1/251. A case report including EM and dna repair investigations in a dermatosis associated with multiple skin cancers: epidermodysplasia verruciformis.This report describes the clinical, histological and electron microscopic observations in a 51-year-old male with epidermodysplasia veruciformis (EV). cells with early signs of malignant transformation were found closely connected with virus infected epidermal regions. skin cancers appeared initially on sun-exposed areas, such as the face and ear lobes. The UV-induced dna repair synthesis was therefore studied, utilizing peripheral leukocytes. The patient had 40% lower UV-induced dna repair synthesis than the mean of nine healthy subjects of the same age. These results suggest that a decrease in UV-induced dna repair synthesis in combination with a possibly oncogenic viral infection may enhance the disposition for somatic mutations and malignant transformation in patients with EV.- - - - - - - - - - ranking = 1keywords = dermatosis (Clic here for more details about this article) |
2/251. Successful treatment of acquired perforating dermatosis with rifampicin in an Asian patient with sclerosing cholangitis.Acquired perforating dermatosis (APD) is a very rare disorder which has been described in association with systemic diseases such as diabetes mellitus, hiv infection or lymphoma. In this report we describe a patient with APD associated with sclerosing cholangitis and diabetes mellitus who was successfully treated with rifampicin. A 33-year-old Indian woman with a history of extensive pancreatic surgery, sclerosing cholangitis and insulin dependent diabetes mellitus was referred to our unit with intractable pruritus. She was treated with cholestyramine, ursodeoxycholic acid, several analgesics, UVB therapy, topical steroids, sedative antihistamines and plasmapheresis without significant improvement. Increasingly severe itching was associated with papular skin changes limited initially to the lower limbs but which later involved her entire body. biopsy of a representative lesion showed the changes of APD. She was subsequently treated with rifampicin which produced a dramatic resolution of pruritus within 3 weeks and the skin changes progressively resolved over subsequent months. In this newly described association of APD with sclerosing cholangitis, rifampicin treatment appeared to be efficient in ameliorating pruritus and the papular skin changes typical of APD.- - - - - - - - - - ranking = 1.25keywords = dermatosis (Clic here for more details about this article) |
3/251. Acquired perforating dermatosis in diabetes mellitus: an unusual case.A case of elastosis perforans serpiginosa in a patient who presented with insulin-dependent diabetes mellitus secondary to pancreatic insufficiency in a background of common variable immunodeficiency and endocrinopathy, as evidenced by pernicious anaemia and growth hormone deficiency, is described. In acquired perforating dermatosis occurring in patients with diabetes or renal failure, there is a spectrum of changes that may show an overlap of histological features of the four classic perforating diseases. The biopsy changes of the patient described in the present study most closely resembled those of elastosis perforans serpiginosa.- - - - - - - - - - ranking = 1.25keywords = dermatosis (Clic here for more details about this article) |
4/251. Acute neonatal scalp abscess and E coli bacteremia in the ED.An atypical presentation of newborn scalp abscess is described in a 9-day-old infant. The infant appeared nontoxic upon presentation on day 9 after birth with a scalp abscess and escherichia coli bacteremia. The infant had been admitted from the emergency department to the newborn intensive care unit on day 4 after birth with the same diagnosis; however, she was discharged within hours without treatment. This case report demonstrates the subtle clinical features, presentation, microbiology, and appropriate emergency department management of neonatal scalp abscesses.- - - - - - - - - - ranking = 0.0097420602978825keywords = scalp (Clic here for more details about this article) |
5/251. Neutrophilic dermatosis-associated sterile chronic multifocal osteomyelitis in pediatric patients: case report and review.Atypical pyoderma gangrenosum (PG) and sweet syndrome are neutrophilic dermatoses that share some common features. Sterile chronic recurrent multifocal osteomyelitis is a rare association of these neutrophilic dermatoses that has only been reported in children. We report a 3-year-old girl who initially presented with pain in her left hand and right leg. Roentgenograms and bone scan revealed findings of multifocal osteomyelitis affecting both femurs, the right tibia, left clavicle, right eighth costochondral junction, and left ulna. She was treated with antibiotics without improvement. Bone biopsy of the left ulna revealed histologic changes consistent with osteomyelitis, however, all cultures for bacteria, mycobacteria, and fungi were negative. She subsequently developed an ulcer surrounded by a violaceous, undermined border at the site of the bone biopsy, which also did not improve during antibiotic treatment. A biopsy specimen from this lesion demonstrated a dense perivascular and periappendageal infiltrate of neutrophils within the dermis and edema of the papillary dermis compatible with a neutrophilic dermatosis. She was treated with oral prednisone which resulted in resolution of skin lesions, bone pain, and soft tissue swelling. This case further documents the association between PG or sweet syndrome and multifocal sterile osteomyelitis.- - - - - - - - - - ranking = 1.25keywords = dermatosis (Clic here for more details about this article) |
6/251. Segmental forms of autosomal dominant skin disorders: the puzzle of mosaicism.Autosomal dominant inherited disorders of the skin sometimes present as a segmental phenotype. In recent years molecular studies have demonstrated that genetic mosaicism leads to such a clinical manifestation. In general the skin outside the segmental disorder is normal. This rather common variant of segmental manifestation has been termed type 1. Recently, Happle delineated a second type of segmental manifestation of autosomal dominant genodermatosis. This variant is characterized by a more diffuse clinical presentation of the disease, and a very marked linear pattern can be recognized. An explanation of this phenotype is a germline mutation of the gene manifests after a postzygotic mutation leading to double inactivation of the gene. The severe linear manifestation then reflects a doubling of the genetic burden. We present a number of clinical cases to demonstrate this phenomenon, and we present a case of the segmental Naegeli-Franceschetti-Jadassohn syndrome born to a mother with the diffuse manifestation of the disorder.- - - - - - - - - - ranking = 0.25keywords = dermatosis (Clic here for more details about this article) |
7/251. keratosis pilaris and ulerythema ophryogenes associated with an 18p deletion caused by a Y/18 translocation.We present a patient with partial monosomy of the short arm of chromosome 18 caused by de novo translocation t(Y;18) and a generalized form of keratosis pilaris (keratosis pilaris affecting the skin follicles of the trunk, limbs and face-ulerythema ophryogenes). Two-color FISH with centromere-specific Y and 18 dna probes identified the derivative chromosome 18 as a dicentric with breakpoints in p11.2 on both involved chromosomes. The patient had another normal y chromosome. This is a third report the presence of a chromosome 18p deletion (and first case of a translocation involving 18p and a sex chromosome) with this genodermatosis. Our data suggest that the short arm of chromosome 18 is a candidate region for a gene causing keratosis pilaris. Unmasking of a recessive mutation at the disease locus by deletion of the wild type allele could be the cause of the recessive genodermatosis.- - - - - - - - - - ranking = 0.5keywords = dermatosis (Clic here for more details about this article) |
8/251. Microcystic adnexal carcinoma: collaborative series review and update.BACKGROUND: Microcystic adnexal carcinoma (MAC) is a malignant appendageal tumor first described in 1982. It can be clinically and histologically confused with other malignant and benign cutaneous neoplasms, leading to inadequate initial treatment. This neoplasm is locally aggressive and deeply infiltrating, characterized by high morbidity and frequent recurrence. Mohs micrographic surgery has been used to conserve tissue and improve the likelihood for cure. OBJECTIVE: We report our experience using Mohs micrographic surgery for the treatment of MAC and compare with earlier reports in the literature. In addition, we review the epidemiology, clinical and histologic characteristics, and optimal treatment of this rare neoplasm. We also describe a 15-year-old white male patient with MAC on the scalp occurring only 7 years after radiation exposure. methods: The medical records of 11 patients with MAC who were treated by Mohs micrographic surgery were reviewed at both departments, and follow-up data were obtained. RESULTS: In all patients treated with Mohs micrographic surgery, there were no recurrences after a mean follow-up of 5 years. CONCLUSION: Mohs technique enables the detection of clinically unrecognizable tumor spread and perineural invasion often encountered with MAC. Aggressive initial treatment by microscopically controlled excision appears to offer the greatest likelihood of cure for this neoplasm, while providing conservation of normal tissue. In addition, we describe the second youngest patient with MAC and readdress the issue of previous radiotherapy as an important predisposing factor.- - - - - - - - - - ranking = 0.0013917228996975keywords = scalp (Clic here for more details about this article) |
9/251. association of lymphocytic colitis with linear IgA dermatosis.The case of a 66-year-old female patient is presented, who suffered from chronic watery diarrhea. In addition, she developed linear IgA dermatosis after oral treatment of a presumed yeast infection with nystatin. To evaluate the reason for her diarrhea, colonoscopy was performed. The macroscopic aspect of the colon mucosa was described as normal with no specific alterations for chronic inflammatory bowel disease or for bacterial infections. In contrast, the histologic examination revealed the typical characteristics of lymphocytic colitis. This disease is thought to be caused by immunological reactions against as yet unknown luminal antigens. After treatment with steroids and dapsone the diarrhea as well as the skin disease disappeared. To our knowledge, the present report describes for the first time the association of linear IgA dermatosis with lymphocytic colitis after oral treatment with nystatin. A possible causative link between these two disease entities is discussed.- - - - - - - - - - ranking = 1.5keywords = dermatosis (Clic here for more details about this article) |
10/251. Subdural empyema complicating cerebrospinal fluid shunt infection.Subdural empyema has not been reported previously as a complication of cerebrospinal fluid (CSF) shunt surgery. An infant submitted to CSF shunt insertion for congenital hydrocephalus developed subdural empyema after a failed attempt to treat a superficial scalp wound infection with oral antibiotics. enterobacter cloacae was isolated from the empyema. Temporizing management of the preceding superficial wound infection with oral antibiotics probably was the cause of this exotic pathogen. The treatment of infected scalp wounds contiguous with shunt hardware must be surgical.- - - - - - - - - - ranking = 0.002783445799395keywords = scalp (Clic here for more details about this article) |
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