Porokeratosis and immunosuppression

Bencini, Pier Luca; Crosti, C.; Montagnino, Giuseppe; Sala, F.

doi:10.1016/s0190-9622(86)80462-5

Cited by 25 publications

(16 citation statements)

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“…In such tumors, allelic loss may originate from a somatic event of recombination, gene conversion, point mutation, deletion, or nondisjunction (1). Because those with the linear type of porokeratosis appear to be particularly prone to develop carcinoma, it seems reasonable to assume that the LOH giving rise to this disorder represents the initial step in the multistage mutational process of carcinogenesis (1,3,4,23–25).…”

Section: Discussionmentioning

confidence: 99%

Linear Porokeratosis Associated with Disseminated Superficial Actinic Porokeratosis: A New Example of Type II Segmental Involvement

Boente¹,

López‐Baró²,

Frontini³

et al. 2003

Pediatric Dermatology

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The coexistence of linear porokeratosis (LP) and disseminated superficial actinic porokeratosis (DSAP) in a 3-year-old girl with a family history of DSAP is presented. Happle proposed loss of heterozygosity (LOH) to explain the origin of this unusual phenomenon. Homozygosity would explain why lesions in LP are far more pronounced than those of the associated heterozygous DSAP lesions. LOH would also explain the early age of presentation of the linear lesions, the family history of DSAP, and why LP cases are particularly prone to malignant transformation. This case is also important for molecular studies because of the presence of heterozygous and homozygous mutated cells in the same patient and the familial occurrence of the heterozygous form of the disease.

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Section: Discussionmentioning

confidence: 99%

Linear Porokeratosis Associated with Disseminated Superficial Actinic Porokeratosis: A New Example of Type II Segmental Involvement

Boente¹,

López‐Baró²,

Frontini³

et al. 2003

Pediatric Dermatology

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“…It has been suggested that immunosuppression might trigger the expression of a mutant clone, either directly or by interfering with epidermal maturation 2 . One study postulated that epidermal Langerhans cells in lesions of transplant patients have decreased expression of HLA‐DR antigens, which could lead to defective immunosurveillance and hence the development of porokeratosis 3 . No particular immunosuppressive drug has been proven to prevent or reduce the risks of porokeratosis.…”

Section: Discussionmentioning

confidence: 99%

Porokeratosis in Singapore: An Asian perspective

Tan

Chong

2012

Aust J Dermatology

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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.

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“…Most authors agree that therapeutic immunosuppression seems to cither trigger or aggravate DSAP in genetically predisposed patients [3][4][5][6][7]. How immunosuppressive therapy acts in the promotion of DSAP lesions is not fully understood.…”

Section: Disseminated Porokeratosis and Myelodysplastic Syndromementioning

confidence: 99%