11/83. epidermodysplasia verruciformis: 14 members of a pedigree with an intriguing squamous cell carcinoma transformation.A 38-year-old farmer was apparently well until 5 years of age. At this age, the patient's mother found mildly itchy, raised eruptions over the scalp during combing of the scalp hair. Since then, the eruptions have progressed insidiously to involve the face and other parts of the body, with a preponderance over the face, upper back, and chest, including the palms. The soles, oral cavity, and genital tract were unaffected. The lesions were persistent in nature and showed no remission or exacerbation. Mild to moderate pruritus/discomfort was experienced following sunlight exposure. A prominent, raised eruption appeared on the right side of the forehead 9 years ago, 25 years after the initial eruptions, which in due course ulcerated. It was progressive in nature and acquired a large size. Two years later, it was diagnosed as squamous cell carcinoma, for which liquid nitrogen cryosurgery was performed. There was a recurrence of the lesion at the site of surgery, which was excised 4 months later. Subsequently, there was a sudden flare up at the same site. It was badly infected with maggot infestation. The relentless course of the disease was evident by the appearance of two similar lesions, one on the right half of the nose and the other on the left preauricular region. A short while ago, fatigue and loss of weight were also recorded. Bladder and bowel functions were normal and there was no loss of appetite, hoarseness of voice, or breathlessness. Four of the patient's six children (three sons and one daughter) were reported to have similar lesions all over the body. In addition, nine other relatives were also affected. Accordingly, a total of 14 (12 males and two females) family members were found to be affected from amongst 41 individuals (26 males and 15 females) spread across several generations (Fig. 1). There was a second-degree consanguinity of marriage, with the patient's grandmother and wife's father being brother and sister. skin surface examination was marked by multiple, discrete, flat-topped, scaly, brownish-black papules of diverse morphology, from hypopigmented macules to verrucous lesions, with a few coalescing to form plaques. The scales were brown, dry, and adherent (Fig. 2a). The lesions were conspicuous by their presence over the face, neck, and front and back of the chest. The extremities were also affected. Nevertheless, the soles and genitalia were spared. The oral mucosae, hair, and nails were normal. Koebner's phenomenon was explicit, whereas Auspitz's sign was not demonstrable. In addition, a perceptible nodulo-ulcerative lesion (size, 3 in x 2 in) with indurated, raised, averted margins was encountered on the right side of the forehead. The ulcer was tender and had a fetid discharge. Necrotic slough was prominent over its base. Similar lesions were located on the left preauricular region and right half of the nose. hematoxylin and eosin-stained sections prepared from biopsies taken from representative lesions of epidermodysplasia verruciformis and squamous cell carcinoma revealed their diagnostic features. epidermodysplasia verruciformis showed features of hyperkeratosis and acanthosis with hardly any papillomatosis. Marginal elongation of the rete ridges was present. Extensive vacuolization was a remarkable feature, and was largely confined to the upper stratum Malpighian and the granular cell layer. Some of the vacuolated cells were fairly large; their nuclei were located in the center and had variable pyknosis. The granular cell layer was uniformly thickened, whereas the stratum corneum had a loosely felted (basket-weave) appearance. The dermis was apparently normal (Fig. 2b,c). The other microsection(s) from squamous cell carcinoma were conspicuous by the presence of hyperkeratosis, acanthosis, and irregular masses of epidermal cells, proliferating downwards and invading the dermis. Varying proportions of differentiated squamous cells formed their bulk. These cells were atypical, characterized by variations in size and shape, hyperplasia and hyperchromasia of the nuclei, absence of prickles, chas, characterization of individual cells, and the presence of both mitotic and atypical mitotic figures (Fig. 3b).- - - - - - - - - - ranking = 1keywords = life (Clic here for more details about this article) |
12/83. Giant basal cell carcinoma: improvement in the quality of life after extensive resection.We describe a rare case of giant basal cell carcinoma which invaded the orbital tissue and the anterior skull base. Though the eyeball in the right orbit was preserved with the tumour at the patient's request, the improvement in the quality of the patient's life was achieved.- - - - - - - - - - ranking = 76.430897627633keywords = quality, life (Clic here for more details about this article) |
13/83. Acral arteriovenous tumor developed within a nevus flammeus in a patient with sturge-weber syndrome.The sturge-weber syndrome consists of a large facial nevus flammeus in the distribution of the ophthalmologic division of the trigeminal nerve accompanied by ipsilateral leptomeningeal angiomatosis. Usually, when angiomatous nodules develop in a nevus flammeus of a patient with sturge-weber syndrome they are pyogenic granulomas. We describe an acral arteriovenous tumor developed within the nevus flammeus of a patient with sturge-weber syndrome. To our knowledge, acral arteriovenous tumor has not been previously described in the cutaneous vascular malformation of patients with sturge-weber syndrome. The development of acral arteriovenous tumor within the vascular malformation of a nevus flammeus in this patient with sturge-weber syndrome probably results from a vascular proliferation secondary to underlying arteriovenous shunts.- - - - - - - - - - ranking = 1keywords = life (Clic here for more details about this article) |
14/83. Non-malignant perineural spread of epithelial tissue in the orofacial region.A 48 year-old male had two operations because of severe neuralgic pains in the region of the right infraorbital nerve. The first operative specimen consisted of connective tissue, striated muscle fibers and two myelinated nerve bundles, surrounded by a ring of well differentiated squamous epithelium. Initially the lesion was assumed to show perineural spread of cancer in this region. As the pain was not alleviated, a second operation was performed. The framework of the second specimen also consisted of connective tissue and muscle. It contained in addition an atheromalike cyst and some nerve bundles, ensheathed by well differentiated epithelial cells partly in a ring-, partly in a horseshoe-like pattern. The perineural epithelial sheaths could be traced to a rupture of the cyst, and to benign proliferation of its epithelial lining in the connective tissue and along the nerve bundles. Following the second operation the patient was relieved of his complaints. During the last five years he had repeated control examinations but no sign of malignant disease could be found; his state of health was perfect. Our finding of benign perineural spread of squamous epithelium has not been described previously. The nature of the so-called perineural lymph spaces is also discussed.- - - - - - - - - - ranking = 1keywords = life (Clic here for more details about this article) |
15/83. Epidermal nevus.A 20-year-old woman presented with an asymptomatic, life-long, verrucous, hyperpigmented plaque on the face and neck that corresponded to the lines of Blaschko. Histopathologic examination shows an epidermal nevus. This nevus presents a challenge in management because of the location and extent of the lesion.- - - - - - - - - - ranking = 1keywords = life (Clic here for more details about this article) |
16/83. PHACES syndrome: a review of eight previously unreported cases with late arterial occlusions.PHACE and PHACES are acronyms for a syndrome of variable expression comprising posterior cranial fossa malformations, facial haemangiomas, arterial anomalies, aortic coarctation and other cardiac disorders, ocular abnormalities and stenotic arterial disease. We review five girls and three boys aged 1 month-14 years with disorders from this spectrum. Six had large facial haemangiomas but recent reports suggest that small haemangiomas may occur; hence our inclusion of two possible cases. We also focus on the recently recognised feature of progressive intracranial arterial occlusions, present in four of our patients, later than previously recognised, from 4 to 14 years of age. We suggest that many elements of this disorder could reflect an abnormality of cell proliferation and apoptosis.- - - - - - - - - - ranking = 1keywords = life (Clic here for more details about this article) |
17/83. Segmental facial hemangioma accompanied by brain anomalies: report of a case.hemangioma of infancy (HOI) is the most common tumor of infancy. Initial rapid proliferation is generally followed by gradual involution and resolution. However, some HOI may be associated with congenital structural abnormalities. We describe a case of HOI associated with hypoplasia of the left cerebellum and arachnoid cyst.- - - - - - - - - - ranking = 1keywords = life (Clic here for more details about this article) |
18/83. Metastatic Merkel cell tumor to the prostate and bladder.Merkel cell carcinoma is an uncommon cutaneous neoplasm of neuroendocrine origin. We report the case of a 71-year-old man with Merkel cell carcinoma metastases to the prostate and bladder presenting with lower urinary tract obstruction. Efforts should be made to optimize the patient's quality of life with the finding of metastatic Merkel cell carcinoma to the bladder or prostate until better adjuvant therapies are identified.- - - - - - - - - - ranking = 15.286179525527keywords = quality, life (Clic here for more details about this article) |
19/83. Distribution of adhesion molecules, IgE, and CD23 in a case of angiolymphoid hyperplasia with eosinophilia.angiolymphoid hyperplasia with eosinophilia developed in a 13-year-old boy. The immune phenotype of infiltrating cells and the distribution of adhesion molecules of the integrin, immunoglobulin, and selectin families were analyzed with monoclonal antibodies. The infiltrate consisted mainly of helper lymphocytes, monocytes, eosinophils, CD1 dendritic cells, and mast cells. A CD23/Fc epsilon receptor was present on 40% of these cells, and surface bound IgE was present on 30% of these cells. The latter was found on all cell types. A cellular adhesion molecule analysis revealed that the proliferating endothelial cells were expressing constitutive ICAM-1, LFA-3, VLA-1, VLA-3, VLA-5, alpha 6, and beta 3-integrin chain as well as activation-dependent VLA-2, VCAM-1, and human lymphocyte antigen-DR. The infiltrating cells expressed LFA-1 and CD2, the correlating ligands for ICAM-1 and LFA-3. Our studies support previous observations of an unusual IgE-mediated immune reaction, and suggest an additional pathogenic role for adhesion events in the development of the pathognomonic cellular composition in angiolymphoid hyperplasia with eosinophilia.- - - - - - - - - - ranking = 1keywords = life (Clic here for more details about this article) |
20/83. Multiple subclinical syringomatous proliferations encountered during mohs surgery for basal cell carcinoma.BACKGROUND: Examination of mohs surgery frozen sections may lead to discovery of incidental benign neoplasms such as syringomas. OBJECTIVE: An unusual occurrence of numerous subclinical syringomatous proliferations discovered during mohs surgery for a basal cell carcinoma that posed a diagnostic and management dilemma is reported. methods: Clinical records and histologic sections are examined. RESULT: Multiple syringomatous proliferations were noted around a basal cell carcinoma during mohs surgery. A few foci were atypical appearing and focally extending into the deep dermis causing a diagnostic dilemma. All atypical syringomatous proliferations were excised. Evaluation of final Mohs margins by permanent sections and biopsies of normal-appearing facial skin showed multiple benign syringomas. CONCLUSION: Although solitary subclinical syringomas have been described in association with basal cell carcinomas, the occurrence of multiple syringomas and syringomatous proliferations has not been previously reported. Dermatologists should be aware of the existence of this phenomenon and consider permanent section evaluation to better determine nature of unusual incidental tumors identified in frozen sections during mohs surgery.- - - - - - - - - - ranking = 8keywords = life (Clic here for more details about this article) |
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