Switching from Tacrolimus to Sirolimus Halts the Appearance of New Sebaceous Neoplasms in Muir-Torre Syndrome

Little is known about the effects of immunosuppression on patients with hereditary nonpolyposis colorectal cancer (HNPCC). We describe a kidney transplant recipient with unrecognized Muir-Torre syndrome in whom the administration of a tacrolimus-based regimen led to the eruption of multiple sebaceous tumors. The patient was later found to harbor an MSH2 mutation. Switching to a sirolimus-based regimen resulted in arrest of the disease. When the patient was switched back to tacrolimus, new facial lesions rapidly appeared. Switching again to sirolimus resulted again in halting the appearance of new lesions. This finding is in line with the known antiangiogenic activity of sirolimus and reports on the regression of cutaneous Kaposi's sarcoma in kidney transplant recipients switched from another immunosuppressive regimen to sirolimus. Further studies on the potential use of sirolimus for the treatment of de novo tumors in immunosuppressed kidney transplant recipients with HNPCC are warranted.     

Grover disease and bullous pemphigoid: a clinicopathological study of six cases

Grover disease (GD) is an idiopathic dermatosis that typically manifests as itchy papules over the trunk in middle‐aged men. Bullous pemphigoid (BP) is an autoimmune bullous disease that affects older people. Not only are the two diseases easily distinguishable on clinical grounds, they are also characterized by differences in histopathology, pathogenesis and response to treatment Thus, the co‐occurrence of these two conditions in the same patient is usually considered coincidental. In this report, we present a multicentre retrospective analysis of six patients who developed both GD and BP over a short period of time, and in all cases but one, GD preceded BP. We discuss the clinical and histopathological features of these patients, and the suggested mechanisms of the diseases. We conclude that GD might predispose to the development of BP.     

Juvenile mycosis fungoides diagnosed before 18 years of age

The literature regarding mycosis fungoides in children is sparse. To shed further light on the characteristics of mycosis fungoides in the paediatric population we analysed the clinicopathological features of 10 patients in whom this malignancy was diagnosed before the age of 18 years. All were Jews and Arabs with histologically proven patch/early plaque stage disease: 4 in stage IA, 4 in IB and 2 with unilesional disease. Seven patients had hypopigmented lesions either constituting the sole manifestation (2 patients) or in combination with classic lesions (5 patients); of these, 3 had light skin and 4 pigmented skin. Most patients had immunohistochemical features characteristic of mycosis fungoides, with a predominance of CD4+ T cells. Some had deletion of CD7+ cells. In 3 patients, however, the epidermotropic cells were exclusively or predominantly CD8+ cells. All patients responded to conventional therapy and during an average follow-up of 3.4 years only one patient showed stage progression, but without extracutaneous involvement. It is concluded that juvenile mycosis fungoides is characterized by early stage disease, occasionally with unilesional disease, usually with hypopigmented lesions irrespective of skin colour, and a good response to therapy. On the basis of our experience and review of the literature, it appears that the CD8+ phenotype is over-represented in juvenile disease.     

Differential expression of proliferation- and apoptosis-related markers in lentigo maligna and solar keratosis keratinocytes

Keratinocytes influence the number, morphology, and proliferation of melanocytes. An interference in the melanocyte-keratinocyte relationship may contribute to melanoma development. This study examined the expression of apoptotic and proliferative markers in keratinocytes in lentigo maligna to characterize the epidermis permissive to these lesions. Formalin-fixed and paraffin-embedded tissues from 25 samples of lentigo maligna, 20 samples of solar keratoses, and 5 samples each of normal sun-exposed and non-sun-exposed skin (controls) were immunostained with antibodies directed against the proapoptotic markers bax and p53, the antiapoptotic marker bcl-2, and the proliferation marker ki-67. Eight percent of the lentigo maligna samples were positive for keratinocyte expression of bcl-2, 24% were positive for p53, and 76% were positive for bax; respective findings for solar keratoses were 35%, 85%, and 90%. Comparison with normal sun-exposed skin yielded lower rates of keratinocyte proliferation in 56% of the lentigo maligna samples, similar rates in 36%, and higher rates in 8%; for solar keratoses, proliferation was higher than controls in 60% of samples, similar in 35%, and lower in 5%. All these differences were statistically significant. These findings indicate that there are variable patterns of epidermal reaction to chronic sun exposure. The epidermis in lentigo maligna shows overall low proliferation and an apparently low apoptotic tendency. The dysfunctional epidermis may be permissive to aberrant melanocyte proliferation in the early stages of melanoma development.     

Carotid artery stenosis: clinical efficacy of two-dimensional time-of-flight MR angiography.

To assess the clinical efficacy of two-dimensional time-of-flight magnetic resonance (MR) angiography in the evaluation of carotid artery stenosis, a group of patients was evaluated in which 73 vessels were studied with both MR and conventional angiography. Four experienced neuroradiologists each scored both the MR and conventional angiograms in a blinded manner by using a standardized scoring scheme. Comparison of the scores revealed a high degree of correlation. In particular, MR angiography served to discriminate reliably between mildly narrowed and severely narrowed or occluded vessels. Furthermore, severe stenoses were accurately discriminated from occlusions in all cases. MR angiography is a robust and accurate modality for the characterization of carotid artery stenosis. It is useful in conjunction with routine MR imaging of the brain in the evaluation of the patient with suspected carotid arterial disease.     

Idiopathic palmoplantar eccrine hidradenitis in children

 Idiopathic palmoplantar eccrine hidradenitis (IPPH) is a recently described disorder characterized by painful erythematous plantar nodules and in three cases, showed a typical neutrophilic infiltrate around and within the eccrine sweat apparatus. Five cases of IPPH on the soles of the feet in healthy children are reported. The disorder presented after intense physical activity in four cases. The course was benign and self-limiting. Complete bed rest for several days without any medical therapy led to alleviation of the pain and disappearance of all the lesions. Conclusion Idiopathic palmoplantar eccrine hidradenitis may be more common than reported. Paediatricians should be aware of it in order to avoid unnecessary diagnostic tests and treatments. Received: January 2000 / Accepted: 22 March 2000