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Notes: Background  A 63-year-old man with therapy-resistant Sézary syndrome was enrolled in a multicenter trial of oral bexarotene for advanced-stage cutaneous T-cell lymphoma (CTCL).Methods  Monthly evaluations for efficacy and side-effects were conducted and documented.Results  Gradual improvement in erythema, pruritus, and scale was noted during the initial 16-week trial period and treatment was extended to 40 weeks. From week 20 to week 40, the erythroderma continued to improve and the lymph node burden decreased, but the absolute Sézary cell count inversely increased. By week 40, intractable pruritus and erythroderma abruptly recurred, and bexarotene was discontinued.Conclusions  Bexarotene is well tolerated and can be efficacious in patients with Sézary syndrome. Shifting of Sézary cells between different compartments was noted. Further studies on the interaction between the skin, lymph nodes, and peripheral blood compartments during bexarotene treatment in this subset of patients with CTCL are needed.

Notes: Cutaneous granulomatous vasculitis is an uncommon histopathologic finding that has been associated with lymphoproliferative disorders, systemic vasculitis, autoimmune inflammatory diseases, and infection. To define further the concept of cutaneous granulomatous vasculitis and to emphasize its clinical importance, we reviewed biopsy material from 8 patients seen from 1985 through 1992. All biopsies showed evidence of blood vessel damage with fibrinoid change or hemorrhage (or both) and granulomatous inflammation in and around vessel walls. Special stains for microorganisms were negative in all cases. Associated medical disorders included neuropathy (2 patients), sarcoid-like disease (2), systemic vasculitis (1), lymphoma and suspected lymphoma (1 each), and associated herpes simplex virus (1). T-cell gene-rearrangement studies were negative in a patient with suspected lymphoma. Granulomatous cutaneous vasculitis is most commonly associated with lymphoma and systemic vasculitis. In selected cases, infection should be considered as an underlying cause.

Notes: Background  The clinical mucocutaneous manifestations of glucagonoma syndrome are recognized easily when they occur in the classic pattern of acral or periorificial lesions evolving in recurrent crops, with an annular and migratory distribution, in a patient with diabetes mellitus who has had recent weight loss and anemia. Not infrequently, noncharacteristic clinical and histopathologic features are observed and, in these cases, the diagnosis of pancreatic neoplasm may be delayed.Aim  To review the clinical and histopathologic features of cutaneous manifestations of glucagonoma syndrome.Methods  The clinicopathologic features of 13 patients (eight women) with widespread or localized cutaneous eruption as a manifestation of islet cell pancreatic carcinoma with marked glucagon secretion (glucagonoma) were reviewed.Results  The definitive diagnosis of the cutaneous eruption was established at the time of diagnosis of the pancreatic neoplasm (three patients) or afterwards (10 patients). In nine patients, the mucocutaneous manifestations preceded the diagnosis of the pancreatic neoplasm by 1 month to 3 years (mean, 12 months). In only eight biopsy specimens were the histopathologic features considered to be suggestive or characteristic of necrolytic migratory erythema. Diffuse parakeratosis, that occasionally arose abruptly from normal epidermis, was observed in 12 biopsy specimens. By the time necrolytic migratory erythema was diagnosed, the pancreatic carcinoma had metastasized to the liver, regional lymph nodes, or bone in 12 patients.Conclusion  Increased awareness of the polymorphic mucocutaneous and nonspecific histopathologic features of glucagonoma syndrome is needed to avoid unnecessary delay in the diagnosis of this syndrome.

Notes: Background  When a patient is identified by patch testing as being sensitive to a specific contact allergen, he or she is generally advised to read the product labels and avoid products that contain the specific allergen. Patients are often confronted with difficult chemical names, synonyms, and cross-reactants for individual allergens. At the same time, dermatologists may spend a considerable amount of time trying to educate their patients about the avoidance of these allergens and explaining which products may contain them.Methods  We applied a new educational approach to inform patients about products that are free of their allergens.Results  We present a patient with multiple contact allergens in whom the Contact Allergen Replacement Database was used to educate about specific allergens. This approach has proved to be an invaluable tool for both physicians and their patients in contact allergy counseling.

Notes: Background  2-Chlorodeoxyadenosine (2-CdA), a purine adenosine analog, is safe and effective chemotherapy for patients with hairy cell leukemia and low-grade lymphomas. Adverse effects include neutropenia, lymphocytopenia, and infectious complications. Our objective was to evaluate the efficacy of 2-CdA (2–6 seven-day cycles) in the treatment of late-stage, recalcitrant Sézary syndrome.Methods  Retrospective review of medical records of six patients with Sézary syndrome who had received 2-CdA cycles at Mayo Clinic, Rochester between March 1995 and March 2000. Variables assessed from the records included improvement in global appearance, extent of erythroderma, size of lymph nodes, pruritus, and leukocyte, lymphocyte, and absolute Sézary cell counts.Results  Two patients, both with stage III Sézary syndrome, whose previous treatment consisted of only two modalities, responded well to the treatment, with moderate to total clearing of erythroderma and pruritus associated with a significant decrease in Sézary cell counts. The other four patients had only a partial response (one patient) or no response (three patients) to 2-CdA. The mortality rate was 50%. All three patients died of Staphylococcus aureus sepsis. However, only one patient was receiving 2-CdA treatment when he died. The other two patients died 8 and 9 weeks after the last 2-CdA cycle. This high mortality rate is attributed to infectious complications after 2-CdA treatment in patients with recalcitrant disease.Conclusion  2-Chlorodeoxyadenosine shows efficacy in stage III Sézary syndrome, but it also carries a substantial risk of septic complications and mortality. It can be used if no other suitable alternatives are available. Caution should be exercised in all these patients regarding skin care and avoidance of infections or sepsis.

Notes: A 27-year-old woman presented with a 2-year history of a progressively enlarging, painful ulcer on her right foot. Two years earlier, she had noticed an apparent wart on her right foot. The lesion had been treated with liquid nitrogen. An ulcer developed at the site of treatment and enlarged progressively, becoming so painful that she had difficulty walking. Extensive surgical debridement and closure were unsuccessful in healing the ulcer; the ulcer grew larger and more painful. After an amputation was recommended by her local doctors, the patient sought another opinion.At physical examination, the patient had a painful, 9.5 cm × 5 cm ulcer on the plantar aspect of the right foot (〈link href="#f1"〉Fig. 1). Exuberant, rolled borders were present, and a yellow exudate covered the base of the lesion. The right inguinal lymph nodes were enlarged and firm. A punch biopsy specimen from the ulcer border was examined.〈figure xml:id="f1"〉1〈mediaResource alt="image" href="urn:x-wiley:00119059:IJD1537:IJD_1537_f1"/〉A 27-year-old woman presented with a 2-year history of a progressively enlarging, painful ulcer on the right foot. At physical examination, she had a painful, 9.5 cm × 5 cm ulcer on the plantar aspect of the right footMicroscopic examination of a hematoxylin and eosin preparation of the punch biopsy specimen showed a reasonably well-demarcated neoplasm within the deep reticular dermis down to the dermal-pannicular junction (〈link href="#f2"〉Fig. 2). This proliferation was composed of a population of round cells and spindle cells. The round cells were arranged in nests separated by delicate, fibrous septa, and the spindle cell proliferation was intercalated between collagen bundles. The nuclei of both cell types were uniform and vesicular with prominent nucleoli. No typical or atypical mitotic figures were identified within this proliferation. Staining with S-100 protein was strongly positive. These findings were consistent with a clear cell sarcoma.〈figure xml:id="f2"〉2〈mediaResource alt="image" href="urn:x-wiley:00119059:IJD1537:IJD_1537_f2"/〉Microscopic examination of the punch biopsy specimen from the edge of the ulcer in 〈link href="#f1"〉Fig. 1 showed a neoplasm composed of round cells and spindle cells in the deep reticular dermis (hematoxylin and eosin; A, × 100; B, × 400)A biopsy specimen from the right inguinal lymph node was positive for metastatic clear cell sarcoma. Chest radiography and computed tomography showed multiple nodules throughout both lungs. The patient received five cycles of therapy with cisplatin, vinblastine, dacarbazine, and interferon-α, and is alive 2 years later.

Notes: Summary The kinetics of uptake and elimination, covalent binding, and macromolecular interactions of 14[C-ring] melphalan was studied after a single oral dose (20 mg/kg, 0.1 mCi/kg) in normal rats. Peak radioactivity level in tissues was observed at 2–4 h after administration. Uptake of label in most tissues was rapid, with a t1/2 of less than 1 h. Elimination was biphasic. Tissues of the gastrointestinal tract showed the most rapid rates of elimination, with t1/2β of 13, 24, 18, and 19 h for stomach, duodenum, and small and large intestines, respectively. Bone marrow also showed a fast rate of elimination of radioactivity, with a t1/2 β of 30 h. Tissues with the slowest rates of elimination were skin, eye, spleen, pancreas, and lung, with t1/2 β of 333, 241, 149, 122, and 109 h, respectively. Covalent binding studies showed that melphalan, or its metabolites, bound irreversibly to all tissue macromolecular fractions. The percentage of covalently bound radioactivity increased with time in all tissues except kidney and eye, reaching up to 70%–80% of the total radioactivity remaining at 72 h. Elimination of covalently bound radioactivity was slower in the DNA fractions of the tissues of the gastrointestinal tract and heart compared with the elimination rate from lipid, protein, or RNA fractions. Slow elimination rates of 14[C-ring] melphalan equivalents from the protein fraction were observed in the skin, eye, and brain. Accumulation, rather than elimination, of radioactivity in this fraction was most prominent in the pancreas. In the bone marrow accumulation of radioactivity was observed in the lipid fraction.