Epidermodysplasia Verruciformis in Two Half Brothers with HIV Infection

2004 ◽  
Vol 8 (5) ◽  
pp. 357-360 ◽  
Author(s):  
Weimin Hu ◽  
Gerard Nuovo ◽  
Marlene Willen ◽  
Stephen Somach

Background: Epidermodysplasia verruciformis (EV) is a rare disorder characterized by widespread flat and common Verrucae. From 25% to 50% of EV cases are inherited, usually with an autosomal recessive pattern. An X-linked inheritance has also been reported. Many EV patients have a cellular immunity defect. HIV-associated lesions have been found to contain HPV-5, HPV-8, and HPV-20. Objective: We describe two HIV-positive Hispanic maternal half brothers who presented with asymptomatic polyangular papules and plaques on the face, trunk, and extremities and which first appeared 4–5 years prior. The histopathology is consistent with EV. HPV-8 was detected by in situ hybridization. The patients were treated with topical imiquimod for two months without improvement. Conclusion: To our knowledge, this is the first reported case of epidermodysplasia verruciformis in HIV-positive pediatric patients.

Blood ◽  
2005 ◽  
Vol 106 (11) ◽  
pp. 4696-4696
Author(s):  
Masumeh Rafati Javidan ◽  
Alpesh J. Amin ◽  
Gunwant Guron

Abstract Plasmablastic lymphoma (PBL) is a rapidly progressive variant of diffuse large-cell lymphoma predominantly diagnosed in HIV positive patients. It is frequently associated with latent EBV infection and is often lacking expression of CD20 and CD45. A retrospective study of HIV positive patients diagnosed with PBL to determine outcomes after CHOP therapy. Two HIV positive patients diagnosed with PBL and treated with CHOP were identified. The first patient is a 48 year old male with CD4 count of 80 cells/mm3 on HAART, who presented with a rectal mass. Pathologic exam revealed granulation tissue with marked inflammation. A second colonoscopy because of ongoing rectal bleeding was done. Histologic exam was interpreted as infiltrating poorly differentiated carcinoma. The third biopsy obtained via colonoscopy (after developing bowel obstruction) was reported as a high grade tumor with neuroendocrine features. The patient received one dose of cisplatin 60 mg/m2 and irinotecan 60 mg/m2 and radiation therapy. Patient underwent a diverting colostomy. The immunohistochemical exam of the tissue was negative for CD20, CD3 and positive for CD 138, suggestive of the diagnosis of plasmablastic lymphoma. In situ hybridization for EBV was positive. The patient received 6 cycles of CHOP (cytoxan 750 mg/m2, vincristine 1.4 mg/m2, doxorubicine 50/m2 and prednisone 100 mg) chemotherapy and 6 more cycles of radiation therapy. He is in complete remission 16 months after the diagnosis, and continues to be followed. The second patient is a 44 year old female on HAART with CD4 count of 415 cells/mm3, who presented with the swelling of the right jaw for three weeks. The histologic exam of the tissue, obtained by the incision biopsy of the mass, favored plasmablastic lymphoma. Immunohistochemical analysis tested positive for CD3, CD138 and CD45. In situ hybridization for EBV RNA was positive. Patient received 3 cycles of CHOP regimen and radiation to the jaw. She has not shown any evidence of relapse and is in complete remission 22 months after diagnosis, and continues to be followed. Durable remissions, 16 and 22 months, were achieved with CHOP therapy in HIV positive patients with PBL. This study supports the use of CHOP as an effective treatment of PBL in HIV positive patients.


2012 ◽  
Vol 30 (15_suppl) ◽  
pp. 8586-8586
Author(s):  
Eric J. Stanelle ◽  
Klaus J. Busam ◽  
Barrie S. Rich ◽  
Daniel G. Coit ◽  
Michael P. La Quaglia

8586 Background: Determining the malignant potential of atypical spitzoid melanocytic proliferations can be diagnostically challenging, and many patients are therefore managed as if they had melanoma. However, few studies focus specifically on atypical spitzoid tumors (AST). Further, cytogenetic analysis using fluorescence in situ hybridization (FISH) has been used to determine malignant potential. We reviewed our institutional experience to determine staging and clinical outcomes, and the correlation between FISH findings and regional nodal positivity in patients with AST. Methods: With IRB approval, we retrospectively reviewed all patients aged <18 years treated for AST from 1981-2011 to determine staging variables, outcome, and the results of 4-probe FISH assay. A total of 44 patients with a median age of 11.5 years were identified. All pathology was re-reviewed by a single dermatopathologist. Staging was based on 2010 AJCC criteria. Correlations were determined using either the Pearson or Spearman correlation coefficient. Results: Median lesion thickness was 2.8 mm (range: 0.55-12) and eight (18%) lesions met spitzoid melanoma criteria. Median followup was 4.1 years for all patients (5.5 years for spitzoid melanomas). Thirty-nine patients (89%) underwent sentinel lymph node biopsies (SLNB) and 15 (38%) were positive. Lymph node (LN) positivity correlated with tumor thickness (p=0.002), stage (p=0.001), and mitotic rate (p=0.05). FISH was available for 17/39 patients who had SLNB; sensitivity and specificity for LN metastases were 50% and 54%, respectively. Of 15 patients with a positive SLNB, 12 underwent completion LN dissection, three of which were positive for 1, 2, and 3 additional nodes, respectively. There were no recurrences or disease-related deaths. Conclusions: Traditional indicators of thickness and stage predicted nodal involvement in pediatric patients with AST. However, FISH results did not predict nodal status and should not be used to guide management. With 100% disease specific survival and no recurrences, AST should be considered a separate entity from melanoma and complete excision may be sufficient therapy.


1989 ◽  
Vol 3 (2) ◽  
pp. 125-132 ◽  
Author(s):  
Mario Pezzella ◽  
Francesco Pezzella ◽  
Mariella Rapicetta ◽  
Graziella Morace ◽  
Beatrice Macchi ◽  
...  

Author(s):  
Marcela Vasco Travassos ◽  
Estela Mari Sandini ◽  
Julia Fogaça Toiello ◽  
Udenilson Nunes da Silva Junior ◽  
Gabriela Rodrigues Alves ◽  
...  

The disease epidermodysplasia verruciformis is a rare genodermatosis, autosomal recessive in most cases, characterized by susceptibility to infection by types of HPV of the genus β (EV-HPV) that do not occur in immunocompetent clinics. With the increased survival of immunosuppressed patients, especially with AIDS, a form of the disease called epidermodysplasia verruciformis acquired from a clinician similar to the genetic disease has been reduced, but challenging to manage, since an antiretroviral therapy leads to a cutaneous one, making patients more resistant to any treatment modality.


2014 ◽  
Vol 1 (2) ◽  
pp. 130-132
Author(s):  
Riad Zerguine ◽  

Epidermodysplasia verruciformis (EV) is a rare autosomal recessive genodermatosis, characterized by an abnormal sensitivity to cutaneous infection for some types of human papillomaviruses (HPV) associated with a cellular immune deficit. It expresses the appearance of squamous macules and spread verrucous papules, with chronic evolution. In half of the cases, before the age of 30, appears preepitheliomatous lesions and squamous-cell carcinomas. Here we report the case of a patient having an EV complicated with squamous-cell carcinoma of the face.


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