Porokeratosis of Mibelli following bone marrow transplantation

Abstract

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.

Pustular porokeratosis

“…DSAP occurring in the setting of immunosuppression, including postorgan transplantation, for inflammatory conditions including pemphigus vulgaris, and human immunodeficiency virus‐acquired immune deficiency syndrome (HIV‐AIDS), is well‐described in the literature. 6,8–11 One case report describes a patient with rapid‐onset DSAP immediately after solid organ transplant induction therapy; 12 our patient provided a similar history for his skin lesions, which he described as appearing ‘overnight’ after increases in azathioprine and prednisone.…”

Genitogluteal porokeratosis: An unusual clinical presentation

“…Studies have highlighted the negative psychological impact of this dermatosis, particularly upon the patients' personal relationships 5 . Histopathologically, no significant differences between porokeratosis in immunosuppressed and immunocompetent individuals have been observed; 9 and to our knowledge, there is only one recent report of GP in an immunocompromised patient 8…”

“…For example, GP has been observed following organ and bone marrow transplantation, immunosuppressive chemotherapy, radiotherapy, treatment with systemic corticosteroids and in the setting of HIV infection. The mechanism is likely to be a loss of immunosurveillance, allowing a proliferation of abnormal keratinocyte clones 9 . Overall, however, the aetiology of GP remains uncertain.…”

Eruptive angiokeratomas and porokeratosis in the setting of sclerodermatous graft‐vs.‐host disease

“…Other entities that have been shown to occur after transplants include angiokeratomas and porokeratosis. However, although there have been reports of angiokeratomas and porokeratosis occurring with GVHD, it is exceedingly rare for all three entities to occur simultaneously.…”

“…Porokeratosis can manifest post bone marrow transplantation and appears clinically as an annular plaque with an atrophic center and hyperkeratotic edges . Histologically, there is a column of parakeratotic cells (cornoid lamella) with an underlying absent granular cell layer and apoptotic keratinocytes (civatte bodies) at its base.…”