Oral focal mucinosis: A multi-institutional study and literature review.

BACKGROUND: Oral focal mucinosis (OFM) is a rare benign condition of unknown etiology, considered the oral counterpart of cutaneous focal mucinosis. We report the clinicopathologic features of 21 cases of OFM in conjunction with a review of the literature. METHODS: Clinical data were collected from the records of five oral and maxillofacial pathology services. All cases were evaluated by hematoxylin and eosin staining, histochemistry, and immunohistochemistry (vimentin, S-100, α-SMA, CD34, and mast cell). RESULTS: The series comprised 14 females (66.7%) and seven males (33.3%), with a mean age of 48.2 ± 20.7 years (range: 8-77 years) and a 2:1 female-to-male ratio. Most of the lesions affected the gingiva (n = 6, 28.6%) and presented clinically as asymptomatic sessile or pedunculated nodules with fibrous or hyperplasic appearance. All cases were negative for S-100 protein, CD34, and α-SMA and positive for Alcian blue staining. Conservative surgical excision was the treatment in all cases, and there was only one recurrence. CONCLUSION: OFM is a rare benign disorder that is often clinically misdiagnosed as reactive lesions or benign proliferative processes. Dermatologists and pathologists should consider OFM in the differential diagnosis of soft tissue lesions in the oral cavity, mainly located in the gingiva.

Oral focal mucinosis of the palate: a rare disease entity.

Oral focal mucinosis (OFM) is an extremely rare, benign oral soft tissue condition; less than 10 documented cases have been reported in the literature in patients under 18 years old. OFM has an unknown aetiology and predominantly presents in the fourth and fifth decades. The pathogenesis of OFM may be due to fibroblast overproduction of hyaluronic acid. Clinically, it remains almost impossible to diagnose definitively, due to its lack of pathognomonic features, therefore such lesions may have multiple differential diagnoses and histological analysis is essential to confirm OFM. We present an unusual presentation of OFM in a 14-year-old female patient. Following excision, focal myxoid degeneration of the connective tissue was apparent. This case highlights this rare condition for consideration in differential diagnosis of clinically similar lesions.

Oral focal mucinosis of the mandible in an adolescent patient.

Oral focal mucinosis (OFM) is the rare oral manifestation of cutaneous focal mucinosis. It is a diagnosis made histopathologically, as OFM remains clinically similar to other more common oral lesions, and radiographs do not provide any diagnostic information. This case is a report of a teenage female with left mandibular involvement of an elevated, rounded, asymptomatic, mucosa-coloured lesion in the facial and lingual gingiva between her left first and second mandibular molars. The cause was unclear, although the patient stated that she may have sustained a laceration in that area several months prior. An incisional biopsy revealed histopathological findings consistent with OFM, and complete surgical excision of the lesion was performed under a general anaesthetic, with no signs of recurrence for 2 months. The histological, clinical and accepted treatment methods on OFM will be discussed. Clinicians, including those serving paediatric populations, should consider OFM in their differential diagnoses when evaluating gingival lesions.

Oral focal mucinosis: a rare case with literature review.

Oral focal mucinosis (OFM) is an unusual disease that affects diverse localised areas of the mouth, where the connective tissue undergoes focal degeneration. It was described for the first time by Tomich in 1974. It presents as an asymptomatic pedunculated or sessile growth, commonly on the gingiva, with most cases being in women. Its pathogenesis is linked to overproduction of hyaluronic acid by fibroblasts during collagen production, ensuing in focal myxoid degeneration. It has no characteristic features and diagnosis depends on histological scrutiny. We report a rare case of oral focal mucinosis affecting a 54-year-old man who presented with a sessile gingival growth mimicking a common tumour-like lesion.

Bilateral oral focal mucinosis on the palate of a 2-year-old child: a case report.

BACKGROUND: Oral focal mucinosis (OFM) is an uncommon benign oral lesion. The aetiology of the lesion is unknown. Histologically, it appears as a well-circumscribed myxomatous mass surrounded by denser, collagenous connective tissue. Most cases of OFM were found in adults. It is very unusual for young children to have OFM. CASE REPORT: A case of OFM in a 2-year-old child is reported. The patient was presented with non-painful bilateral enlargements on the palate. The overlying mucosa was smooth and not ulcerated and appeared in the same colour as the adjacent tissue. The histology of the lesion showed myxomatous mass indicative of OFM. Treatment consisted of surgically removing the lesions under general anaesthetic. CONCLUSION: Paediatric dentists should consider OFM in their differential diagnosis of soft tissue oral lesions in children.

Slow-growing gingival mass.

OBJECTIVES: A 33-year-old woman presented with a slow growing palatal gingival mass. The clinical differential diagnosis included benign tumors and tumor-like lesions, including the pyogenic granuloma, peripheral giant cell granuloma, peripheral ossifying fibroma, giant cell fibroma, peripheral odontogenic tumors, and oral focal mucinosis. STUDY DESIGN: The lesion was excised and histopathological examination followed by immunohistochemical staining was carried out. RESULTS: The microscopic findings and the immunohistochemical reactivity was diagnostic for a nerve sheath myxoma. CONCLUSIONS: The clinical features, microscopic findings, immunohistochemistry, and the differential diagnosis including the relationship to the neurothekeoma are discussed.

Treatment of acral persistent papular mucinosis with electrocoagulation.

BACKGROUND: Acral persistent papular mucinosis is a rare localized form of lichen myxedematosus with few case reports and no documented therapeutic options. OBJECTIVE: To report full resolution of acral persistent papular mucinosis after electrocoagulation. METHODS: Case report of a 51-year-old white female diagnosed with an acral persistent papular mucinosis. The clinical and histopathologic features, treatment provided, and response to treatment are detailed. RESULTS: Acral persistent papular mucinosis presented as multiple asymptomatic normochromic papules on the wrists. Treatment with topical and intralesional steroids was unsatisfactory. Gentle electrocoagulation led to complete resolution of the lesions and negligible scarring. The favorable results remained for 6 months of follow-up, and no new lesions have emerged. CONCLUSION: Our case of acral persistent papular mucinosis was successfully treated with electrocoagulation and long-lasting, excellent cosmetic results.

Cutaneous focal mucinosis arising from the chin.

Cutaneous focal mucinosis is a type of degenerative-inflammatory dermal mucinoses characterized with asymptomatic, single, dermal mucin deposition. Because of its rarity, it is often mistaken clinically for other disorders such as sebaceous cyst, fibroma, myxoma, and xanthoma. In this study, we will discuss a case of cutaneous focal mucinosis arising from the chin of a 27-year-old man.

Oral focal mucinosis associated with cervical external root resorption: a case report.

BACKGROUND: Oral focal mucinosis (OFM) and cervical external root resorption are both rare lesions with an unknown etiology. In this article we report on a rare occurrence of OFM associated with cervical external root resorption. METHODS: A 44-year-old female patient presented with sharp pain while drinking cold beverages and a firm localized gingival overgrowth adjacent to the buccal aspect of tooth #19. An excisional biopsy of the gingival lesion was taken and a deep cavity was found in the buccal aspect of the root. The cavity was restored with an amalgam filling. RESULTS: The histological findings were of a well-circumscribed lesion composed of myxomatous connective tissue, which contained numerous stellate-shaped fibroblasts. Based on the findings, a diagnosis of OFM was made. CONCLUSION: A rare case of OFM and cervical external root resorption occurring in the same site was described. A possible link between these 2 phenomena was hypothesized.

Oral focal mucinosis: report of two cases / Mucinose oral focal: relato de dois casos

Purpose: Oral focal mucinosis (OFM), an oral counterpart of cutaneous focal mucinosis, is a rare disease of unknown etiology. Its pathogenesis may be due to overproduction of hyaluronic acid by fibroblast at the expense of collagen production, resulting in focal myxoid degeneration of connective tissue primarily affecting the mucosa overlying bone. It has no distinctive clinical features, since the diagnosis is solely based on histopathological features. This paper reports two cases and discusses clinicopathological, immunohistochemical features and differential diagnosis of myxomatous lesions of the oral cavity. Case description: The two cases of OFM lesions were present in a 50 year-old patient on the hard palate and in a 26 year-old female patient in the mandible, which seem to be the first report in the Indian population. Conclusion: The histopathological and immunohistochemical analysis of Vimentin and S-100 protein may play a vital role in the correct diagnosis of OFM.

Objetivo: Mucinose oral focal (MOF), uma lesão equivalente à mucinose cutânea focal, é uma doença rara de etiologia desconhecida. Sua patogênese pode ser devido a superprodução de ácido hialurônico pelo fibroblasto às expensas de produção de colágeno, resultando em degeneração mixoide focal de tecido conjuntivo primariamente afetando a mucosa sobre o osso. Não tem características clínicas distintas e o diagnóstico é baseado somente em características histopatológicas. Este artigo relata dois casos e discute as características clinico-patológicas e imuno-histoquímicas, bem como o diagnóstico diferencial de lesões mixomatosas da cavidade bucal. Descrição dos casos: Os dois casos de lesões de MOF estavam presentes no palato duro de um paciente do sexo masculino, de 50 anos de idade, e na mandíbula de uma paciente do sexo feminino, de 26 anos. Estes parecem ser os primeiros casos relatados na população da India. Conclusão: A análise histopatológica e imuno-histoquímica de Vimentin e proteína S-100 podem ter um papel importante no correto diagnóstico de MOF.

An unusual inguino-scrotal presentation of disseminated peritoneal adenomucinosis.

We report a rare case of disseminated peritoneal adenomucinosis presenting as an inguino-scrotal mass. Imaging studies showed the presence of a mass, with liquid content, expanding from right colon into the retrovesical space and downward into the right inguinal canal. Intraoperatively, we observed and removed an appendiceal gelatinous mass. The inguinal-scrotal mass consisted of a hernial sac filled with mucinous material. Histological examination allowed the diagnosis of peritoneal adenomucinosis.

Nodular mucinosis of the breast: a case report with pathologic, ultrasonographic, and clinical findings and review of the literature.

Nodular mucinosis is an extremely rare breast lesion. This benign mass usually presents clinically as a poorly circumscribed, subareolar, myxoid mass in young female patients. We report a case of this rare breast lesion and discuss its clinical, radiologic, and histopathologic features. A 21-year-old white woman presented with a mass in the left breast of 6 months' duration. She had never been pregnant or had any history of breast feeding, surgery, trauma, or use of exogenous hormones or a family history of breast cancer. Clinical breast examination demonstrated a 1 cm "rubbery" mass directly under and continuous with the left nipple. The skin that covered the mass had an edematous and irregular appearance without erythema or drainage from the nipple. Ultrasonography demonstrated a 1-cm, nonintraductal, circumscribed, homogeneous, isoechoic mass that was continuous or part of the base of the left nipple. The mass was smooth, with a thin echogenic rim. Doppler flow showed some vascularity. These findings suggested a benign breast lesion, including a fibroadenoma or nipple adenoma. Despite reassurance, the patient desired excision of the lesion. Gross examination revealed a nodular, rubbery-firm, ovoid, pink, polypoid mass that measured 1.5 x 0.9 x 0.8 cm. Microscopic examination showed a well-circumscribed tumor with a nodular appearance, which consisted of an accumulation of pink myxoid tissue and contained spindle cells with bland-appearing nuclei, no mitosis, and mild cellularity. The pink myxoid tissue was stained with Hale colloidal iron and Alcian blue. The Alcian blue stain was removed by pretreatment with hyaluronidase. The spindle cells stained with vimentin and smooth muscle actin; however, they did not express smooth muscle myosin or cytokeratin. This report presents and discusses the pathologic, ultrasonographic, and clinical findings of this rare entity.

Nodular mucinosis of the breast: a case report with clinical and imaging findings.

We present a very rare case of nodular mucinosis of the breast. A 30-year-old woman noticed a right breast lump and consulted at our hospital because it gradually increased in size. On physical examination, the lump was 30 x 25 mm in size, and was located in the upper outer quadrant close to the nipple of the right breast. It was well-demarcated, mobile and hard. Ultrasonography (US) showed a clearly circumscribed, lobulated, and homogeneous hypoechoic lesion. Mammography (MMG) showed a round-lobular-shaped radiopaque mass without microcalcifications or spicula formation. Fine needle aspiration cytology (FNA) revealed no malignancy and mucin. Histologically, the excised tumor consisted of an abundant myxoid substance with scattered spindle cells without epithelial elements in the mucous lake. The mucinous substance stained positively with Alcian blue. Nodular mucinosis, simulating mucinous carcinoma or phyllodes tumor on clinical and imaging examinations, should be included in the differential diagnosis in cases of mucinous lesions occurring near the nipple in a young woman.

Treating REM syndrome with the pulsed dye laser.

BACKGROUND AND OBJECTIVE: REM syndrome (reticular erythematous mucinosis) is a benign but bothersome skin disease that common occurs in middle age and among women. Local and systemic treatment measures are often associated with a high rate of side effects and relapses are common. We evaluated the pulsed dye laser as an alternative method because of its good efficacy in vascular skin diseases. STUDY DESIGN/MATERIALS AND METHODS: We treated two female patients with REM syndrome using the pulsed dye laser. RESULTS: In both patients the erythematous skin changes were almost completely removed after five and three laser sessions, respectively. Other than transient hypopigmentation, no side effects occurred. In one case there is still no evidence of recurrence 6 years after a trial treatment was conducted. In the same patient, clinical success was histologically confirmed. CONCLUSIONS: This is the first report on the successful treatment of REM syndrome of two female patients with the pulsed dye laser.