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. METHODS: This report reviews the available literature and presents 3 patients with lupus miliaris disseminatus faciei. RESULTS: The histopathologic characteristics of lupus miliaris disseminatus faciei--an epithelioid cell granuloma with central necrosis--may be found in granulomatous rosacea, whilst the clinical features and course of lupus miliaris disseminatus faciei are often similar to cutaneous sarcoidosis. CONCLUSIONS: We put forward lupus miliaris disseminatus faciei as a distinctive rosacea-like syndrome and not as a granulomatous form of rosacea.

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. The patients' medical workup and final diagnoses were traced by means of the medical records. RESULTS: All but one patient presented with amyloidosis of the tongue, most of them manifesting as macroglossia. In 7 of the 11 included patients a diagnosis of myeloma could be established shortly after their referral to the above-stated departments. Three of the 4 remaining patients appeared to have a monoclonal gammopathy of undetermined significance, and 1 patient was diagnosed with a lymphoplasmacytoid non-Hodgkin lymphoma (immunocytoma). CONCLUSIONS: Amyloidosis of the oral cavity predominantly involves the tongue, mainly manifesting as macroglossia. Amyloidosis of the tongue is associated with an occult underlying plasma cell dyscrasia, in particular myeloma, and, therefore, should be regarded as a paraneoplastic phenomenon of these hematologic diseases.

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. METHODS: The clinical features, histopathology, association with Crohn's disease, and results of nonsurgical and surgical therapy in 13 patients with cheilitis granulomatosa were investigated in a retrospective case study with a mean follow-up period of 8.2 years. RESULTS: There was a low chance of developing Crohn's disease. Most patients in this study responded to nonsurgical treatment modalities. Patients with deterioration of lip swelling usually responded to intralesional injections with triamcinolone or to short courses of systemic glucocorticoids. Nonsteroidal systemic modalities, such as clofazimine, hydroxychloroquine, or sulfasalazine, were alternatives to glucocorticoid regimens, thus avoiding the long-term side effects of corticosteroids. Surgical intervention should only be performed in severely disfiguring cases. CONCLUSIONS: The management of cheilitis granulomatosa remains a challenge. As this study revealed a low chance of developing Crohn's disease, it does not seem justified to inform patients with cheilitis granulomatosa of the possibility that they might develop Crohn's disease. Patients with a negative history of gastrointestinal complaints should not be exposed to routine investigations of the gastrointestinal tract.

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. One year afterward, acute loss of visual acuity from optic neuropathy developed as another rare extraintestinal manifestation of Crohn's disease.