Marcello Menta Simonsen Nico

Actinic cheilitis: histologic study of the entire vermilion and comparison with previous biopsy.

Background:  Actinic cheilitis (AC), is the very superficial, incipient form of actinically induced squamous cell carcinoma of the lower lip. Few studies of actinic cheilitis analyzed the entire lip vermilion searching for microscopic areas of progressing carcinoma.

Lupus erythematosus: Clinical and histopathological study of oral manifestations and immunohistochemical profile of the inflammatory infiltrate

Background:  Lupus erythematosus (LE) is a multifactorial autoimmune disease, which may affect the oral mucosa in either its cutaneous and systemic forms, with varied prevalence.

Head and neck mucosal melanoma: a review.

Abstract:Head and neck mucosal melanoma (MM) is an aggressive and rare neoplasm of melanocytic origin. To date, few retrospective series and case reports have been reported on MM. This article reviews the current evidence on head and neck MM and the molecular pathways that mediate the pathogenesis of this disease. Head and neck MM accounts for 0.7%–3.8% of all melanomas and involve (in decreasing order of frequency) the sinonasal cavity, oral cavity, pharynx, larynx, and upper esophagus. Although many studies have examined MM of the head and neck and the underlying molecular pathways, individual genetic and molecular alterations were less investigated. Further studies are needed to complement existing data and to increase our understanding of melanocytes tumorigenesis.

Primary oral mucosal melanoma: a series of 35 new cases from South America.

Oral mucosal melanoma is rare and reported to be more aggressive than its cutaneous counterpart. Due to the rarity of this entity, data on epidemiology, tumor behavior, treatment, follow-up, and survival of patients are mainly based on single case reports. The few existing series of patients show that oral mucosa melanoma has its peak between 41 and 60 years of age, and male to female ratio is 2:1. Preferred oral sites include hard palate and maxillary alveolar crests. Risk factors have not been clearly identified, and surgical treatment is still the treatment of choice for oral mucosal melanomas. The authors retrospectively studied 35 patients with primary melanoma of the oral cavity to report their clinical and pathological features, such as age, sex, site of the tumor, metastasis, treatment, response to therapy, and outcome. We found no significant sex predominance, and the mean age of the patients was 60.6 years, with a range from 9 to 91 years. The majority of the patients (71.42%) had palate commitment, and invasive histopathological aspect was observed in 80% of the specimens (grade 3). Long-distance metastasis was found in 60% of the cases. Fourteen patients were submitted to wide surgical resections, with local relapse being observed in 11 of them (78.5%). The authors suggest that improved outcome in oral malignant melanoma requires the development of new therapies and the prevention of distant metastasis.

Mucocele in Pediatric Patients: Analysis of 36 Children

Abstract:  Mucoceles are common benign lesions of the oral cavity that develop following extravasation or retention of mucous material from major or minor salivary glands. Most series report a higher incidence of mucocele in young patients but no studies exist for this specific age group. The records of 104 patients presenting with mucocele who underwent surgery in the Oral Diseases Clinic of the Department of Dermatology of the University of São Paulo, Brazil, between 1991 and 2006, were reviewed. Of these, 36 (34.6%) were 15 years old or younger, the youngest being 2 years old. Twenty‐six patients were girls, 10 were boys. Lesions were located on the lower labial mucosa (30 patients), tongue (three), floor of the mouth (one), buccal mucosa (one), and in one patient the location was unknown. Histopathology revealed an extravasation pattern in 35 patients and a retention pattern in one. Of the distinct subtypes of mucocele, tongue lesions (mucocele of the glands of Blandin‐Nuhn) seem to be more prevalent and retention mucoceles seem to be very rare in children.

Oral manifestations of inflammatory bowel disease: a review based on the observation of six cases

Inflammatory bowel disease (IBD) comprises two chronic, tissue‐destructive, clinical entities: Crohns disease (CD) and ulcerative colitis (UC), both immunologically based. Bowel symptoms are predominant, but extra‐intestinal complications may occur, including involvement of the oral cavity. Oral involvement during IBD includes several types of lesions: the most common are aphthae; uncommon lesions include, among others, pyostomatitis vegetans and granulomatous lesions of CD. Starting with a presentation of six patients with oral manifestations, which were crucial for the final diagnosis of IBD, a review on the subject is presented. Oral involvement in IBD may be previous or simultaneous to the gastrointestinal symptoms. However, in the majority of cases, bowel disease precedes the onset of oral lesions by months or years. In many patients, the intestinal symptoms may be minimal and can go undetected; thus, most authors believe that the bowel must be thoroughly examined in all patients with suspected IBD even in the absence of specific symptoms. Usually, the clinical course of oral lesions is parallel to the activity of IBD; therefore, oral manifestations are a good cutaneous marker of IBD.

Subepidermal calcified nodule : Report of two cases and review of the literature

Abstract: Subepidermal calcified nodule (SCN) is a form of calcinosis cutis that most commonly occurs in children. The typical clinical presentation is of a solitary verrucous nodule on the face, but occasionally multiple lesions may be seen. We describe SCN on the eyelids of two female patients; one of whom had four lesions. The histopathology was typical of calcinosis cutis. Both patients were in good health.

Lupus erythematosus: clinical and histopathological study of oral manifestations and immunohistochemical profile of epithelial maturation

Background:  Lupus erythematosus (LE) is an autoimmune disease of unknown cause. Prevalence of oral involvement in patients with LE is uncertain but may vary from 9 to 45% in patients with systemic disease and from 3 to 20% in patients with chronic cutaneous involvement.

Rothmund-Thomson syndrome in three siblings and development of cutaneous squamous cell carcinoma

Abstract: Rothmund–Thomson syndrome (RTS) is a rare autosomal recessive genodermatosis. It is characterized by early onset of progressive poikiloderma and several other cutaneous and extracutaneous findings including alopecia, dystrophic teeth and nails, juvenile cataracts, short stature, hypogonadism, and bone defects. There are several reported cases of skin malignancies in RTS patients, indicating a possibly higher incidence of cutaneous and noncutaneous malignancies. We report three siblings with RTS who developed cutaneous squamous cell carcinoma (SCC).

Exuberant juvenile hyaline fibromatosis in two patients.

Abstract:  Juvenile hyaline fibromatosis and infantile systemic hyalinosis are rare autosomal recessive disorders of infancy and early childhood that are histologically characterized by deposition of hyaline material. The main clinical features are papulo‐nodular skin lesions, gingival hypertrophy, joint contractures, and bone abnormalities. However, infantile systemic hyalinosis has a more severe clinical presentation, including visceral involvement and premature death. Very recently, genetic studies identified mutations in the same gene in patients with both conditions, strongly suggesting that they belong to the same disease spectrum. We report two new nonrelated patients who met the criteria for the diagnosis of juvenile hyaline fibromatosis/infantile systemic hyalinosis. Clinical, histopathologic, immunohistochemical, and ultrastructural findings are presented, as well as an extensive review of the literature. Recent information regarding pathogenesis and treatment is discussed.