scholarly journals Efficacy of the Radiotherapy on Darier’s Disease: An Indirect Evidence

2013 ◽  
Vol 2013 ◽  
pp. 1-4 ◽  
Author(s):  
Ala Podgornii ◽  
Patrizia Ciammella ◽  
Dafne Ramundo ◽  
Cinzia Iotti
Keyword(s):  
Breast Cancer ◽  
Radiation Therapy ◽  
Local Control ◽  
Autosomal Dominant ◽  
Indirect Evidence ◽  
Skin Lesions ◽  
Linear Pattern ◽  
The Third ◽  
Darier’S Disease ◽  
Darier's Disease

Darier’s disease (DD) is an autosomal dominant dermatosis characterized by hyperkeratotic papules that are mainly located in the seborrheic areas and pushups, handheld wells, and nails. The disease often appears at a young age, typically by the third decade, with no sex predilection. There is currently no standard therapy and there are usually topical palliative therapies. We present the case of an affected 42-year-old woman treated with radiation therapy for early breast cancer. Before the radiotherapy, the patient showed hyperkeratotic, brownish papules extending in a linear pattern for the neck to the abdomen, especially on both breasts and inframammary area. During the radiation, she developed grade 1 to 2 dermatitis in the irradiated area. At a followup of 6 months, the patient has no skin lesions in the irradiated zone. This report suggests that the radiotherapy is not contraindicated and may indeed be effective in local control of skin lesions in DD.

scholarly journals A Rare Clinical Presentation of Intraoral Darier's Disease

2011 ◽  
Vol 2011 ◽  
pp. 1-3
Author(s):  
K. G. D. Manoja ◽  
B. S. M. S. Siriwardena ◽  
P. R. Jayasooriya ◽  
D. J. L. Siriwardane ◽  
W. M. Tilakaratne
Keyword(s):  
Autosomal Dominant ◽  
Clinical Presentation ◽  
Skin Lesions ◽  
Keratosis Follicularis ◽  
Dental Examination ◽  
Darier’S Disease ◽  
Dyskeratosis Follicularis ◽  
Darier's Disease ◽  
Adult Male Patient ◽  
Head Neck

Darier's disease, also known as keratosis follicularis or dyskeratosis follicularis, is a rare disorder of keratinization. It is an autosomal dominant genodermatosis with high penetrance and variable expressivity. Its manifestation appears as hyperkeratotic papules primarily affecting seborrheic areas on the head, neck, thorax, and less frequently the oral mucosa. When oral manifestations are present, the palatal and alveolar mucosae are primarily affected. They usually asymptomatic and are discovered in routine dental examination. Histologically, the lesions present as suprabasal clefts in the epithelium with acantholytic and dyskeratotic cells represented by “corps ronds and grains.” This paper reports a case of an adult male patient who presented with painful whitish lesions on buccal mucosa with crusty lips as the only clinical sign of Darier's disease. As this patient did not have skin lesions or family history, an intraoral biopsy confirmed the diagnosis of Darier's disease by a multidisciplinary team.

scholarly journals Surgical dilemma of the management of breast cancer in a patient with neurofibromatosis: case report and a review of the literature

2020 ◽  
Vol 2020 (10) ◽  
Author(s):  
Miguel Johnson ◽  
Lorna Cook ◽  
Fabio Rapisarda ◽  
Riccardo Bonomi ◽  
Dibendu Betal
Keyword(s):  
Breast Cancer ◽  
Radiation Therapy ◽  
Case Report ◽  
Autosomal Dominant ◽  
Neurofibromatosis Type ◽  
Skin Lesions ◽  
Review Of The Literature ◽  
Dominant Disease ◽  
Ongoing Surveillance

Abstract Neurofibromatosis type 1 (NF-1)—also commonly known as Von Recklinghausen’s disease—is an autosomal dominant disease that represents a constellation of clinical features. There are well-established links between NF-1 and many tumors; however, the link between NF-1 and breast cancer has more recently been elucidated. While the management of breast cancer is generally well established, there are unique challenges noted in patients with NF-1. There may be delayed presentations due to difficulty in differentiating underlying neurofibroma from a sinister breast mass. Additionally, multiple skin lesions seen in NF-1 create challenges in the interpretation of mammography. Furthermore, a surgical conundrum is created, as these patients appear to have a higher risk of angiosarcoma following radiotherapy. A mastectomy may be the best option as it obviates the need for radiation therapy and ongoing surveillance. A case exemplifying these dilemmas and a review of the literature are presented.

Early-stage breast cancer: local control after conservative surgery and radiation therapy with immediate interstitial boost.

Radiology ◽  
1993 ◽  
Vol 187 (1) ◽  
pp. 95-98 ◽  
Author(s):  
L Krishnan ◽  
W R Jewell ◽  
C M Mansfield ◽  
E K Reddy ◽  
J H Thomas ◽  
...  
Keyword(s):  
Breast Cancer ◽  
Radiation Therapy ◽  
Local Control ◽  
Early Stage ◽  
Conservative Surgery ◽  
Early Stage Breast Cancer

Radiomodification with fluor-pyrimidines in radiation therapy of breast cancer

2007 ◽  
Vol 25 (18_suppl) ◽  
pp. 11073-11073
Author(s):  
N. V. Malyshev ◽  
E. V. Kostrova ◽  
N. V. Bochkova ◽  
A. H. Dosakhanov ◽  
V. B. Sirota
Keyword(s):  
Breast Cancer ◽  
Radiation Therapy ◽  
Clinical Effect ◽  
Radical Mastectomy ◽  
Complete Response ◽  
Pathological Response ◽  
Breast Cancer Patients ◽  
Beam Radiation ◽  
The Third ◽  
Level 3

11073 Background: to assess the effectiveness of fluor-pyrimidines used in altered fractionated radiation therapy (RT) of breast cancer. Methods: 122 breast cancer patients (stage II) were included in this trial. Mean age - 51.3 years. All of them were randomly assigned to three groups. The first group - 41 patients treated by preoperative course of extra-beam radiation therapy, with double daily fractionation by 2 Gy (time between fractions - 4.5 hours, 4 Gy per day, 8 days), total dose - 32 Gy. The second group - 39 patients treated by the same regimen of radiation therapy, but with the concurrent application of the factory-made papers, sodden with 5-fluorouracil, on the irradiated breast. Papers covered the breast for 8 days, and were removing only during irradiation (2 times a day). The third group - 42 patients treated by the same regimen of radiation therapy, but with the concurrent radiomodification by capecitabine (1000mg/sq.m. per os twice daily before each irradiation, 8 days). In all three groups the radiation therapy was always followed by radical mastectomy in 1–2 days. The endpoints were: frequency of clinical effect and of level 3–4 pathological response. Results: positive clinical effect (complete response and partial response) and stabilization of disease were observed in the first group in 34.1% and 65.9% of patients, in the second group in 33.3% and 66.7% and in the third group in 42.9% and 57.1%. Progression was never observed. There were no signs of pathological response in 17% of patients of the first group, in 5.1% of patients of the second group and in 16.7% of patients of the third group. Level 1–2 and level 3–4 pathological response were observed in the first group in 63.4% and accordingly in 19.6% of patients, in the second group in 12.8% and 46.2% of patients and in the third group in 57.2% and 23.8% of patients. Conclusions: Therefore, using of papers with 5-fluorouracil in preoperational RT of breast cancer demonstrated more frequent level 3–4 pathological response. Using of capecitabine, on the contrary, demonstrated the highest clinical effect, but lower rate of level 3–4 pathological response. No significant financial relationships to disclose.

scholarly journals Possible Implication of Local Immune Response in Darier's Disease: An Immunohistochemical Characterization of Lesional Inflammatory Infiltrate

2010 ◽  
Vol 2010 ◽  
pp. 1-8 ◽  
Author(s):  
Clelia Miracco ◽  
Francesco Pietronudo ◽  
Vasileios Mourmouras ◽  
Michele Pellegrino ◽  
Monica Onorati ◽  
...  
Keyword(s):  
Immune Response ◽  
Dendritic Cells ◽  
Inflammatory Infiltrate ◽  
Normal Skin ◽  
Pemphigus Vulgaris ◽  
Skin Lesions ◽  
Cell Mediated Immunity ◽  
Local Immune Response ◽  
Darier’S Disease ◽  
Darier's Disease

Cell-mediated immunity is considered to be normal in Darier's Disease (DD), an inherited skin disorder complicated by skin infections. To date, there are no investigations on the local inflammatory infiltrate in DD skin lesions. In this immunohistochemical study we characterized and quantified it, making comparisons with two other inflammatory skin disorders, that is, pemphigus vulgaris (PV) and lichen ruber planus (LRP), and with the normal skin (NSk). We found a significant () decrease of CD1a+ Langerhans cells (LCs) in DD, compared to PV, LRP, and NSk, and of CD123+ plasmacytoid dendritic cells (pDCs), compared to PV and LRP. We hypothesize that the genetic damage of keratinocytes might result in a loss of some subsets of dendritic cells and, consequently, in an impaired local immune response, which might worsen the infections that inevitably occur in this disease.

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