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Intraoral facial and laryngeal features of tumoral calcinosis are reviewed in six patients of Jewish-Yemenite descent. Extraoral features included calcified masses, erythematous patches, and angular cheilitis. Oral soft tissue findings included papillary hyperplasia of the lip vermilion and velvety-red macules on the tongue, palate, and buccal mucosa. Gingival findings included advanced periodontitis and an erythematous marginal gingivitis. In all patients, orthognathic evaluation revealed a concave profile, retruded maxilla, and relative mandibular prognathism. Although previously reported clinical dental abnormalities were not present, extracted teeth demonstrated abnormal dentin. Hoarseness was present in all patients and was the earliest appearing feature in two patients. The appearance of these head and neck findings preceded the classical manifestations of tumoral calcinosis and may be helpful in the early diagnosis of this entity. The varied reported features of tumoral calcinosis support the notion that this disease may present multiple formes frustes with variable clinical expressivity.

Actinic cheilitis is not an uncommon disease and it can be an occupational hazard, particularly in those geographic areas with a great deal of sunshine. This condition has definite tendencies toward the development of carcinoma. Careful counseling and observation are necessary for good professional care of these patients, and occasionally vermilionectomy may be indicated.

The Melkersson-Rosenthal syndrome (MRS) is a rare triad of unilateral facial paralysis, facial swelling, and fissured tongue; often only two of the components are manifest. The occurrence of swollen lips together with the characteristic microscopic appearance is called granulomatous cheilitis, which many believe is the oligosymptomatic form of MRS. This article reviews the literature on granulomatous cheilitis associated with MRS. An unusual case of a swollen upper lip together with a fissured tongue, strongly indicating the oligosymptomatic manifestations of MRS, is presented.

Investigation of a 15-year-old white male patient with chronic granulomatous cheilitis revealed asymptomatic Crohn's disease of the lower gastrointestinal tract. This is reported to support the suggestion that chronic granulomatous cheilitis and Crohn's disease may be intimately related. Tests for nutritional status are shown to be important in the investigation and management of patients with this condition, being the first indication of any gastrointestinal pathology.

Cheilitis glandularis is a rare disorder characterized by enlarged mucous glands, usually in the lower lip, and possibly predisposing to squamous cell carcinoma. A case affecting the upper lip is presented, which appears to be the first report of the disorder in that site.

Cheilitis glandularis is an uncommon lesion affecting the lower lip. Its origin remains obscure, although it is hypothesized that chronic irritation secondary to prolonged solar exposure causes hypertrophy of the labial salivary glands and eventual enlargement and ectropion of the lower lip. Although carcinoma of the lower lip has been reported in association with it, cheilitis glandularis is still considered a benign process that is adequately managed with conservative surgery. A case is presented and its surgical management is described.

Actinic prurigo (AP) is a chronic, familial, photodermatitis that primarily affects American Indians. It is more prevalent in young females and may be evident clinically as a pruritic lower lip cheilitis that typically does not respond to conventional therapy. Other clinical features associated with AP include conjunctivitis, alopecia of the eyebrows, and formation of pterygia. The histologic features of AP have been described as nonspecific. We are presenting five cases of AP in which the major clinical manifestation was a pruritic, unsightly lower lip cheilitis. All of our patients were American Indians. Three patients were from the same family. The youngest patient was 10 years old and the oldest was 69 years old. All five cases were characterized histologically by numerous germinal centers within the lamina propria and a dense perivascular plasma cell infiltrate. In four of the five cases, there was a moderate to dense infiltrate of eosinophils. All five patients had been generally unresponsive to conventional therapy. With the exception of the youngest patient, all had had the disease for at least several years. One patient, now deceased, had the lower lip lesion surgically removed for cosmetic reasons. To our knowledge, this is the first report in the dental literature that describes the clinical and histologic features of AP of lower lip.

A case of cheilitis glandularis simplex occuring in a 65-year-old white man is presented. The condition is characterized by an everted lower lip, with enlarged labial salivary glands secreting a clear, thick mucus. A history of sun exposure, dry atrophic lip, and histologic findings of epithelial dysplasia, sclerosed glands, markedly dilated ducts, chronic inflammation, and basophilic degeneration of collagen indicate a solar etiology. Surgical excision by vermilionectomy gave excellent results. A high incidence of severe epithelial dysplasia and squamous cell carcinoma associated with cheilitis glandularis indicate surgery as the treatment of choice.

Six cases of an unusual form of cheilitis are reported, and it is proposed that this condition be named cheilitis of benign lymphoplasia. The chief complaint was of varying degrees of itching of the vermilion border. Histologically, the condition was characterized by a dense lymphocytic infiltrate with numerous lymphoid follicles containing well-differentiated germinal centers.

Thirty-three patients (seventeen females and sixteen males), six with complete and twenty-seven with abortive forms of Melkersson-Rosenthal syndrome (MRS) have been studied. Only patients with histologic granulomatous involvement were included in the study. The onset of MRS occurred predominantly during the second decade of life; the disease had a median duration of 6 1/2 years and a range of 6 months to 31 years. Recurrent lip swelling gradually turning into a permanent enlargement was the dominating sign, but changes in the buccal, palatal, sublingual, and gingival mucosa were frequently recorded as well. Peripheral facial paralysis occurred in 20 percent of the patients and plicated tongue in 40 percent. Laboratory investigations showed no specific changes. The elimination of odontogenic infectious foci was followed by regression or disappearance of the swellings in eleven of sixteen patient, suggesting a pathogenic mechanism. The present study does not seem to justify resections of the swellings when exacerbations are still occurring. A conservative approach is recommended.

Sixteen cases of plasma-cell gingivostomatitis were studied at the University of California San Francisco between 1966 and 1971. Twelve women and four men made up the group, with ages ranging from 15 to 70 years. Prior to treatment, the duration of the disease ranged from 5 to 60 months. Each of the sisteen patients manifested a homogeneous, diffuse, erythematous gingivitis associated with a cheilitis, and fifteen also had a marked glossitis. Each subject was symptomatic, and nine also complained of bleeding. Every biopsy specimen demonstrated a characteristically diffuse and dense plasma-cell infiltrate. There were no spontaneous remissions. Six patients completely responded to systemic corticoids alone, two to discontinuation of gum chewing or the use of their dentifrice, one to antifungal therapy, and the other seven to a combination of the preceding modalities. After completion of therapy, fifteen patients were followed for 2 to 55 months without any evidence of recurrence. Of interest, 69 per cent were gum chewers. The therapeutic benefit of discontinuing gum chewing by itself was not clear. It is assumed that the now nonexistent plasma-cell gingivostomatitis syndrome was a hypersensitivity response to an unidentifiable antigen used in various substances.

A 17-year-old white boy with signs, symptoms, and family history of angiokeratoma corporis diffusum universale, Anderson-Fabry disease (AFD), developed recurrent and then persistent swelling of both lips, erythematous hyperplastic gingivae, and a pebbled tongue. Positive blood findings were raised serum IgE, decreased T-cell level, and increased B-cell level. Histopathology of the gingiva showed noncaseating granulomas with multinucleate giant cells containing Schaumann bodies and large plasma-cell infiltrates in which immunofluorescence demonstrated immune globulins of several classes. Electron microscopy and histochemistry demonstrated ceramide in the vasculature. No glycolipid was found in the macrophages or giant cells of the granulomas which, in contrast, resembled sarcoid reactions. Plasma cells with Russell bodies and immune reaction-induced degranulation of mast cells were also identified. The pathogenesis of the oral findings possibly relates to altered immune reactivity associated with damage to the microvasculature analogous to that in Melkersson-Rosenthal syndrome.