Diffuse Large B-Cell Lymphoma in the Setting of Chronic Lymphocytic Leukemia.

A 77-year-old man was referred to our department with a 6-month history of a painless, rapidly enlarging tumor on the left leg. Fourteen years ago, he was diagnosed with chronic lymphocytic leukemia (CLL), and he had presented with lymphocytosis and multiple lymphadenopathies (Rai stage II). Both bone marrow aspiration and peripheral blood immunophenotyping identified a monoclonal B-cell population expressing surface CD5, CD20, CD23, CD43, and IgG kappa. At that time, he underwent eight courses of treatment with oral fludarabine, followed by complete remission, and he had been clinically stable ever since (Rai stage 0).

Vasculitic Leg Ulcer Revealing an Essential Thrombocythemia.

A 64-year-old man was referred to our department due to painful ulcers on the right leg that had evolved over the previous 6 months. There was also progressive weight loss. He had no relevant medical history. Clinically, we observed multiple ulcers, some of them with a necrotic base, located over the medial malleolus and calcaneus (Figures 1 and 2), with an associated livedoid appearance of the dorsum of the feet. A skin biopsy revealed epidermis with ulceration. The superficial and deep dermis showed perivascular and interstitial neutrophilic inflammatory infiltrate, with fibrinoid necrosis of the vessels as well as leukocytoclasia (Figure 3). Blood analysis showed significant thrombocytosis (1128×106 per µL) and leukocytosis (21.38×106 per µL). The autoimmune study showed no abnormalities. Abdominal ultrasound showed hepatosplenomegaly. The patient was seen in the hematology department, and a bone marrow biopsy was obtained that was compatible with essential thrombocythemia (ET). The patient had a karyotype that showed no metaphases, and was BCR-ABL-negative and JAK2-positive. He started treatment with α-interferon 1.8 million units, three times per week. Daily polyacrylate wound dressing was carried out to debride the skin lesions, and there was gradual improvement of the ulcers (Figure 4).

Medication-Related Osteonecrosis of the Jaw in a Patient with Nonuremic Calciphylaxis.

A 62-year-old man was referred to the emergency department of our hospital with pain and swelling in the left mandibular region that had evolved over the previous 2 months (Figure 1). His medical history included nonuremic calciphylaxis and systemic sclerosis (Figure 2). Since the diagnosis of the nonuremic calciphylaxis 5 years before, the patient had been treated with intravenous (IV) sodium pamidronate 60 mg per week for 11 months, without improvement, followed by IV sodium thiosulfate 25 mg twice a week for 18 month. During year 3 of treatment, the calciphylaxis lesions reappeared, and IV sodium pamidronate 60 mg per week was reintroduced to the patient's treatment. The patient remained with double treatment for the next 2 years, but 3 months before the patient's presentation, the IV treatment had been suspended due to an absence of peripheral venous access.

Nonuremic Calciphylaxis: Four Cases Associated with Autoimmune Diseases.

Calciphylaxis is a rare and severe syndrome of vascular calcification with unclear pathogenesis. This disease mainly affects patients with end-stage renal disease; however, it also has been described in nonuremic patients. We report four cases of nonuremic calciphylaxis (NUC) with ulcerated lesions associated with autoimmune disease. These cases support the literature suggesting that bisphosphonates and sodium thiosulfate are effective drugs in the treatment of calciphylaxis regardless of renal function.

Piebaldism and neurofibromatosis: state of knowledge.

Despite progress in understanding the molecular basis, the diagnosis of neurofibromatosis 1 (NF 1) is based on clinical criteria, established by the National Institute of Health (NIH) Consensus Conference in 1987. The association of NF1 and piebaldism has been reported, but some authors disagree with this co-occurrence. In the light of present knowledge, we highlight that both entities might co-exist in the same patient.

Urticarial vasculitis reveals unsuspected thyroiditis.

A 38-year-old woman presented with erythematous, violaceous plaques with a serpiginous and unusual appearance located on the left shoulder, left thigh, and right buttock, evolving for 5 days, which eventually became generalized. A skin biopsy revealed leukocytoclastic vasculitis and a diagnosis of urticarial vasculitis was made. The complete blood count, biochemistry, complement levels, and other immunological test results were unremarkable. However, antithyroid antibody titers were increased. Despite having normal thyroid function tests and an absence of specific symptoms, the patient underwent a thyroid ultrasound, which revealed features of thyroiditis, and was subsequently referred to an endocrinologist. Several diseases can be associated with urticarial vasculitis, namely infections and autoimmune connective-tissue disorders such as systemic lupus erythematosus and Sjögren syndrome. Thyroiditis is an uncommon association.

Thrombophilia in venous leg ulcers: a comparative study in early and later onset.

BACKGROUND: Several studies found that the patients with chronic venous ulcers (CVU) have an increased prevalence of thrombophilia (44-75%), similar to that observed in deep vein thrombosis (DVT). The patients who develop CVU before their 50 th birthday appear to represent a distinct group in terms of etiology, natural history and prognosis. AIM: To analyze the nature and prevalence of thrombophilia in patients with early onset of CVU (before 50-years old) compared with a group of patients with later onset. METHODS: Twenty-seven consecutive patients of each group were studied. They underwent clinical assessment and blood testing for factor V Leiden, prothrombin G20210A, methyltetrahydrofolate reductase C677T, plasminogen activator inhibitor type 1 (PAI-1) mutations, antithrombin, proteins C and S levels, and also antiphospholipid antibodies (anticardiolipin antibodies and lupus anticoagulant), cryoglobulins and cryoagglutinins. RESULTS: All the patients had at least one thrombophilia. The prevalences of single, 2 and ≥3 thrombophilias were 29.6%, 40.7% and 29.6%, respectively, in the early onset group, compared with 33.3%, 59.2% and 7.4% in the later onset group. The PAI-1 4G/4G homozygous mutation was significantly more common in patients with early onset of ulcer. The prevalences of factor V Leiden, prothrombin G20210A, elevated titer of antiphospholipid antibodies and the presence of cryoglobulins were higher in the early onset group, although the differences were not statistically significant. CONCLUSION: Our study brings evidence of a higher thrombophilic risk among the patients with early onset of the CVU as they had significantly higher prevalence of multiple (≥3) thrombophilias (P=0.03), homozygous mutations (P=0.03) and family history of leg ulcer (P=0.02) when compared with patients with later onset. Thrombophilia screening is important in patients with CVU before the age of 50 in order to stratify the thrombotic risk and to allow an appropriate prophylactic and therapeutic management.

Recurrent Wells' syndrome associated with allergic asthma exacerbation.

Wells syndrome is an inflammatory eosinophilic dermatosis of unknown pathogenesis characterized by clinical polymorphism, a suggestive but nonspecific histopathologic traits, usually with a recurrent course and inconstant response to therapy. It seems to be an unspecific hypersensitivity reaction in response to various exogenous and endogenous stimuli, such as insect bites, infections, drug eruption or underlying internal disorders. We present a patient with allergic asthma and atopic dermatitis in whom a skin eruption developed in the sequence of allergic asthma exacerbation, which was clinically and histologically consistent with the diagnosis of eosinophilic cellulitis. The authors discuss the probability of a common pathogenesis and the role of IL-5. To our best knowledge this is the first pediatric case where this association is reported.

[Uncommon causes of leg ulcers: investigative approach and therapeutics]. / [In Process Citation]

Chronic wounds of the lower limbs are a major public healthcare problem affecting 1 percent of the adult population and 3 to 5 percent of people older than 65 years. These numbers are rising in the western population as a result of increased life expectancy and increased risk factors for atherosclerotic occlusion, such as smoking, obesity, and diabetes mellitus. This very debilitating condition, which reduces significantly the quality of life, causes social discomfort and generates considerable cost, not only to the patient but also to the society. Treating chronic leg ulcers is always a challenge. Over the last years, the treatment of this condition has been given more attention because of the frequent ineffectiveness of the methods used, the awareness of the psychological and social impact, and the recognition of the risks of malignancy in these wounds. Determining the etiology is an essential factor for directed and more effective wound care. Although 90 percent of ulcers are of vascular origin, the list of other possible causes responsible for the other 10 percent is extremely long. In this review, the authors focus on the differential diagnosis of chronic leg ulcers and the impact of directed treatment in the prognosis of this condition.