A 75-Year-Old Man With Progressive Generalized Erythroderma and History of Anaplastic Large Cell Lymphoma: Challenge

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A 75-year-old Philippino man with a history of anaplastic large-cell lymphoma in remission was referred to the University of Virginia Department of Dermatology for the evaluation of an intensely pruritic widespread eruption that he had had for 1 year. The rash had begun on his back and subsequently spread to his chest, scalp, arms, and legs (Fig. 1). His medications, which he had been taking for many years before the development of the rash, included amlodipine, atenolol, hydrochlorothiazide, atorvastatin, enalapril, metformin, montelukast sodium, glucosamine, vitamins, calcium, and aspirin. All of these medications had been discontinued for a couple of months without improvement in the rash. The rash cleared with prednisone, leaving only postinflammatory hyperpigmentation but recurred when prednisone was discontinued (Fig. 2). At the time of his referral, he was being maintained on 20 mg of prednisone a day. Triamcinolone cream for the rash and betamethasone valerate for the scalp were not helpful.
On physical examination, the patient was well appearing but visibly uncomfortable because of the intense itching. He had a widespread eruption consisting of reddish brown dermal papules and plaques without scale, involving his back, chest, scalp, arms, and legs (Fig. 3). There were some areas of postinflammatory hyperpigmentation where some lesions had resolved. There was marked sparing of the axillae and skinfolds on the abdomen, clinically consistent with the “deck-chair sign.” Laboratory studies were unremarkable except for a slight neutrophilia and eosinophilia.
A biopsy of the lesion showed epidermal acanthosis, with a vague psoriasiform pattern of hyperplasia and a moderate degree of spongiosis (Fig. 4). Within the dermis, there was a superficial and perivascular, lymphocytic, inflammatory infiltrate with many dermal eosinophils. The lymphocytes were predominantly small to medium in size, but a few of them were larger, with vesicular nuclei and prominent nucleoli. Additionally, many of the large cells showed mitotic figures. Immunostains were performed to further characterize the cells, and particularly given the clinical history of anaplastic large-cell lymphoma. CD3 was positive in most of the lymphocytes, indicating the presence of numerous T cells. CD4 was positive in most of the T cells, whereas CD8 stained a much smaller population of T cells (Fig. 5). The CD4:CD8 ratio was increased (approximately 8:1). CD7 showed partial loss of staining among the T cells (approximately 30% loss). CD30 was positive in scattered clusters of large cells (possibly immunoblasts). T-cell receptor gene rearrangement studies (TCR beta) showed a polyclonal population of T cells.

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