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Notes: A 48-year-old woman presented with extensive facial ulceration of 1 year in duration. Based on the combination of the clinical appearance and a “nondiagnostic” biopsy taken elsewhere, the patient was started on oral prednisone at a dose of 40 mg/day, with a working diagnosis of pyoderma gangrenosum. According to the patient, the ulceration worsened over the 2 months whilst on prednisone and pain control was a major issue, controlled for the most part with oral oxycodone. One month prior to our evaluation of the patient, she was started on dapsone at 50 mg/day with no added benefit. Approximately 2 years prior to our evaluation, she developed raised, reddish, skin lesions on the abdomen and legs which recurred, but healed spontaneously each time after a few weeks.Her past medical history was remarkable for the remote use of intravenous drugs (which she had stopped for the past 20 years) and the past and continued use of alcohol on a daily basis. She had recently been tested elsewhere and was found to be positive for hepatitis C, but not human immunodeficiency virus (HIV). Examination revealed several ulcerations of the face and forehead, with an “apple jelly” coloration to the periphery and necrotic center. There was complete erosion of the nasal sidewall with apparent involvement of the septum (〈link href="#f1"〉Fig. 1). There were also scattered, smaller, nonulcerated, reddish to purplish lesions on the abdomen. Otolaryngologic examination revealed ulceration of the right ala and erosion of the nasal septum, but was otherwise unremarkable. No cervical or submental lymphadenopathy was noted.〈figure xml:id="f1"〉1〈mediaResource alt="image" href="urn:x-wiley:00119059:IJD2035:IJD_2035_f1"/〉Facial ulceration at the time of initial evaluationRoutine laboratory tests showed the patient to be anemic, with a hemoglobin level of 11 g/dL (normal, 12–15.5 g/dL) and a white blood count of 13,300 (normal, 3500–10,500) The total bilirubin was normal at 1.0 mg/dL, but several liver function tests were elevated by 2–4 times the upper limit of normal, including aspartate aminotransferase (AST), alanine aminotransferase (ALT), and γ-glutamyl transpeptidase (G-GT). Hepatitis C tests were abnormal, including antibody to hepatitis B core antigen (anti-HBc) and anti-hepatitis C virus (anti-HCV) antibodies, and hepatitis C-RNA was positive. Anti-hepatitis B surface antigen (anti-HBsAg) and antibody were negative. Human immunodeficiency virus (HIV) was negative. Antinuclear antibodies, antineutrophil cytoplasmic antibodies (ANCA), serologic tests for syphilis, cryoglobulins and cryofibrinogens, renal function, and urinalysis were all either negative or normal. The chest X-ray showed bilateral pulmonary nodules, confirmed by computed tomography (CT) scan of the chest. Serologic tests for several fungal organisms, including Sporothrix and Cryptococcus, were negative. Tuberculin skin testing was negative.Two 4-mm punch biopsies were taken from the right cheek and from the right lower lateral abdomen near the hip. Biopsy of facial tissue cultures for acid-fast bacilli and fungal elements was negative. Bacterial culture revealed 1+Staphylococcus aureus and coagulase-negative Staphylococcus. Polymerase chain reaction (PCR) testing for herpes and varicella viruses was negative.Histologic examination of the skin biopsies revealed identical findings from both sites. There was parakeratosis, serum crust, and mild irregular epidermal acanthosis. Focal interfacial dermatitis was present and characterized by basal vacuolization and melanophages in the superficial dermis. Epidermotropism of atypical lymphocytes singly and in cell clusters was seen in the epidermis. In the papillary and reticular dermis, extending to the subcutaneous fat, there was a dense, diffuse, perivascular, interstitial, and periappendageal infiltrate composed of atypical lymphocytes, lymphocytes, histiocytes, plasma cells, and neutrophils. The atypical lymphocytes infiltrated the hair follicles with little associated spongiosis (pilotropism) (〈link href="#f2"〉Fig. 2). The atypical lymphocytes also surrounded and infiltrated the eccrine glands and blood vessels. Immunoperoxidase studies were performed on paraffin tissue and showed that the atypical lymphocytes were immunoreactive with CD3 (〈link href="#f3"〉Fig. 3) and βF1, but not with CD8, CD56, T-cell-restricted intracellular antigen-1 (TIA-1), or granzyme B. There was a reactive population of B lymphocytes immunoreactive with CD20. In situ hybridization for Epstein–Barr virus (EBV), performed on paraffin sections, was negative. Lung biopsy of several nodules was accomplished via video-assisted thoracoscopy and revealed a peripheral T-cell lymphoma. Gene rearrangement studies of skin and lung nodules showed similar T-cell clones and were positive for T-cell rearrangement by PCR (TCR PCR, T γ-chain positive).〈figure xml:id="f2"〉2〈mediaResource alt="image" href="urn:x-wiley:00119059:IJD2035:IJD_2035_f2"/〉Histopathology of a skin lesion. (a) Dense atypical lymphocytic infiltrate demonstrating pilotropism and epidermal involvement with little associated spongiosis (hematoxylin and eosin stain, × 200). (b) Higher power view better demonstrating the dense atypical lymphocytic infiltrate (hematoxylin and eosin stain, × 400)〈figure xml:id="f3"〉3〈mediaResource alt="image" href="urn:x-wiley:00119059:IJD2035:IJD_2035_f3"/〉Strong CD3 immunoreactivity of the infiltrate (CD3 stain, × 200)The final diagnosis was peripheral T-cell lymphoma with pulmonary and skin involvement. There was no evidence for EBV infection.The patient returned home to receive treatment with four cycles of hyper-CVAD (cyclophosphamide, vincristine, adriamycin, and dexamethasone), followed by autologous stem cell transplantation. The facial erosions and ulcerations, as well as the systemic nodules, responded well to therapy, with only limited recurrence of the right lower lateral abdominal lesions, which were subsequently treated with radiation (〈link href="#f4"〉Fig. 4). The patient's clinical status 18 months after treatment is complete remission from lymphoma, but she has developed fulminant liver failure secondary to cirrhosis associated with hepatitis C.〈figure xml:id="f4"〉4〈mediaResource alt="image" href="urn:x-wiley:00119059:IJD2035:IJD_2035_f4"/〉Facial lesions after completion of chemotherapy

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.