Porokeratosis of Mibelli Following Heart Transplant

Abstract

A 59-year-old white man with a history of Sydenham's chorea received a mitral valve prosthesis in 1962. He sustained an anterolateral myocardial infarction in 1983. In 1984, he received a heart transplant. To prevent heart rejection, he was initially treated with cyclosporine 12 mg/kg/day and prednisone 90 mg b.i.d. In 1987, azathioprine 100 mg daily was added. In 1989, at the time of our evaluation, his medications included cyclosporine 80 mg b.i.d., prednisone 10 mg b.i.d., and azathioprine 75 mg/day. Since his heart transplant surgery he had not taken any thiazide medication. The patient noted a lesion on his right thigh; the lesion appeared in 1986, 2 years after his heart transplant. On examination in 1989, the lesion was a 2 cm wide annular plaque with a shiny atrophic center and raised border. Both the clinical appearance and pathology were consistent with a diagnosis of porokeratosis of Mibelli. No family history of porokeratosis was elicited.

Disseminated superficial actinic porokeratosis in black skin

“…5 Immunosuppression may be a factor in the extension or onset of DSAP. [9][10][11][12][13][14][15][16] It is proposed that immunosuppressants, which would include ultraviolet (UV) light and chemotherapeutic agents, promote porokeratosis via impairment of the Langerhans cells' immune surveillance function. 9 A role for UV light in the pathogenesis of DSAP is supported by the fact that fair-skinned individuals are most commonly affected; lesions rarely arise in dark-skinned individuals who have natural protection against sunlight.…”

Superficial disseminate porokeratosis in a patient with myelodysplastic syndrome

“…Immunosuppression might aid in the phenotypic expression of disseminated superficial actinic porokeratosis, and perhaps superficial disseminated porokeratosis and other forms of PK, in genetically predisposed individuals by two proposed mechanisms: (i) directly triggering expression of an abnormal clone of keratinocytes (4,5); or (ii) disrupting growth dynamics in the epidermis which promotes the proliferation of an abnormal clone. 4 , 5 , 6 It is thought that immunosuppressive drugs and ultraviolet light promote the development of PK in renal allograft patients by decreasing the density and impairing the immune surveillance function of epidermal Langerhans cells. 1 , 3 , 7 There is further speculation that epidermal Langerhans cells in PK have defective expression of HLA‐DR antigens which results in failure of their immunosurveillance function and aids in the development of abnormal clones of keratinocytes.…”

Bullous and Pruritic Variant of Disseminated Superficial Actinic Porokeratosis: Successful Treatment with Grenz Rays

“…Reed and Leone [20]have suggested that in a genetically predisposed individual, the disease results from the proliferation of abnormal clones of epidermal cells, which may be triggered by several stimuli, including sunlight or artificial UV light [18, 21, 22], exposure to electron beam radiotherapy [23], immunosuppressive treatments (as e.g. chemotherapy, immunosuppressive drugs or systemic corticosteroids) [4, 5, 6, 7, 8, 9, 10]or HIV infection [11]. A pathogenetic relationship between a latent virus infection and porokeratosis has been hypothesized, although never demonstrated.…”

“…Therefore clinical surveillance of patients is indicated when there is a history of intense sun exposure, X-ray irradiation or immunosuppressive disorders [4, 6, 18, 19, 21, 24, 25, 26, 27]. …”

“…The histopathological characteristic is the so-called cornoid lamella (a column of parakeratotic cells). Porokeratosis has also been previously reported in association with: drug-induced immunosuppression [4]; organ transplantation [5, 6, 7]; hematological malignancies [8, 9]; autoimmune disorders [10], or human immunodeficiency virus (HIV) infection [11]. …”