[Vulvar angioedema during pregnancy]. / Vulvair angio-oedeem tijdens de zwangerschap.

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. Based on clinical criteria, angioedema was diagnosed. Laboratory testing did not find any C1 inhibitor deficiency and hereditary angioedema was excluded for that reason. The swelling spontaneously disappeared within 2 weeks. CONCLUSION: In this case, the cause of the angioedema could not be found and the swelling disappeared on its own. Investigation into the cause may nevertheless be relevant, since choice of therapy depends on the underlying mechanism.

[Diagnostic image (319). A man with multiple yellow nodules]. / Diagnose in beeld (319). Een man met verspreide gele bultjes.

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. These granulomas are histologically indistinguishable from those found in both Crohn's disease and sarcoidosis, therefore, patients with ano-genital granulomatosis with accompanying gastro-intestinal or pulmonary symptoms should be investigated for the presence of Crohn's disease or sarcoidosis, respectively. The value of ano-genital granulomatosis as a unifying clinicopathologic concept is to provide a label for the affliction as well as to stimulate a careful search for possibly underlying systemic disorders, thus also permitting a more specific approach to therapy.

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. The patient showed a fair response to prednisolone and dapsone. Treatment to control the LABD was no longer required when interferon-alfa was started as palliative therapy for the metastasized renal cell carcinoma. The association of LABD and malignancies has been documented before and is not due to mere chance alone.

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.