[Vulvar angioedema during pregnancy].

Background: Angioedema is a condition of acute and extensive fluid accumulation in skin or mucosae due to increased blood vessel permeability. Angioedema can have several causes, including pregnancy.

Case Description: A healthy 33-year-old pregnant woman had acute, substantial swelling of the labia minora with no other symptoms.

Evaluation of dermal pericapillary fibrin cuffs in venous ulceration using confocal microscopy.

Dermal pericapillary fibrin is a hallmark of venous disease and is thought to play a pathogenic role in the development of ulceration. However, the actual spatial configuration of pericapillary fibrin is unknown, and it remains unclear whether it truly represents a barrier that can impair physiological exchanges between the blood and dermis. Using confocal microscopy on tissue specimens taken from the edges of venous ulcers in six patients, we report a detailed analysis of dermal pericapillary fibrin deposits.

[Diagnostic image (319). A man with multiple yellow nodules].

A 57-year-old man with liver function disturbances, probably due to alcohol abuse, presented with multiple yellow skin nodules: eruptive xanthomas. He used to consume pork excessively and had strongly elevated plasma triglyceride and cholesterol levels.

Orofacial granulomatosis in a patient with Crohn's disease.

Orofacial granulomatosis encompasses the previously recognized clinical entities Melkersson-Rosenthal syndrome and cheilitis granulomatosa. We report the case of a 39-year-old patient with cheilitis granulomatosa, intestinal Crohn's disease, and optic neuropathy. Cheilitis granulomatosa and optic neuropathy represent 2 rare manifestations of orofacial granulomatosis in Crohn's disease.

Ano-genital granulomatosis: the counterpart of oro-facial granulomatosis.

Ano-genital granulomatosis encompasses the previously recognized clinical entities of vulvitis granulomatosa, posthitis granulomatosa, and ano-perineitis granulomatosa. We report three patients with ano-genital granulomatosis. The pathological features of the disease are lymphoedema and the presence of non-caseating giant cell granulomas.

Lupus miliaris disseminatus faciei: a distinctive rosacea-like syndrome and not a granulomatous form of rosacea.

Background: Lupus miliaris disseminatus faciei is an eruption of discrete red-brown, dome-shaped papules, histologically characterized by epithelioid cell granulomas. The pathogenesis of the disorder remains controversial.

Objective: The authors discuss the place of lupus miliaris disseminatus faciei among granulomatous disorders.

Amyloidosis of the tongue as a paraneoplastic marker of plasma cell dyscrasia.

Objective: Our objective was to study the results of the medical workup in patients with amyloidosis of the oral cavity.

Study Design: Patients diagnosed with amyloidosis of the oral cavity during the period from January 1971 to January 2001 at the Departments of Oral and Maxillofacial Surgery/Oral Pathology and Dermatology of the VU University Medical Center, Amsterdam, The Netherlands, were included in this retrospective case study. In total, this series comprised 11 patients, 9 women and 2 men.

Cheilitis granulomatosa: overview of 13 patients with long-term follow-up--results of management.

Background: Cheilitis granulomatosa, often regarded as a subtype of orofacial granulomatosis, is characterized by recurrent or persistent swelling of one or both lips. Classically, a non-necrotizing granulomatous inflammation is seen at histologic examination. Although a relationship has been proposed between Melkersson-Rosenthal syndrome (and the monosymptomatic form, cheilitis granulomatosa) and Crohn's disease on the basis of the orofacial swelling and similar histology, several studies of Melkersson-Rosenthal syndrome have not found an association with Crohn's disease.

Cheilitis granulomatosa and optic neuropathy as rare extraintestinal manifestations of Crohn's disease.

Crohn's disease can be accompanied by extraintestinal manifestations. The authors report on a 39-year-old patient who presented with cheilitis granulomatosa as the first manifestation of Crohn's disease. Four years later, intestinal Crohn's disease was diagnosed.

Cheilitis granulomatosa.

Cheilitis granulomatosa is a rare inflammatory disorder with unclear aetiology. It is a disorder characterized by recurrent or persistent swelling of one or both lips that may be part of the Melkersson-Rosenthal syndrome or may be a manifestation of Crohn's disease. An overview of the clinical features, histopathology, differential diagnosis, management strategies and prognosis of cheilitis granulomatosa is presented and discussed with regard to the literature.

Linear IgA bullous dermatosis in a patient with renal cell carcinoma.

Linear IgA bullous dermatosis (LABD) is an autoimmune subepidermal bullous disease with heterogeneous clinical manifestations, characterized by linear deposition of IgA along the epidermal basement membrane zone. We report a patient with a metastasized renal cell carcinoma who developed an extensive blistering eruption. The lesions showed immunopathological findings characteristic of LABD.

Pericapillary fibrin cuffs in venous disease. A reappraisal.

Background: Over the last several years, a number of hypotheses have been proposed to explain the pathogenesis of venous ulceration. According to an early model suggested by Browse and Burnand, pericapillary fibrin cuffs, developing as a result of venous hypertension and extravasation of fibrinogen, act as a barrier to the diffusion of oxygen and nutrients; ultimately, tissue anoxia, cell death, and ulceration would follow. More recent hypotheses include the idea that macromolecules leaking from the vasculature trap growth factors and adhesion molecules, and the notion that white blood cells adhere to and damage endothelial cells in the microcirculation.