Progressive multifocal leukoencephalopathy (PML) is a rare opportunistic infection of the central nervous system (CNS) caused by the JC (John Cunningham) virus. This virus is common and generally innocuous in an immunocompetent host, but in immunocompromised individuals, the JC virus causes CNS demyelination that is usually irreversible and fatal. The incidence and reported deaths due to PML increased significantly with the onset of the AIDS epidemic. Also, PML has been reported in the setting of iatrogenically induced immunosuppression, most recently in 3 patients with psoriasis treated with efalizumab. While PML is rare, it must be considered in any immunosuppressed patient presenting with neurologic deficits. Dermatologists using immunosuppressant agents should have a very low threshold to involve neurologists in the care of these patients.
Sclerotic chronic graft-vs-host disease (cGVHD) is an uncommon but debilitating consequence of allogeneic hematopoietic stem cell transplantation. Because a full-thickness incisional biopsy specimen from the level of fascia may be needed to make a definitive diagnosis, a noninvasive diagnostic and assessment technique would be of value. In this case series, Clark et al use magnetic resonance imaging (MRI) to evaluate cGVHD with subcutaneous or fascial involvement and suggest that MRI may be useful in the research setting for serial long-term monitoring of patients with sclerotic soft-tissue disease.
Xeroderma pigmentosum (XP) is a rare genodermatosis characterized by DNA repair defects and an increased frequency of cutaneous malignant neoplasms, including melanoma. Primary cancer prevention strategies focus on education and aggressive UV radiation protection. Secondary measures focus on early detection strategies. In this case report, Green et al describe a 39-year-old woman with XP who developed 38 primary melanomas. Monthly monitoring using total-body cutaneous examinations, total-body photography, and dermoscopy allowed early detection of her melanomas. All melanomas were treated using wide local excision, and she has had no evidence of local or in-transit metastases.
Although full-body skin examinations (FBSEs) have been demonstrated to identify melanomas at an earlier stage than would be found otherwise, the evidence remains insufficient to recommend routine screening. In this retrospective analytical case series, Kantor and Kantor demonstrate that in a Florida dermatology practice, most melanomas were found as a result of dermatologist-initiated FBSE rather than patient complaint. These data bolster the case that FBSE may minimize the substantial public health and financial impact of melanoma.
Neurofibromatosis type 1 (NF-1) is a common multisystem autosomal disorder, the clinical diagnostic criteria for which include cutaneous findings of 6 or more café au lait macules (CALMs), 2 or more neurofibromas of any type or 1 plexiform neurofibroma, and axillary or inguinal freckling. Infants and young children most frequently exhibit only CALMs and cannot be diagnosed as having NF-1 until a second feature develops. In this retrospective study, Nunley et al demonstrate that most patients with 6 or more CALMs will eventually meet the diagnostic criteria for NF-1. The likelihood increases with increasing number and typical morphologic characteristics of CALMs. These data support the practice of referring children with 6 or more CALMs to specialists experienced in the care of children with NF-1.
This Month in Archives of Dermatology. Arch Dermatol. 2009;145(8):865. doi:10.1001/archdermatol.2009.178