Pityriasis rubra pilaris (PRP) is a papulosquamous disorder comprising 6 clinical types. Some factors – including abnormal vitamin A metabolism, internal malignancies, autoimmune diseases, infection and trauma – are thought to be involved in the etiology. Recently, human immunodeficiency virus (HIV)-associated PRP has been reported to have distinct clinical features, such as nodulocystic acne and lichen spinulosus alongside PRP. We report here the case of a 38-year-old female with onset of classical PRP after a high fever. Virological studies indicated that the patient had primary systemic cytomegalovirus (CMV) infection and not HIV infection. Our case suggested that primary CMV infection might have triggered typical PRP that was clinically different from HIV-associated PRP.
‘Sarcoidosis-lymphoma syndrome’ is known as an association of sarcoidosis with malignant lymphoma. We report a 56-year-old woman with systemic sarcoidosis who was seropositive for antibody against human T cell lymphoma/leukemia virus type I (HTLV-I). This patient showed integration of HTLV-I proviral DNA within cutaneous sarcoid nodules, but not in peripheral blood mononuclear cells. Neither atypical lymphocytes nor a T cell receptor β1 gene rearrangement were observed in peripheral blood mononuclear cells or in cutaneous nodules, indicating that the patient did not have a smouldering type of adult T cell lymphoma/leukemia. Detection of integration of HTLV-I proviral DNA in cutaneous sarcoid nodules could suggest that the sarcoid nodules might have been generated as a protective response to chronic stimuli of HTLV-I.
Neurofibromatosis type 1 (NF1) caused by a loss-of functional mutation in NF1 encoding neurofibromin is an autosomal dominant disorder characterized by café-au-lait spots, neurofibromas, intertriginous freckles, and Lisch nodules. Segmental neurofibromatosis (SN) represents a postzygotic mutation and loss of heterozygosity in neurofibromin. SN occurring in the elder persons may be associated with internal malignant tumors. Here, we reported a case of 58-year-old woman with concomitant occurrence of SN and lung adenocarcinoma. The onset of SN in aged persons would be a sign of concomitant occurrence of internal malignant tumors
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