Faculty Bibliography

Intravascular lymphoma (IVL) is a rare subtype of extranodal diffuse large B-cell lymphoma in the World Health Organization classification. Although the majority of cases are of B-cell lineage, cases of IVL with a T-cell phenotype and, rarely, histiocytic and natural killer (NK)-cell phenotypes have been reported. We report a case of T-cell IVL with a cytotoxic phenotype. A 62-year-old male presented with erythematous patches and plaques on the lower extremities, and a biopsy revealed IVL with an activated cytotoxic phenotype (CD56(+), perforin+, granzyme B+, TIA-1+, CD3epsilon(+), CD20(-), CD4(-), CD8(-), CD5(-), and T-cell receptor [TCR] betaF1(-)), consistent with either NK-cell or T-cell origin. TCR gene analysis showed a monoclonal T-cell population, supporting the diagnosis of a T-cell IVL. Although the patient's skin lesions were refractory to combination chemotherapy and salvage chemotherapy regimens, there has been no evidence of disease progression in 24 months of follow-up.

There are T cells within normal, noninflamed skin that most likely conduct immunosurveillance and are implicated in the development of psoriasis. We isolated T cells from normal human skin using both established and novel methods. Skin resident T cells expressed high levels of CLA, CCR4, and CCR6, and a subset expressed CCR8 and CXCR6. Skin T cells had a remarkably diverse TCR repertoire and were mostly Th1 memory effector cells with smaller subsets of central memory, Th2, and functional T regulatory cells. We isolated a surprising number of nonexpanded T cells from normal skin. To validate this finding, we counted T cells in sections of normal skin and determined that there are approximately 1 x 10(6) T cells/cm(2) normal skin and an estimated 2 x 10(10) T cells in the entire skin surface, nearly twice the number of T cells in the circulation. Moreover, we estimate that 98% of CLA(+) effector memory T cells are resident in normal skin under resting conditions. These findings demonstrate that there is a large pool of memory T cells in normal skin that can initiate and perpetuate immune reactions in the absence of T cell recruitment from the blood.

BACKGROUND: For nearly a century, the terms "keloid morphea" and "nodular scleroderma" have been used interchangeably without defined clinical or histologic criteria. OBJECTIVE: To define the conditions "keloid morphea" and "nodular scleroderma" by correlating the clinical and histologic features. METHODS: We retrospectively identified six patients with keloidal lesions and nodules from 70 consecutive patients with scleroderma seen in the dermatology clinic. The clinical presentation and histopathological findings were reviewed. RESULTS: Six of 70 patients with scleroderma (45 systemic and 25 morphea) exhibited keloidal or nodular lesions. All these patients had systemic sclerosis. Clinically one patient (case 1) had nodules; five (cases 2-6) had keloids. The nodular lesions had histologic findings consistent with keloid, while the keloidal plaques were variably keloids or morphea histopathologically. There was no correlation between the clinical morphology and the histologic findings, except for cases 5 and 6. These patients were African-American, with a family history of keloids and typical keloids clinically and histologically that developed from sites with normal skin. CONCLUSION: Keloid morphea and nodular scleroderma are clinical terms that describe keloidal and nodular lesions in patients most commonly with scleroderma. The clinicopathologic association is variable. Based on a review of the English literature and our series of six patients, we identified two clinical variants; (1) keloidal or nodular lesions arising from sclerodermatous skin with histologic findings of keloid or scleroderma, (2) typical keloids clinically and histologically, arising in normal skin in patients with a family history of keloids. Awareness of these entities is important for proper diagnosis of the cutaneous lesions and for recognizing that the cutaneous findings may be a sign of systemic sclerosis

Ki-1 (CD30) positive anaplastic large cell lymphoma (ALCL) is an uncommon, high-grade non-Hodgkin's lymphoma with distinct morphologic and immunohistochemical features. Solitary or multiple ulcerated nodules typically characterize cutaneous involvement. Erythroderma is very rare. We report the first case of primary nodal ALCL in which disease relapse manifested as generalized papules, nodules, and erythroderma. This unusual case expands the spectrum of cutaneous disease associated with Ki-1 positive ALCL and highlights the need for early biopsy in patients with a history of Ki-1 positive ALCL who have skin lesions develop.