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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: 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  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: 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.