R.I.F. van der Waal

Oral metastases: report of 24 cases.

AIM To study patients with oral metastatic tumours for the distribution of sex and age, the oral site and histopathological type of the metastasis, the primary tumour site and length of follow-up. PATIENTS AND METHODS All patients who had an oral metastasis diagnosed during the period January 1970-January 2001 at the Department of Oral and Maxillofacial Surgery/Oral Pathology, VU University Medical Centre, Amsterdam, The Netherlands, were included in this retrospective case study. RESULTS Of 1537 patients with newly diagnosed oral cancers, 24 had metastatic tumours. There was an equal sex distribution and age at the time of diagnosis ranged from 8 to 90 years (median 60). The metastatic tumours most commonly involved the bone (18/24), the mandible being the most common (15/18). The predominant histological type was adenocarcinoma. In most patients (n = 16) the primary tumour was already known before the oral metastatic lesion appeared. The most common primary tumours were breast, lung, kidney, and prostate, in that order. Prognosis was poor (median survival 6 months, range 1-60). CONCLUSIONS Oral metastases are rare and may present at any age in both sexes and predominantly involve bony structures, particularly the mandible. A third of oral metastases appeared to be the first indication of an occult malignant process elsewhere.

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.

Paraneoplastic hypertrichosis lanuginosa acquisita: uncommon or overlooked?

Acquired hypertrichosis lanugo‐type or hypertrichosis lanuginosa acquisita (HLA) is often associated with metabolic and endocrine disorders and use of certain drugs. The occurrence of HLA with malignancy was first noted in 1865, and it has since been described in 56 patients as a paraneoplastic syndrome both in women and in men. Sometimes HLA occurs concurrent with acanthosis nigricans, papillary hypertrophy of the tongue, and glossitis. The predominance of female cases is striking. Malignancy‐associated HLA seems to occur especially in the age group 40–70 years. In women with HLA the most frequent malignancy is colorectal cancer, followed in order by lung cancer and breast cancer; in men lung cancer is the malignancy most frequently associated with HLA, followed by colorectal cancer. In 3 years we saw 10 patients with HLA, in whom the malignancy was usually metastasized. Only one patient had local disease; after removal of the primary tumour it took 2 years before the lanugo hair recurred. The aetiology of the syndrome is not clear: no specific hormonal or biochemical abnormalities have been identified as yet. The difference between hirsutism and lanugo‐type hypertrichosis is discussed. It is stressed that the appearance of lanugo‐type hypertrichosis in body areas previously perceived by patients as ‘hairless’ is highly indicative of internal malignancy.

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 Crohns disease and sarcoidosis, therefore, patients with ano‐genital granulomatosis with accompanying gastro‐intestinal or pulmonary symptoms should be investigated for the presence of Crohns 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.

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 Crohns disease, and optic neuropathy. Cheilitis granulomatosa and optic neuropathy represent 2 rare manifestations of orofacial granulomatosis in Crohns disease.

Paraneoplastic pemphigus as the presenting symptom of a lymphoma of the tongue

A patient is described who initially presented with an acrovesicular eczema which subsequently developed into erythema multiforme with histopathological features of bullous pemphigoid. Although the various laboratory studies pointed to the diagnosis of paraneoplastic pemphigus (PNP), the underlying neoplasm was not detected until 6 months later, when the biopsies of an oral lesion showed the presence of an underlying non-Hodgkin lymphoma.

Paraneoplastic pemphigus caused by an epithelioid leiomyosarcoma and associated with fatal respiratory failure

A patient is described who initially presented with pemphigus vulgaris, limited to the oral cavity, and weight loss. Although the various laboratory studies pointed to the diagnosis of paraneoplastic pemphigus (PNP), the underlying neoplasm was not detected until 6 months later, when the patient developed shortness of breath and routine physical examination on admission revealed an abdominal mass, which eventually was proven to be an epithelioid leiomyosarcoma. In spite of radical excision of the tumour and intensive treatment of the dyspnoea, the patient died of respiratory failure 19 months after the PNP had been diagnosed. Early diagnosis of PNP is stressed to possibly prevent fatal pulmonary involvement.

7 Huidafwijkingen van het aangezicht

Tandartsen en kaakchirurgen worden met enige regelmaat geconfronteerd met patienten met aandoeningen van het mondslijmvlies die gepaard kunnen gaan met soortgelijke aandoeningen van de huid of van andere slijmvliezen dan die van de mond. Voorbeelden van dergelijke afwijkingen zijn lichen planus, discoide lupus erythematodes en pemphigus vulgaris. Daarnaast kan bij een patient die door een tandarts of kaakchirurg wordt onderzocht, sprake zijn van andere huidafwijkingen van het aangezicht. Het is wenselijk dat deze tandheelkundige professionals onderscheid kunnen maken tussen onschuldige en minder onschuldige afwijkingen en hun patienten zo nodig verwijzen naar hun huisarts of dermatoloog (huidarts) voor nadere diagnostiek en eventuele behandeling. Op deze plaats zullen enkele huidafwijkingen worden besproken die met enige regelmaat in het aangezicht kunnen voorkomen.