent patients with DSP (group B). The two groups were similar with regard to age, sex, area of skin involvement and mean follow-up. There was a family history of DSP in only two patients in group A, compared with five patients in group B (P= 0.03). The skin type, based on the tanning response to sunlight, was not significantly different between the two groups. Two of the 24 patients in group A had high sun exposure, compared with five of the 13 patients in group B (P = 0.03). Moreover, 10 patients in group A and 11 in group B (P = 0.01) exhibited worsening of the disease after exposure to sunlight, usually during the summertime. These observations appear to support the hypothesis that sun exposure is not always essential for the development of porokeratosis in immunosuppressed patients. None of our patients developed skin cancer in porokeratotic lesions during the follow-up period.     

Disseminated superficial actinic porokeratosis: Appearance associated with photochemotherapy for psoriasis

A patient with chronic psoriasis treated with psoralens plus ultraviolet A (PUVA) developed characteristic lesions of disseminated superficial actinic porokeratosis (DSAP). Since other processes associated with ultraviolet irradiation, including epidermal dysplasia, actinic keratoses, squamous cell carcinomas, and keratoacanthomas, have been reported to result from PUVA, it is possible that her DSAP lesions were induced by this therapy.     

Recurrent porokeratosis

A 63-year-old man developed linear, annular and accuminate lesions which showed the histological features of porokeratosis. The lesions cleared spontaneously, recurred several months later and ulcerated, then cleared again. The spontaneous resolution and recurrence of the lesions is a hitherto undescribed pattern of porokeratosis.     

Multiple porokeratomas (porokeratotic acanthoma) coexisting with disseminated superficial porokeratosis: Clinical,dermoscopic and pathological observations,and review of published work

Porokeratoma is a recently described solitary or multiple tumor-like acanthoma, sharing the histological feature of cornoid lamellae with porokeratosis. Whether porokeratoma is a variant of porokeratosis is controversial. We report a rare case of a 53-year-old Chinese woman who presented with multiple, symmetrical, hemispherical and verrucous plaques on her lower extremities that had been present for 20 years. The clinical manifestation resembled the fungal disease of chromoblastomycosis. The diagnosis of multiple porokeratoma coexisting with disseminated superficial porokeratosis was rendered according to the clinical, dermoscopic and pathological features. Oral acitretin (30 mg/day) and laser therapy were administrated. After 6 months of treatment, the number of plaques on her limbs was significantly reduced without recurrence. From this case, we speculate that porokeratoma is a rare and special variant of porokeratosis. In complex cases with multiple lesions, oral acitretin can be combined with surgery, cryotherapy and laser therapy.     

Superficial disseminated porokeratosis with dermal amyloidosis]

A case of non-actinic disseminated superficial porokeratosis with dermal amyloid deposits in a 53 year-old-man is reported. The lesions of the trunk and arms were typical, but annular lesions of the ankles were lichenoid. The amyloid deposits were present under the cornoid lamella in the typical lesions and absent in the annular lesions. The different clinical variants of porokeratosis are discussed. The origin of amyloidosis is debated; it seems to be epidermic, due to degeneration of the keratinocytes. The secondary cutaneous amyloidosis is usually described in association with epithelial tumors or psoriasis, but exceptionally with porokeratosis.     

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.