Johnson square procedure for lentigo maligna and lentigo maligna melanoma.

BACKGROUND: Lentigo maligna (LM) and lentigo maligna melanoma (LMM) can be difficult to manage surgically. Predetermined margins can be inadequate because of subclinical spread, or can affect function when margins are adjacent to the eye or mouth. AIM: To describe our 5-year experience in Nottingham of using the staged square procedure (Johnson square) in excising difficult facial LM and LMM. METHODS: The square procedure is a staged technique useful for ill-defined lesions and for lesions that have a high recurrence rate due to subclinical spread. It uses paraffin wax-embedded peripheral vertical sections for margin control, ensuring complete clearance as the surgical margins are usually examined at distances of 2-5 mm from the periphery of the lesion. RESULTS: We treated 21 patients with LM or LMM with the staged square procedure over a 5-year period. Of the 21 patients, 10 needed only one stage of surgery, 6 needed two stages, 3 needed three stages and 2 needed four stages. To date, there has been only one recurrence, which was of an extensive lesion that crossed the medial canthus, making margin control impossible because of the anatomical limitations. CONCLUSIONS: The staged square procedure is an effective treatment for LM and LMM. It attempts to conserve tissue while ensuring a higher clearance rate. This offers favourable cosmetic outcomes and better prognosis, especially for facial LM and LMM.

Anti-neutrophil cytoplasmic antibody-positive neutrophilic dermatosis of the dorsal hands.

Neutrophilic dermatosis of the hands is a localized variant of Sweet syndrome (SS). It was first reported in 1995, and is an uncommon condition, with < 100 cases reported to date. The female preponderance, morphological and histological features, and response to treatment are similar to SS, but it differs in its distribution on the body. There may also be a lack of systemic features and inconsistent laboratory findings. Significantly, about half of all cases are associated with haematological problems, i.e. myelodysplasia and leukaemia. Other cases may be associated with ulcerative colitis or solid tumours. We describe a case of a 71-year-old man with neutrophilic dermatoses of the hands, who also had involvement of the lips. There was an associated rise in his anti-neutrophil cytoplasmic antibody level, which corresponded with the activity of the disease.

Acral histiocytic nodules: a possible new variant of non-X histiocytosis.

Many types of histiocytoses have been described. We present a case of a 56-year-old woman who presented with multiple nodules on the hands. On histological examination of an excision biopsy, a mainly dermal lesion was seen, with scattered mononuclear inflammatory cells and occasional multinucleated cells in the background. The tumour was composed of short spindle-shaped histiocyte-like cells with vesicular nuclei and small nucleoli. These cells were positive for CD68 and focally positive for smooth-muscle actin. There was no lipid or haemosiderin pigment, and no cholesterol clefts. Further investigations did not find evidence of bony or systemic involvement. The non-Langerhans cell histiocytoses, also known as non-X histiocytoses, are rare and of unknown aetiology. Clinicopathologically, this case does not seem to fit with any of the recognized subtypes, and we propose that it may represent a previously undescribed variant of the disease.

Challenges of stroke prevention in patients with atrial fibrillation in clinical practice.

Strokes and transient ischaemic attacks in patients with atrial fibrillation (AF) can be largely prevented. Risk stratification and appropriate prophylactic regimens help to alleviate the burden of AF-related thromboembolism. Guidelines recommend routine anticoagulation with oral vitamin K antagonists (VKAs) for patients at moderate-to-high risk of stroke, and acetylsalicylic acid (ASA) for those at low risk of stroke. ASA is less effective at reducing the risk of stroke than VKAs; however, ASA does not require monitoring or dose adjustment. Trials of anticoagulants show consistent benefits of oral VKAs for primary and secondary stroke prevention in patients with AF. Nevertheless, VKAs do require frequent coagulation monitoring and dose adjustment because of their variable dose-response profile, narrow therapeutic window, increased risk for bleeding complications and numerous food and drug interactions. This review aims to provide an overview of the clinical challenges of anticoagulant therapy for the prevention of stroke in patients with AF.

A novel case of follicular mucinosis after autologous stem-cell transplantation for multiple myeloma.

Follicular mucinosis is a rare inflammatory disorder of unknown aetiology, characterized by mucin deposition in hair follicles and sebaceous glands. FM can occur as a benign idiopathic primary disorder or secondary to malignant lymphoproliferative processes, most notably mycosis fungoides. We report a novel case of FM developing after autologous stem-cell transplantation for multiple myeloma, a correlation not previously reported in the literature.

Transcriptome and proteome analysis of ovaries of arrhenotokous and thelytokous Venturia canescens.

Under arrhenotoky, unfertilized haploid eggs develop as males but under thelytoky they develop into diploid females after they have undergone diploidy restoration. In the parasitoid wasp Venturia canescens both reproductive modes occur. Thelytoky is genetically determined but the underlying genetics of diploidy restoration remain unknown. In this study we aim to identify the genes and/or proteins that control thelytoky. cDNA-amplified fragment length polymorphism (cDNA-AFLP) analysis of total ovarian RNA and two-dimensional protein electrophoresis in combination with mass spectrometry revealed putative transcripts and proteins involved in arrhenotokous and thelytokous development. The detected tubulin and actin protein differences are most likely functionally related to the two types of reproduction.

Systemic mastocytosis with diffuse cutaneous involvement and haematological disease presenting in utero treated unsuccessfully with vincristine.

Mastocytosis is a disorder characterised by abnormal mast cell proliferation. The diverse spectrum of clinical presentations is dependent on the tissues and organs involved. We report a rare case of aggressive systemic mastocytosis presenting in utero with diffuse cutaneous involvement, and haematological abnormalities. There is little published evidence to guide treatment.

The many faces of secondary syphilis: a re-emergence of an old disease.

There has been a recent dramatic re-emergence of syphilis in the UK. This article describes the varied clinical manifestations of secondary syphilis and describes the diagnosis and current guidelines for treatment, especially in association with human immunodeficiency virus infection.

Eccrine porocarcinoma.

We present an unusual case of eccrine porocarcinoma in that diagnosis was delayed for many years after the initial lesion appeared, resulting in relatively large tumor.

Two squamous cell carcinomas arising in a linear epidermal naevus in a 28-year-old female.

The development of a squamous cell carcinoma (SCC) in a linear epidermal naevus is a rare event previously reported in long-standing and often extensive lesions of middle-aged to elderly patients. To the best of our knowledge, this case appears to be only the second report of an SCC arising in a linear epidermal naevus in a young patient and the first report of two SCCs developing in a small epidermal naevus, over a short time, in a young patient with no other risk factors.

Hodgkin's disease in patients older than 70 years of age: a registry-based analysis.

Between 1973 and 1993, 529 patients aged 15 years and over with Hodgkin's disease (HD) were entered into a lymphoma registry. Twenty-eight cases (1 only diagnosed at autopsy) of histologically proven HD in patients aged 70 years or older were identified. The distribution of sex, 'B' symptoms, histology and stage was not significantly different from that of younger patients, except for the fact that there were no patients aged 70 years or older with lymphocyte predominant HD. Nineteen patients were treated radically, 5 patients palliatively and 4 patients received no radiotherapy or chemotherapy. Three of the 14 patients treated with chemotherapy achieved the planned dose intensity. The cause-specific 5-year survival was 75% for patients aged 15-69 years and 28% for patients aged 70 years and over (logrank chi(2) = 43.7, P < 0.00001). The younger and older groups treated with radical intent had complete response rates of 97% and 74%, respectively (logrank chi(2) = 17.91, P < 0.00001) and relapse rates at 5 years of 27% and 56%, respectively (logrank chi(2) = 4.86, P = 0.0275). The main reason for the poorer prognosis of patients aged 70 years and over was the increasing difficulty of chemotherapy delivery associated with advancing age.

Cervical squamous cell carcinoma in situ with intraepithelial extension to the upper genital tract and invasion of tubes and ovaries: report of a case with human papilloma virus analysis.

A 55-year-old woman, who was found to have malignant squamous cells on a routine cervical smear, underwent a conization biopsy, followed by hysterectomy with bilateral salpingo-oophorectomy. No gross tumor was present in the uterus, but both ovaries, which were of normal size, contained multiple cysts filled with light brown, soft material. Microscopic examination showed squamous cell carcinoma in situ of the cervix with contiguous spread to the endometrium, fallopian tubes, and ovaries; squamous cell carcinoma extensively replaced the endometrial and tubal epithelium, focally invaded the wall of the fallopian tubes, and involved the parenchyma of both ovaries. Although an invasive cervical carcinoma occasionally spreads to the ovary, this case illustrates that exceptionally an in situ tumor spreads along the epithelium of the upper genital tract and the ovarian surface and invades the ovary and tubes. The detection of human papillomavirus DNA in the cervical, endometrial, tubal, and ovarian tumors by the polymerase chain reaction suggests a role for human papilloma virus infection in this case.