Cutaneous Mucinosis of Infancy Report of a Rare Case and Review of the Literature
Ethiop J Health Sci. 2013 Jul; 23(2): 178–182.
Oral Focal Mucinosis: A Rare Case Report of Two Cases
Sharma Ena
1Department of Pediodontics, Oxford Dental College and Infirmary, Bangalore
Nadellamanjari
aneDepartment of Pediodontics, Oxford Dental College and Hospital, Bangalore
Chatterjeeanirban
1Department of Pediodontics, Oxford Dental Higher and Infirmary, Bangalore
Alampalli Ramesh
oneSection of Pediodontics, Oxford Dental College and Hospital, Bangalore
Abstract
Oral focal mucinosis (OFM) is a rare soft tissue lesion of unknown etiology. Clinically, it is near commonly constitute on the gingiva and presents as a painless, sessile or pedunculated mass of the same colour as the surrounding mucosa. Histologically, it is characterized by focal myxoid degeneration of connective tissue. OFM occurs predominantly in adults during the 4th and fifth decade of life, although it has been reported infrequently in children and adolescents. Its diagnosis mainly relies on histological analysis and the handling involves complete surgical excision. Its recurrence is unreported. The aim of this report of ii cases is to depict the clinical and histological presentation and subsequent management of OFM. The cause of OFM remains unknown. The cases presented in this study bring OFM to the attention of anatomical pathologists while considering the differential diagnosis of myxoid lesions of the oral crenel.
Keywords: Oral focal mucinosis, Soft tissue lesions, Gingival lesions
Introduction
Oral Focal Mucinosis (OFM) is an oral analogue of dermal lesion known as coetaneous focal mucinosis (CFM) or cutaneous myxiod cyst which is misdiagnosed every bit intraoral myxoma(1). OFM is presented as an innocuous swelling that may be pedunculated or sessile. The gingiva was confirmed as the nigh mutual site for OFM, with predominance in females. It has an unknown etiology, peradventure from overproduction of hyaluronic acid by fibroblasts (2). Clinically, the lesion appears as asymptomatic round elevations, which are histologically characterized by a localized area of myxomatous connective tissue containing mucinous material surrounded by relatively dense collagenous connective tissues(3).
Histopathologically, fibroblast-like cells and foamy cells diffusely increased in a well-localized area of myxoid matrix, surrounded past collagenous fibrous connective tissue. Histochemically, fibroblast-like cells, foamy cells and myxoid matrix were revealed on metachromasia with toluidine bluish at pH4.one and pH 7.0 (three,four Information technology was start described in 1974 by). Tomich who reported 8 cases equally oral counterpart of cutaneous focal mucinosis. He stated that most lesions, if non all, diagnosed as oral soft tissue myxomas are in reality lesion of this entity (5).
These ii cases are significant clinically besides as histopathologically. The mucosa straight overlying bone appears to exist especially vulnerable. The lesions by and large show no color change (3, 6). The lesions are difficult to diagnose clinically every bit there are no clinical distinctive features. They are most commonly diagnosed equally fibroma or granuloma. Thus, the histopathological diagnosis becomes of import in these conditions (vii).
Description of the Cases
Case ane
A 26 years old male patient was reported with a master complaint of enlargement of gums in in relation to upper anterior teeth, with a history of trauma one yr back when he noticed a swelling which grew to the present size. On examination, well-demarcated, ruddy, pedunculated, partly polish and shiny with pebbeled surface, blanching, haemorrhage, pinpoints, ovoid growth measuring about 15mmx 15mm in its greatest dimension was noted on the left side of the anterior teeth in relation to 22 and 23 extending upwardly to mucogingival junction. An excisional biopsy was performed with scalpel to excise the tissue which was stored in x% formalin and sent for histopathological examination.
Case 2
A 36-year-old female reported a chief complaint of an asymptomatic enlargement on the gingiva labial to 21. The swelling was initially minor which gradually attained the present size. The lesion was approximately 8mm × 7mm in diameter and round in shape, business firm, sessile and not-tender to palpation. The overlying mucosal surface was shine, not ulcerated and showed no colour change. The lesion was extending from distal aspect of 21 medially to mesial aspect of 11. Excisional biopsy was performed with diode light amplification by stimulated emission of radiation (320nm).
Histopathological findings
Hematoxylin and eosin stained microscopic slides of both cases revealed a stratified squamous hyper-parakeratinized epithelium and the underlying connective tissue stroma was composed of loose fibro-myxoid stroma with stellate shaped fibroblasts. Deeper stroma showed spindle shaped fibroblasts interspersed between sparse collagen cobweb bundles and numerous small blood capillaries.
Clinical Course
The patients were observed regularly; both cases healed satisfactorily with no complications or recurrence in last 8 months and six months respectively.
Give-and-take
OFM is a rare clinico-pathologic status considered to be the microscopic counterpart of the cutaneous focal mucinosis or cutaneous myxoid cyst (one, 5).Three-fourths of all cases occur on the gingiva and the hard palate and presents every bit a sessile, soft, painless nodule with normal surface coloration, sometimes with a somewhat pale appearance (8).
Oral focal mucinosis consists of a submucosal, well-localized but non-encapsulated nidus of very loose, myxomatous or "mucinous" connective tissue. More than superficial lesions may produce cloudburst and loss of rete ridges of the overlying squamous epithelium. Fibroblasts are seen in minimal inside the mucinous expanse, often demonstrating fragile, fibrillar processes. The mucinous zone is much less vascular than the surrounding connective tissues, and inflammatory cells are not associated with the lesion except equally a perivascular infiltrate of lymphocytic T-cells at the periphery (9).
A slight similarity is also seen between mucinosis and the nervus sheath myxoma, a variant of neurofibroma which rarely affects mucosa of the upper aerodigestive tract. The nerve sheath myxoma is more circumscribed, has gristly septa between multiple myxoid nodules, and has more plump stromal cells (10).
Oral focal mucinosis is treated by conservative surgical removal. It does not recur with gingival excision.
In the outset case, there is well-demarcated, reddish, pedunculated, partly smooth and shiny with peddled surface near 23, ovoid growth measuring about 15mm× 15mm in its greatest dimension. The second instance occurred as a sessile gingival mass, measuring nearly 8mmx 6mm with smooth non ulcerated soft to firm mass.
OFM has no distinctive clinical features and most often thought to be clinically as fibroma, pyogenic granuloma, mucocele or like lesions. Traumatic fibroma, pyogenic granuloma and minor salivary gland tumor were the differential diagnosis (7). A review of all reported cases prove that it was never diagnosed clinically as 'oral focal mucinosis' (four, 7).The histological features are always the basis for the diagnosis. Microscopic examination of both cases showed a well-localized but non-encapsulated surface area of loose, myxomatous connective tissue stroma surrounded by dumbo, normal sparse collagen bundles. The sub-epithelial pale eosinophilic myxoid stromas representing an overproduction of hyaluronic acid with stellate shaped fibroblasts were noted. Deeper stroma showed spindle fibroblasts, sparse bundles of collagen fibers and numerous small blood capillaries forth with lengthened infiltration of mixed inflammatory cells consisting of mainly lymphocytes and plasma cells. All these features were suggestive of oral focal mucinosis (10).
It must be emphasized that in many focal gingival lesions, clinically preoperative diagnosis is impossible due to their rarity. However, OFM should be considered in the differential diagnosis of soft tissue lesion in adults. The histopathological evaluation is always the basis of diagnosis. Out of 52 cases reported, 33 cases have been noted on the gingiva, 10 cases on the hard palate and 3 cases each on alveolar mucosa, buccal mucosa, and tongue; and 2 cases on lips. Oral focal mucinosis rarely recurs after surgical excision.
Preoperative view of the lesion
Surgically excised tissue
Mail service operative view of lesion afterwards 8 months
Preoperative view of lesion wrt 11, 21
Post operative view of lesion afterward 6months
Histopathological picture of lesion
References
1. Neville BW, Damm DD, Allen CM, Bouquot JE. Oral and maxillo facial pathology. 2nd ed. Philadelphia, Pennsylvania: Elsevier; 2002. [Google Scholar]
two. Buchner A, Merrell Pw, Leider AS, Hansen LS. Oral focal mucinosis. Int J Oral Maxillofac Surg. 1990;xix:337–340. [PubMed] [Google Scholar]
3. Madhusudhan As, Nagarajappa D, Manjunatha BS, Saawarn Swati, Charan Babu HS. Oral focal mucinosis: report of two cases. Rev Odonto Cienc. 2010;25:310–313. [Google Scholar]
iv. Lima AAS, Machado MAN, Martins WD, Grégio AMT, Dirschnabel AJ, three, Mattioli TMF, et al. Oral focal mucinosis. Quintessence Int. 2008;39:611–615. [PubMed] [Google Scholar]
five. Tomich CE. Oral focal mucinosis: A clinic pathologic and histochemical 4. Study of viii cases. Oral Surg. 1974;38:714–724. [PubMed] [Google Scholar]
6. Saito I, Ide F, Enomoto T, Kudo I. Oral focal mucinosis. J Oral Maxillofac Surg. 1985;43(5):372–374. [PubMed] [Google Scholar]
7. Aldred MJ, Talacko AA, Ruljancich M, Story RD, Newland S, Chen ST, vii, et al. Oral focal mucinosis: report of xv cases and review of the literature. Pathology. 2003;35:393–396. [PubMed] [Google Scholar]
viii. Reed RJ, Clark WH, Mihm MC. The cutaneous mucinosis. Hum Pathol 6. 1973;4:201–207. [PubMed] [Google Scholar]
9. Green TL, Leighty SM, Walters R. Immunohistochemical evaluation of oral myxoid lesions. Oral Surg Oral Med Oral Pathol. 1992;73:469–471. [PubMed] [Google Scholar]
10. Wilk M, Schmoeckel C. Cutaneous focal mucinosis: A histopathological and immunohistochemical analysis of 11 cases. J Cutan Pathol. 1994;21:446–452. [PubMed] [Google Scholar]
Articles from Ethiopian Periodical of Wellness Sciences are provided here courtesy of College of Public Health and Medical Sciences of Jimma University
Source: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3742896/
0 Response to "Cutaneous Mucinosis of Infancy Report of a Rare Case and Review of the Literature"
Post a Comment