Publications by authors named "Lorenzo Cerroni"

299 Publications

E-Cadherin Expression and Blunted Interferon Response in Blastic Plasmacytoid Dendritic Cell Neoplasm.

Am J Surg Pathol 2021 Oct;45(10):1428-1438

Department of Molecular and Translational Medicine, Section of Pathology.

Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is an aggressive neoplasm derived from plasmacytoid dendritic cells (pDCs). In this study, we investigated by immunohistochemical analysis the expression of E-cadherin (EC) on pDCs in reactive lymph nodes and tonsils, bone marrow, and in BPDCN. We compared the expression of EC in BPDCN to that in leukemia cutis (LC) and cutaneous lupus erythematosus (CLE), the latter typically featuring pDC activation. In BPDCN, we also assessed the immunomodulatory activity of malignant pDCs through the expression of several type I interferon (IFN-I) signaling effectors and downstream targets, PD-L1/CD274, and determined the extent of tumor infiltration by CD8-expressing T cells. In reactive lymph nodes and tonsils, pDCs expressed EC, whereas no reactivity was observed in bone marrow pDCs. BPDCN showed EC expression in the malignant pDCs in the vast majority of cutaneous (31/33 cases, 94%), nodal, and spleen localizations (3/3 cases, 100%), whereas it was more variable in the bone marrow (5/13, 38,5%), where tumor cells expressed EC similarly to the skin counterpart in 4 cases and differently in other 4. Notably, EC was undetectable in LC (n=30) and in juxta-epidermal pDCs in CLE (n=31). Contrary to CLE showing robust expression of IFN-I-induced proteins MX1 and ISG5 in 20/23 cases (87%), and STAT1 phosphorylation, BPDCN biopsies showed inconsistent levels of these proteins in most cases (85%). Expression of IFN-I-induced genes, IFI27, IFIT1, ISG15, RSAD2, and SIGLEC1, was also significantly (P<0.05) lower in BPDCN as compared with CLE. In BPDCN, a significantly blunted IFN-I response correlated with a poor CD8+T-cell infiltration and the lack of PD-L1/CD274 expression by the tumor cells. This study identifies EC as a novel pDC marker of diagnostic relevance in BPDCN. The results propose a scenario whereby malignant pDCs through EC-driven signaling promote the blunting of IFN-I signaling and, thereby, the establishment of a poorly immunogenic tumor microenvironment.
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http://dx.doi.org/10.1097/PAS.0000000000001747DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8428867PMC
October 2021

Lupus erythematosus tumidus in a patient with mycosis fungoides stage IB after complete response to PUVA.

J Eur Acad Dermatol Venereol 2021 Jun 1. Epub 2021 Jun 1.

Department of Dermatology and Venereology, Medical University of Graz, Graz, Austria.

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http://dx.doi.org/10.1111/jdv.17425DOI Listing
June 2021

Lichen Sclerosus et Atrophicus With Histopathologic Features Mimicking Mycosis Fungoides: A Large Series of Cases Comparing Genital With Extragenital Lichen Sclerosus.

Am J Surg Pathol 2021 May 27. Epub 2021 May 27.

Unit of Pathology, Department of Medicine and Surgery, University of Insubria, Varese, Italy Kempf und Pfaltz Histologische Diagnostik Department of Dermatology, University Hospital Zurich, Zurich, Switzerland Research Unit Dermatopathology, Department of Dermatology, Medical University of Graz, Graz, Austria.

Lichen sclerosus et atrophicus (LSA) is a chronic inflammatory dermatosis of unknown etiology involving the genital and/or extragenital area, showing histopathologically a characteristic homogeneization and sclerosis of the superficial collagen with variably dense lymphoid infiltrates. Intraepidermal lymphocytes may be observed, and in some cases may pose differential diagnostic problems with mycosis fungoides (MF). We studied the histopathologic features of 121 cases of LSA with dense lymphoid infiltrates (genital: 94; male:female: 93:1; age range: 2 to 87 y; median age: 11 y; extragenital: 27; male:female: 0.1:1; age range: 11 to 79 y; median age: 59 y), to better characterize the intraepidermal lymphoid infiltrate and to compare genital with extragenital cases. Epidermotropic lymphocytes mimicking the histopathologic features of MF were present in 93.6% of the genital specimens but none of the extragenital cases. Interestingly, typical features of LSA were mssing in 39.4% of genital LSA, and in a further 25.5% were present only focally. Immunohistochemical analyses showed a predominance of CD8+ T-lymphocytes within the epidermis. Molecular studies of the T-cell receptor genes revealed a monoclonal population of T-lymphocytes in nearly half of the cases. Our study shows that MF-like histopathologic features are extremely common in genital LSA but are never encountered in extragenital cases. A diagnosis of MF in the genital area should be made only upon compelling features, keeping in mind the frequent pseudolymphomatous aspects of LSA.
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http://dx.doi.org/10.1097/PAS.0000000000001738DOI Listing
May 2021

Subcutaneous panniculitis-like T-cell lymphoma, lupus erythematosus profundus, and overlapping cases: molecular characterization through the study of 208 genes.

Leuk Lymphoma 2021 May 8:1-11. Epub 2021 May 8.

Department of Pathology, Fundación Jiménez Díaz, Universidad Autónoma de Madrid, Madrid, Spain.

Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is a rare cytotoxic cutaneous lymphoma. Differential diagnosis with lupus erythematosus panniculitis (LEP) can be challenging and overlapping cases have been described. In this study, we investigate whether gene expression profiling may or not identify markers that can be used to improve our understanding of the disease and to make a precise differential diagnosis. SPTCL, LEP, and overlapping cases were analyzed using a customized NanoString platform including 208 genes related to T-cell differentiation, stromal signatures, oncogenes, and tumor suppressor genes. Gene expression unsupervised analysis of the samples differentiated SPTCL from LEP samples. Most overlapping cases were clustered with LEP cases. Differentially expressed genes were observed when comparing SPTCL with LEP cases; and overlapping with LEP cases. Gene set enrichment analysis recognized gene sets defining each group. In conclusion, SPTCL and LEP have distinctive molecular profiles and the molecular background of overlapping cases more closely resembles LEP.
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http://dx.doi.org/10.1080/10428194.2021.1901098DOI Listing
May 2021

Long-standing purpuric exanthema.

JAAD Case Rep 2021 May 13;11:38-40. Epub 2021 Mar 13.

Department of Dermatology and Venereology, Medical University of Graz, Graz, Austria.

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http://dx.doi.org/10.1016/j.jdcr.2021.03.013DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8063691PMC
May 2021

Genomic landscape of cutaneous follicular lymphomas reveals 2 subgroups with clinically predictive molecular features.

Blood Adv 2021 02;5(3):649-661

Department of Pathology, Massachusetts General Hospital, Boston, MA.

Primary cutaneous follicle center lymphomas (PCFCLs) are indolent B-cell lymphomas that predominantly remain skin restricted and manageable with skin-directed therapy. Conversely, secondary cutaneous involvement by usual systemic follicular lymphoma (secondary cutaneous follicular lymphoma [SCFL]) has a worse prognosis and often necessitates systemic therapy. Unfortunately, no histopathologic or genetic features reliably differentiate PCFCL from SCFL at diagnosis. Imaging may miss low-burden internal disease in some cases of SCFLs, leading to misclassification as PCFCL. Whereas usual systemic FL is well characterized genetically, the genomic landscapes of PCFCL and SCFL are unknown. Herein, we analyzed clinicopathologic and immunophenotypic data from 30 cases of PCFCL and 10 of SCFL and performed whole-exome sequencing on 18 specimens of PCFCL and 6 of SCFL. During a median follow-up of 7 years, 26 (87%) of the PCFCLs remained skin restricted. In the remaining 4 cases, systemic disease developed within 3 years of diagnosis. Although the SCFLs universally expressed BCL2 and had BCL2 rearrangements, 73% of the PCFCLs lacked BCL2 expression, and only 8% of skin-restricted PCFCLs had BCL2 rearrangements. SCFLs showed low proliferation fractions, whereas 75% of PCFCLs had proliferation fractions >30%. Of the SCFLs, 67% had characteristic loss-of-function CREBBP or KMT2D mutations vs none in skin-restricted PCFCL. Both SCFL and skin-restricted PCFCL showed frequent TNFRSF14 loss-of-function mutations and copy number loss at chromosome 1p36. These data together establish PCFCL as a unique entity with biological features distinct from usual systemic FL and SCFL. We propose 3 criteria based on BCL2 rearrangement, chromatin-modifying gene mutations (CREBBP, KMT2D, EZH2, and EP300), and proliferation index to classify cutaneous FL specimens based on the likelihood of concurrent or future systemic spread.
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http://dx.doi.org/10.1182/bloodadvances.2020002469DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7876877PMC
February 2021

Connective tissue nevus in Marfan syndrome successfully treated with intralesional steroid injections.

G Ital Dermatol Venereol 2020 Dec 14. Epub 2020 Dec 14.

Department of Dermatology, University of Rome Tor Vergata, Rome, Italy.

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http://dx.doi.org/10.23736/S0392-0488.20.06798-XDOI Listing
December 2020

Infiltrierte erythematöse Plaques im Lendenbereich.

J Dtsch Dermatol Ges 2020 Nov;18(11):1331-1334

Universitätsklinik für Dermatologie und Venerologie, Medizinische Universität Graz, Österreich.

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http://dx.doi.org/10.1111/ddg.14326_gDOI Listing
November 2020

Pruritic papules after extensive fasting period.

JAAD Case Rep 2020 Nov 18;6(11):1191-1192. Epub 2020 Nov 18.

Department of Dermatology and Venereology, Medical University of Graz, Graz, Austria.

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http://dx.doi.org/10.1016/j.jdcr.2020.09.030DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7680911PMC
November 2020

Syringotropic Melanoma: A Diagnostic Challenge With Prognostic Implications.

Am J Dermatopathol 2020 Nov 16. Epub 2020 Nov 16.

Departments Dermatology and.

The presence of neoplastic melanocytes within the eccrine apparatus into the reticular dermis and/or subcutaneous tissue is extremely rare. The staging of syringotropic melanomas and their biological behavior are still controversial. We present 6 new cases of syringotropic melanoma and their main histopathologic features; review the previous literature; and discuss about the origin, staging, and prognosis of this rare variant of melanoma.
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http://dx.doi.org/10.1097/DAD.0000000000001853DOI Listing
November 2020

Infiltrated erythematous plaques on the lumbar area.

J Dtsch Dermatol Ges 2020 Nov 16;18(11):1331-1334. Epub 2020 Nov 16.

Department of Dermatology, Medical University of Graz, Graz, Austria.

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http://dx.doi.org/10.1111/ddg.14326DOI Listing
November 2020

Merkel cell carcinoma: A review.

J Cutan Pathol 2021 Mar 3;48(3):411-421. Epub 2020 Dec 3.

Research Unit of Dermatopathology, Department of Dermatology, Medical University of Graz, Graz, Austria.

Merkel cell carcinoma has been a focus of active scientific investigation in recent years and new information on the topic has emerged. Although uncommon, this primary cutaneous neuroendocrine carcinoma, usually involving the head/neck of elderly individuals, has a poor prognosis. Within the past two decades, an increase in the incidence of the tumor and the discovery of its link to the Merkel cell polyomavirus have focused medical attention on the lesion. The resulting studies have improved our understanding of the biology of the neoplasm and contributed to clinical care. Specifically, two pathogenic subsets of the tumor have come to light, the majority due to Merkel cell polyomavirus and the minority caused by ultraviolet radiation-induced genetic damage. This dichotomy carries prognostic implications favoring the former subset. In addition, having capitalized on the known susceptibility of the tumor to immune influences, investigators have recently discovered its responsiveness to immune checkpoint inhibition. This revelation has constituted a therapeutic milestone at the clinical level. Herein we provide an overview of the topic, outline updates in the field and place an emphasis on dermatopathologic aspects of Merkel cell carcinoma.
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http://dx.doi.org/10.1111/cup.13910DOI Listing
March 2021

A case of spontaneous regression of metastatic skin undifferentiated carcinoma.

Ital J Dermatol Venerol 2021 Jun 21;156(3):404-405. Epub 2020 Oct 21.

Unit of Dermatology and Venereology, Tor Vergata University, Rome, Italy.

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http://dx.doi.org/10.23736/S0392-0488.20.06701-2DOI Listing
June 2021

Cutaneous lymphohistiocytic infiltrates with foamy macrophages: A novel histopathological clue to Stenotrophomonas maltophilia septicemia.

J Cutan Pathol 2021 Jan 5;48(1):160-164. Epub 2020 Nov 5.

Research Unit Dermatopathology, Department of Dermatology, Medical University of Graz, Graz, Austria.

The best-known cutaneous manifestations of septicemia in the skin are the so-called "septic vasculitis" and "septic vasculopathy," which represent two sides of the same pathogenetic process. The spectrum of cutaneous presentations of septicemia is, however, more complex, extending beyond septic vasculitis/vasculopathy. We describe the exceptional histopathological findings of skin lesions associated with Stenotrophomonas maltophilia septicemia, featuring a lymphohistiocytic infiltrate characterized by predominance of foamy macrophages containing granular basophilic material negative for PAS, Gram, Fite, and Grocott. Albeit an uncommon occurrence, S. maltophilia septicemia should be included in the broad differential diagnosis of cutaneous lesions occurring in immunocompromised individuals with worsening general conditions. Awareness of these histopathological findings may facilitate the identification of this insidious infectious agent as a source of nosocomial septicemia.
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http://dx.doi.org/10.1111/cup.13893DOI Listing
January 2021

Blastic plasmacytoid dendritic cell neoplasms: results of an international survey on 398 adult patients.

Blood Adv 2020 10;4(19):4838-4848

Department of Pathology, University of Montréal, Hospital Maisonneuve-Rosemont, Montreal, QC, Canada.

The purpose of this study is to describe the clinical and prognostic features and to evaluate the outcome of different therapeutic approaches among patients with blastic plasmacytoid dendritic cell neoplasm (BPDCN) who have been diagnosed and treated in different institutions. A total of 398 patients from 75 centers were included in the study. Treatment consisted of non-Hodgkin lymphoma (NHL)-like regimens in 129 (32.8%) patients and acute leukemia (AL)-like regimens in 113 (23.5%) patients. In 61 (15.5%) and 16 (4.1%) patients, chemotherapy was followed by allogeneic and autologous hematopoietic stem cell transplantation (HSCT), respectively. Twenty-seven (6.9%) patients received radiotherapy, 6 (1.5%) received new agents, and 62 (15.7%) received palliative care. After a median follow-up of 12 months, median overall survival (OS) was 18 months. Patients who received NHL/AL-like regimens, followed by allogeneic HSCT, had the best outcome; median OS was not reached. OS was 65 months for patients who underwent autologous HSCT; 18 months and 14 months, respectively, for those treated with AL-like and NHL-like regimens without consolidation; and 4 months for those receiving palliative care (P < .001). In BPDCN, chemotherapy with lymphoma- or AL-like regimens, followed by transplantation, represents the therapeutic strategy associated with the best outcome. Consolidation with allogeneic HSCT, when feasible, appears superior to autologous HSCT.
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http://dx.doi.org/10.1182/bloodadvances.2020002474DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7556130PMC
October 2020

A 47-Year-Old Patient With Multiple Desquamative Patches and Subsequent Onset of Papular Lesions: Answer.

Am J Dermatopathol 2020 Oct;42(10):791-792

Department "G.F. Ingrassia," Section of Anatomic Pathology, University of Catania, Catania, Italy.

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http://dx.doi.org/10.1097/DAD.0000000000001486DOI Listing
October 2020

Intracorneal pustular drug eruption associated with nivolumab in a patient with metastatic renal cancer.

Int J Dermatol 2020 Dec 31;59(12):e435-e437. Epub 2020 Aug 31.

Department of Dermatology, Medical University of Graz, Graz, Austria.

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http://dx.doi.org/10.1111/ijd.15149DOI Listing
December 2020

Keratotic Nodules and Plaques on the Lower Extremities: Challenge.

Am J Dermatopathol 2020 Aug;42(8):e113-e114

Research Unit Dermatopathology, Department of Dermatology, Medical University of Graz, Austria; and.

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http://dx.doi.org/10.1097/DAD.0000000000001669DOI Listing
August 2020

A Large Subcutaneous Plaque on the Left Flank: Challenge.

Am J Dermatopathol 2020 Aug;42(8):e111-e112

Surgical Pathology, Galliera Hospital, Genoa, Italy.

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http://dx.doi.org/10.1097/DAD.0000000000001678DOI Listing
August 2020

Keratotic Nodules and Plaques on the Lower Extremities: Answer.

Am J Dermatopathol 2020 Aug;42(8):613

Research Unit Dermatopathology, Department of Dermatology, Medical University of Graz, Austria; and.

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http://dx.doi.org/10.1097/DAD.0000000000001668DOI Listing
August 2020

A Large Subcutaneous Plaque on the Left Flank: Answer.

Am J Dermatopathol 2020 08;42(8):612

Surgical Pathology, Galliera Hospital, Genoa, Italy.

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http://dx.doi.org/10.1097/DAD.0000000000001679DOI Listing
August 2020

Correlation Between Dermoscopic and Histologic Features of Uncommon Cutaneous Melanoma Variants.

JAMA Dermatol 2020 09;156(9):1029-1030

Research Unit Dermatopathology, Department of Dermatology, Medical University of Graz, Graz, Austria.

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http://dx.doi.org/10.1001/jamadermatol.2020.2480DOI Listing
September 2020

Intravascular Cutaneous Disorders. A Clinicopathologic Review.

Am J Dermatopathol 2021 Feb;43(2):119-136

Department of Dermatology, Fundación Jiménez Díaz, Universidad Autónoma, Madrid, Spain; and.

Abstract: Intravascular proliferations of the skin are clinically heterogeneous and may present with a wide range of clinical features, including violaceous papules, nodules, plaques, or other unspecific cutaneous lesions. Histopathologically, these conditions are characterized by proliferation of different cell types within the lumina of dermal vessels and endothelial cell hyperplasia. Immunohistochemistry is the best tool to identify the nature of the intravascular proliferating cells and the type of involved vessel. In this review, we analyzed the clinicopathologic and immunohistochemical characteristics of intravascular large cell lymphoma, T-cell and natural killer-cell intravascular large cell lymphoma, intralymphatic variant of CD30+ cutaneous lymphoproliferative disorders, benign atypical intralymphatic CD30+ T-cell proliferation, reactive angioendotheliomatosis, intralymphatic histiocytosis, papillary intralymphatic angioendothelioma or Dabska tumor, glomeruloid hemangioma, papillary hemangioma, intravascular papillary endothelial hyperplasia or Masson phenomenon, and the intralymphatic involvement of Merkel cell carcinoma, cutaneous metastases, and cutaneous angiosarcoma.
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http://dx.doi.org/10.1097/DAD.0000000000001706DOI Listing
February 2021

Bendamustine Monotherapy for Primary Cutaneous Gamma-Delta T-Cell Lymphoma.

JAMA Dermatol 2020 10;156(10):1029-1030

Department of Dermatology, Venereology, and Allergology, HELIOS St Elisabeth Hospital Oberhausen, University Witten/Herdecke, Oberhausen, Germany.

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http://dx.doi.org/10.1001/jamadermatol.2020.1231DOI Listing
October 2020

Cutaneous borreliosis: An insidious mimicker of patch-type granuloma annulare.

J Cutan Pathol 2020 09 25;47(9):876-878. Epub 2020 Jun 25.

Research Unit Dermatopathology, Department of Dermatology, Medical University of Graz, Graz, Austria.

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http://dx.doi.org/10.1111/cup.13760DOI Listing
September 2020

Melanoma of the proximal nail fold mimicking Hutchinson sign.

JAAD Case Rep 2020 May 29;6(5):411-413. Epub 2020 Apr 29.

Department of Dermatology and Venereology, Medical University of Graz, Graz, Austria.

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http://dx.doi.org/10.1016/j.jdcr.2020.03.009DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7200188PMC
May 2020

[Melanocytic lesion with halo-good guy or bad guy?]

Hautarzt 2020 Jun;71(6):482-484

Abteilung für Dermatologie & Venerologie, Klinik Maggiore, Universität Triest, Triest, Italien.

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http://dx.doi.org/10.1007/s00105-020-04569-4DOI Listing
June 2020
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