Pyoderma gangrenosum: a retrospective review of patient characteristics, comorbidities and therapy in 103 patients.

BACKGROUND: Pyoderma gangrenosum (PG) is an uncommon and challenging disease, highly associated with comorbidities, but poorly characterized from a diagnostic and therapeutic perspective. OBJECTIVES: To describe the epidemiology of PG in a hospital-based retrospective review, focusing on demographics, comorbidities and treatments. METHODS: We conducted a retrospective chart review. Patient data were taken from the Research Patient Data Repository of Brigham and Women's Hospital and Massachusetts General Hospital from 1 January 2000 to 31 December 2007. We identified and confirmed 103 cases of PG, and collected data on anatomical location, number and size of the PG lesions, patient demographics, comorbidities, mortality rate and treatments. RESULTS: Of the 103 patients, 78 (76%) were female, and only 7% had a biopsy suggestive of PG. The lower leg was the most common location with 78% of PG ulcers occurring there, and 67 (65% of patients) had two or more ulcers at some point. Thirty-five individuals (34%) had inflammatory bowel disease (IBD), 21 (20%) had haematological disorders, 14 (14%) had major depression, 20 (19%) had seronegative arthritis, 11 (11%) had psoriasis, and nine (9%) had hepatitis. Therapy was generally multimodal. The mortality rate during the 8-year study period was 16%. CONCLUSIONS: We present one of the largest PG case series to date. In our study, we found that biopsy of a PG lesion rarely yielded characteristic features of the disease and tissue pathology should not be used to exclude a PG diagnosis. We also found a female predominance and associations with IBD and haematological disorders. Patients with PG in this series had high rates of depression and hepatitis. Further work is needed to establish the mechanism(s) underlying these findings.

Effects of alpha particles on zebrafish embryos.

Dechorionated zebrafish embryos were irradiated at 1.5 h post fertilization (hpf) to low-doses of alpha particles, viz., 1.4, 2.8, 5.6, 11.2 mGy (determined using Monte Carlo simulations). At 24 hpf, these embryos were then examined for apoptotic cells through acridine orange staining. The mean number of apoptotic cells was found to decrease significantly from controls to 1.4-mGy irradiation, and then to increase almost linearly to 2.8, 5.6 and 11.2-mGy irradiation. This trend is a typical characteristic of a hormetic effect.

Pathological features of primary breast cancer in the elderly based on needle core biopsies--a large series from a single centre.

Most breast cancer patients are diagnosed at>65 years but research efforts are mainly focussed on younger patients. Knowledge related to elderly breast cancer is urgently needed. Patients>70 years presenting with early operable primary breast cancer were studied. Pathological features of diagnostic needle core biopsies taken from 2078 tumours from 2061 consecutive patients managed under a dedicated elderly breast cancer service, in 1987-2006, were reviewed. There were 1996 invasive carcinoma of mammary type (96%) with (N=200) or without associated ductal carcinoma in situ (DCIS); 81 were DCIS only (3.9%). One malignant adenomyoepithelioma was seen. Among the invasive carcinomas, ductal carcinoma of no special type was seen in 87.1% while lobular and mucinous features were noted in 6.9% and 3.1%, respectively. Histological grades and oestrogen receptor (ER) status were assessed respectively in 826 and 1557 invasive carcinomas. Majority were grade 2 (62.7%), followed by grade 1. Around 82% were ER-positive. Their pattern was compared with that in 2674 tumours from younger (< or =70 years) counterparts. In all age groups there was a marked biphasal distribution of ER-positivity, but in patients>70 years this distribution was more marked, with a great preponderance of highly ER-positive tumours, and a substantial minority being ER-negative, with very few in intermediate groups. We believe that this is the largest dataset of pathological features of elderly primary breast cancer from one institution. We have clearly confirmed the high frequency of ER-positive tumours in elderly patients. Further work is underway to assess long-term outcome and clinical relevance.

Syringolymphoid hyperplasia and follicular mucinosis in a patient with cutaneous T-cell lymphoma.

Syringolymphoid hyperplasia with alopecia is an uncommon chronic dermatosis of which 9 cases have been reported, with or without follicular mucinosis or cutaneous T-cell lymphoma. We report a patient with cutaneous T-cell lymphoma and syringolymphoid hyperplasia and follicular mucinosis and review the previously reported cases. All reported cases with syringolymphoid hyperplasia were men (10 of 10), with the clinical findings of alopecia (9 of 10) and anhidrosis (3 of 10). Only 3 of 10 cases had associated follicular mucinosis. Of the 7 cases investigated, 6 were found to hve cutaneous T-cell lymphoma. Three patients were not investigated for cutaneous T-cell lymphoma. Although syringolymphoid hyperplasia can be idiopathic, it can also reflect a syringotropic cutaneous T-cell lymphoma. Careful follow-up with a biopsy of persistent lesions is recommended to evaluate for the presence of lymphoma.

Diagnostic clues to dermatitis artefacta.

We report a case of dermatitis artefacta in a 36-year-old man who had progressive, nonhealing ulcers and swelling of his right arm. The lesions spared two tattoos. In addition, our patient had several of the classic features of dermatitis artefacta. We outline more than a dozen diagnostic clues that may help the clinician faced with this difficult problem.

Off-label dermatologic therapies. Usage, risks, and mechanisms.

Off-label refers to the prescribing of Food and Drug Administration-approved drugs for a use not indicated on the package insert. The prescribing of off-label drugs may benefit patients with many dermatologic diseases including angiogenesis-related conditions. We surveyed 55 dermatologists from a single large academic program to assess their use of particular drugs for specific skin conditions, their perception of such use as being for Food and Drug Administration-approved or for off-label indications, and their attitudes towards off-label therapies. The practice of prescribing off-label drugs was common among the respondents, many of whom had misperceptions about which conditions are Food and Drug Administration-approved indications and about the legal ramifications of off-label therapies. We suggest that understanding the principles of off-label prescribing in conjunction with the mechanisms of drug action in diseases may help clinicians exercise their judgment in finding innovative therapies for their patients.

Lichen planus localized to the nails.

BACKGROUND: Lichen planus may rarely be localized to the nails. OBJECTIVE: A case of ungual lichen planus is reported that was misdiagnosed as onychomycosis. METHODS: A 52-year-old woman had progressive nail dystrophy for 13 months that was unresponsive to oral terbinafine. A nail biopsy was performed. RESULTS: Histopathologic findings were consistent with lichen planus. CONCLUSION: Onychomycosis may be confused clinically with other causes of nail dystrophy. It is important to confirm the diagnosis of onychomycosis with appropriate laboratory or histologic analysis.

Loose anagen syndrome and loose anagen hair.

Loose anagen syndrome, or loose anagen hair, is a recently described condition of unknown etiology that may be under-recognized. The typical patient is a child with sparse fine hair that can easily be pulled out. The diagnosis is confirmed by microscopic examination of firmly pulled hairs, many of which are in the anagen phase but lacking an inner and outer root sheath and demonstrating a ruffled cuticle. Some presentations of alopecia areata may be confused with this condition, but the pull test analysis serves to differentiate them. A variety of theories have been postulated to explain the pathophysiology of loose anagen syndrome. In some cases, there is an autosomal dominant pattern of inheritance. In most cases, this condition spontaneously improves with age.

Microvessel count and cerebrospinal fluid basic fibroblast growth factor in children with brain tumours.

Tumour growth is angiogenesis-dependent; brain tumours have more intense neovascularisation than other tumours and produce basic fibroblast growth factor, a potent angiogenic mediator. Because little is known about the release of basic fibroblast growth factor from brain tumours into extracellular fluids, we tested cerebrospinal fluid (CSF) from 26 children and young adults with brain tumours and 18 controls for basic fibroblast growth factor and for proliferative activity on cultured capillary endothelial cells. We also measured the density of microvessels in tumours by immunohistochemical staining. Basic fibroblast growth factor was detected in the CSF of 62% (16 of 26) patients with brain tumours but in none of the controls. Specimens with basic fibroblast growth factor stimulated DNA synthesis of capillary endothelial cells in vitro. Endothelial proliferative activity was blocked by neutralising antibodies to basic fibroblast growth factor. Basic fibroblast growth factor correlated with mitogenic activity in CSF in vitro (p < or = 0.0001), and with density of microvessels in histological sections (p < or = 0.005). A microvessel count of > or = 68 per 200 x field was associated with tumour recurrence (p = 0.005) and with mortality (p = 0.02). Basic fibroblast growth factor in brain tumours may mediate angiogenesis as measured by microvessel density in histological sections, so has potential as both a marker for neoplasia and a target for tumour treatments. Furthermore, evaluation of cerebrospinal fluid basic fibroblast growth factor, along with microvessel quantitation in biopsied tumours, may provide improved prognostic information for the management of patients with brain tumours.