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Siena, Italy

Tomasini D.,Dermatology Section | Berti E.,University of Milan Bicocca
Giornale Italiano di Dermatologia e Venereologia

Subcutaneous panniculitis like T-cell lymphoma derived from α/β T-cells (SPTCL-AB) belongs to the group of primary cutaneous T-cell lymphoma, and it represents less than the 1% of all primary cutaneous T-cell lymphomas. It affects patients in the 4th decade of life (median age of 36 years) with a female preference (male/female ratio 0.5) with 19% of patients being 20 years or younger. It can be sometimes complicated by a hemophagocytic syndrome, and patients without hemophagocytic syndrome had a significantly better survival (5-year OS: 91% vs. 46%). Histopathologically, SPTCL-AB is characterized by a lobular lymphocytic panniculitis. Tumor cells distribute between individual adipose lobules, proliferating and forming "rim" and "capping" images, conferring a lace-like appearance at scanning magnification. This is not an entirely disease-specific feature, and can also be seen in other lobular lymphocytic panniculitis, either of inflammatory and neoplastic origin. Tumor cells are phenotypically CD45RO+, βF1+ (a monoclonal antibody able to identify the alpha/beta chain of TCR), CD3+, CD4-, CD8+, and express cytotoxic granules (TIA-1, granzyme and perforin), whereas they show variable deletion of T-cell restricted antigens like CD2, CD5 and CD7. The majority of cases show a monoclonal rearrangement for TCR beta and gamma genes and do not show genomic integration of EBV. The present review will focus on histopathologic, immunophenotypical and molecolare data useful to overcome to a specific diagnosis of SPTCL-AB and to differentiate SPTCL-AB from other lymphomas of T-cell or NK/T cell origin and with benign panniculitidis sharing with SPTCL-AB a predominant lobular lymphocytic pattern of involvement of subcutaneous tissue. Source

Caldarella A.,Cancer Prevention and Research Institute | Fancelli L.,Dermatology Section | Manneschi G.,Cancer Prevention and Research Institute | Chiarugi A.,Cancer Prevention and Research Institute | And 2 more authors.
Journal of Cancer Research and Clinical Oncology

Introduction: In 2009, the American Joint Committee on Cancer (AJCC) incorporated the tumor mitotic rate in the melanoma pathological TNM staging system. To investigate the effect of this change on the pT1 substaging of primary cutaneous melanomas, we reclassified the cases collected by a cancer registry according to the 6th and the 7th editions of AJCC melanoma staging. Methods: Patients with pathological T1 melanoma diagnosed in the period 2000–2008 were selected from Tuscan Cancer Registry. The histological reports were reviewed and pT1 melanomas classified according to both the 6th and the 7th editions of the AJCC staging system. The shift of melanomas between pT1 substages was analyzed. Results: Among the 242 pT1 melanomas collected in the study period and with mitotic index available, there were 202 (83 % of all pT1) and 175 (72 %) pT1a, according to the 6th and the 7th editions of the AJCC melanoma staging, respectively. When the 7th edition was used, 20 % of all pT1a melanomas shifted to pT1b, and 32 % of all pT1b melanomas shifted to pT1a. A poor level agreement between the two TNM staging systems, measured by the Cohen’s kappa coefficient, was found (K = 0.37). Conclusions: The addition of mitotic activity to the pathological staging resulted in an increase in pT1b proportion and in a change in the classification of some cases. This modification could influence the clinical approach, with a different use of the sentinel lymph node biopsy, and underlines the role of mitosis evaluation in the management of thin melanoma patients. © 2015, Springer-Verlag Berlin Heidelberg. Source

Ortiz A.,Rheumatology Section | Ceccato F.,Rheumatology Section | Albertengo A.,Rheumatology Section | Roverano S.,Rheumatology Section | And 2 more authors.
Journal of Clinical Rheumatology

A 30-year-old woman was referred on April 2002 for a plaque that involved the internal aspect of the right leg, an erythema nodosum-like lesion on the lower extremities, and periarthritis on her left ankle. Subsequently, the patient developed anular, atrophic, growing, porcelain-white papules, with a thin rim of erythema and telangiectases over her upper and lower extremities. Clinically and histologically, these lesions were the characteristics of Degos disease. Despite arthritis and myositis that required treatment, low level C3 and C4, positive antinuclear antibodies, and elevated anticardiolipin antibodies only once, in a follow-up of 6 years the patient never developed a specific connective tissue disease or other systemic involvement. In conclusion, because clinical and histological findings of Degos disease might mimic connective tissue diseases, rheumatologists must be aware that this reaction pattern can be seen in a wide clinical spectrum of diseases. Copyright © 2010 by Lippincott Williams & Wilkins. Source

Naim M.,Dermatology Section | Weyers W.,Center for Dermatopathology | Metze D.,University of Munster
American Journal of Dermatopathology

Although exanthematous drug eruptions of the macular and papular type are common and often cause diagnostic problems, histopathologic features are not precisely defined in the literature. We present the first prospective histopathologic study of maculopapular drug eruption in 48 patients in whom the diagnosis had been made on the basis of clinical examination, history of a known offending drug, and follow-up. Because more than 1 biopsy was taken in 11 patients, 60 biopsy specimens could be examined. The most consistent epidermal features were mild spongiosis mainly of the lower layers (97% of biopsies), some hyperplasia (72%), a few lymphocytes (82%), and neutrophils (32%). The dermoepidermal junction revealed discrete vacuolization (97%), scattered lymphocytes (75%), and rare necrotic keratinocytes (32%). All cases showed a dermal perivascular inflammatory infiltrate that was superficial only in 72% of biopsies and superficial and deep in 28% of biopsies. An interstitial infiltrate in the papillary dermis could be found in 93%, more often patchy than lichenoid. In general, the perivascular infiltrate was mild and composed of lymphocytes (100%), eosinophils (60%), and neutrophils (50%). In the papillary dermis, neutrophils often outnumbered the eosinophils. Another feature were the clusters of neutrophils (38%) and eosinophils (20%) in the lumina of dilated, otherwise normal, blood vessels. Rashes induced by anticonvulsants and anxiolytics were characterized by predominance of neutrophils and largish lymphocytes. Edema of the papillary dermis was encountered frequently (85%), whereas wiry collagen bundles were an exceptional finding. In conclusion, our study defined a constellation of histopathologic findings highly suggestive of the diagnosis of exanthematous drug eruption of the macular and papular type. Copyright © 2011 by Lippincott Williams & Wilkins. Source

Chiner E.,Hospital Universitario San Juan Of Alicante | Ballester I.,Dermatology Section | Betlloch I.,Dermatology Section | Blanquer J.,Universitario Of Valencia | And 6 more authors.
Scandinavian Journal of Infectious Diseases

Varicella-zoster virus (VZV) pneumonia is one of the most serious complications of this infection in adults. The objective of this study was to analyze the epidemiological and clinical characteristics in a large sample of patients with VZV pneumonia. This was a 10-y retrospective, descriptive, observational study. We studied 46 patients with VZV pneumonia, 21 men and 25 women, with a mean age 36 ±11 y. A contact with an index case was observed in 57%, 76 were active smokers, 6.5% consumed drugs and 2 women were pregnant. The symptoms were: fever (83%), cough (83%), dyspnoea (63%), pleuritic pain (70%), and haemoptysis (6%) and started 35 days after the onset of blisters, except in 11% in whom respiratory symptoms appeared first. Arterial blood gases showed a mean PO2/FiO2 of 308 ±101 and 30 patients had a PO2 of <55 mmHg - 11 of these (4%) were admitted to the ICU, 8 required mechanical ventilation. Comparison of patients in the ICU with those on the general ward showed differences in the duration of fever (6.1 ± 4.2 vs 3.2 ± 1.1 days, p <0.001), mean stay (16.8±9.3 vs 7.2±2.4 days, p <0.001) and complications such as acute renal failure (p = 0.01) and acute respiratory failure (p < 0.001). Despite the severity of disease, no patient died. Once diagnosed, 98% were treated with acyclovir, combined with steroids in 6 and with antibiotics in 3 complicated with bacterial pneumonia. The prevalence for the period was 0.33 cases/100,000 inhabitants/y. In conclusion, VZV pneumonia has a severe course and accounts for a high percentage of admissions to the intensive care unit. The absence of mortality may be related to early treatment with acyclovir. Smoking was a risk factor for VZV pneumonia. © Informa UK Ltd. 2010. Source

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