Faculty Bibliography

HTLV-1 is a virus that is endemic in southwesternJapan and the Caribbean and has been implicatedin the development of ATLL. ATLL, which is anuncommon malignant condition of peripheralT-lymphocytes, is characterized by four clinicalsubtypes, which include acute, lymphomatous,chronic, and smoldering types, that are based onLDH levels, calcium levels, and extent of organinvolvement. We present a 52-year- old woman withpruritic patches with scale on the buttocks and withtender, hyperpigmented macules and papules oftwo-years duration. Histopathologic examinationwas suggestive of mycosis fungoides, laboratoryresults showed HTLV-I and II, and the patient wasdiagnosed with primary cutaneous ATLL. We reviewthe literature on HTLV-1 and ATLL and specifically theprognosis of cutaneous ATLL. The literature suggeststhat a diagnosis of ATLL should be considered amongpatients of Caribbean origin or other endemicareas with skin lesions that suggest a cutaneousT-cell lymphoma, with clinicopathologic features ofmycosis fungoides. Differentiation between ATLLand cutaneous T-cell lymphoma is imperative as theyhave different prognoses and treatment approaches.

Diffuse intrinsic pontine glioma (DIPG) is a fatal brain cancer that arises in the brainstem of children, with no effective treatment and near 100% fatality. The failure of most therapies can be attributed to the delicate location of these tumors and to the selection of therapies on the basis of assumptions that DIPGs are molecularly similar to adult disease. Recent studies have unraveled the unique genetic makeup of this brain cancer, with nearly 80% found to harbor a p.Lys27Met histone H3.3 or p.Lys27Met histone H3.1 alteration. However, DIPGs are still thought of as one disease, with limited understanding of the genetic drivers of these tumors. To understand what drives DIPGs, we integrated whole-genome sequencing with methylation, expression and copy number profiling, discovering that DIPGs comprise three molecularly distinct subgroups (H3-K27M, silent and MYCN) and uncovering a new recurrent activating mutation affecting the activin receptor gene ACVR1 in 20% of DIPGs. Mutations in ACVR1 were constitutively activating, leading to SMAD phosphorylation and increased expression of the downstream activin signaling targets ID1 and ID2. Our results highlight distinct molecular subgroups and novel therapeutic targets for this incurable pediatric cancer.

A 76-year-old woman with a history of eczematous dermatitis presented with a 9-month history of a pruritic, eczematous eruption of the trunk. This eruption responded to topical glucocorticoids but recurred on discontinuation of treatment on multiple occasions. A biopsy specimen showed extragenital lichen sclerosus et atrophicus. The diagnosis of extragenital lichen sclerosus is reviewed with a comparison to classic lichen sclerosus in terms of clinical features, histopathologic characteristics, and treatment approaches

Allergy to colophony is well noted in the literature, however, there have been few case reports of allergic contact dermatitis to propolis in musicians and instrument makers. We report a case of a stringed instrument craftsman who developed allergic contact dermatitis to propolis, a component of Italian varnish. A review of the components, applications, and the clinical manifestations of hypersensitivity reactions to propolis are presented

BACKGROUND: Data on the relative frequency of the various forms of primary cutaneous lymphomas (PCLs) are largely limited to European institutions. OBJECTIVE: Our purpose was to document the relative frequencies of various PCLs seen at 3 US institutions with active cutaneous lymphoma programs and to compare those with the European data. METHODS: Included in this study are newly registered patients seen at MCP Hahnemann University, New York University, and the University of California, San Francisco from July 1, 1995 to June 30, 1998. RESULTS: A total of 755 patients were seen. The frequency distribution of the major diagnostic groups was as follows: mycosis fungoides/Sezary syndrome, 82.3%; lymphomatoid papulosis, 12.6% (including patients with associated mycosis fungoides/Sezary syndrome); CD30(+) anaplastic large-cell lymphoma, 0.9%; peripheral T-cell lymphomas, 2.9%; B-cell lymphoma, 4.5%. CONCLUSION: The most striking finding is the much lower relative frequency of primary cutaneous B-cell lymphomas at US institutions (4.5%) versus the approximately 20% reported by European groups. The reason for this difference requires further study