Résumé
Cutaneous focal mucinosis is a rare condition that manifests with asymptomatic small white to flesh-colored papules on the face, neck, trunk and extremities. Johnson and Helwig first described it in 1961 and it is histologically characterized by localized accumulation of mucin in the upper and mid-dermis with scattered spindle-shaped fibroblasts. We report here on a case of cutaneous focal mucinosis that presented as a nodule with several small papules on it on the face.
Sujets)
Humains , Membres , Fibroblastes , Mucinoses , Mucines , CouRésumé
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.
Sujets)
Humains , Mâle , Femelle , Adulte , Adulte d'âge moyen , Mucinoses/chirurgie , Mucinoses/diagnostic , Muqueuse de la bouche/anatomopathologie , Acide hyaluroniqueRésumé
Cutaneous focal mucinosis is a rare condition characterized with asymptomatic, single, dermal mucin deposition anywhere on the body surface except on the joint surfaces of the hands, wrists and feet. Histopathologically, homogenous mucinous material is deposited in the dermis to replace dermal composition mucin. We herein report a case of cutaneous focal mucinosis with the review of the literature.
Sujets)
Derme , Pied , Main , Articulations , Mucinoses , Mucines , PoignetRésumé
Cutaneous focal mucinosis was first described by Johnson and Helwig in 1961 and is histo logically characterized by localized accumulation of mucin in the upper and middermis with scattered spindle-shaped fibroblasts. The mucin is jelly-like acid mucopolysaccharide consisting of hyaluronic acid bound to small amounts of chondroitin sulfate and heparin. The pathogenesis of cutaneous mucinosis is unclear. However, studies of various cutaneous mucinoses suggest that there may be an unknown circulating factor that stimulates fibroblasts to produce this material. Differential diagnoses include papular mucinosis, myxoid cyst, cutaneous myxoma. Simple surgical excision is the treatment of choice. In the present case, cutaneous focal mucinosis occurs on the nail bed with onycho mycosis.
Sujets)
Diagnostic différentielRésumé
Cutaneous focal mucinosis is a condition with an asymptomatic, small, dermal, white to flesh-colored papule with a predilection for the face, neck, trunk, and extremities. This is usually presented as solitary lesion but rarely as multiple papules or nodules. Histologically, a localized area of the collagen in the upper dermis is largely replaced by homogenous mucinous material in which spindle-shaped fibroblasts are scattered. We report a case of cutaneous focal mucinosis which was presented as multiple lesions covering widespread skin lesion throught the face, chest and back.
Sujets)
Collagène , Derme , Membres , Fibroblastes , Mucinoses , Mucines , Cou , Peau , ThoraxRésumé
A solitary, asymptomatic lesion, clinically resembling a transpare it cyst, has been present for 4 months in the vaginal mucosa of a 43-year-old healthy woman. An xcisional biopsy revealed the lesion to be a focal accumulatition of hyaluronic acid replacing the collagen fibers and fibroblastic proliferation in the dermis consistant with cutaneous focal mucinosis. During the follow-up period for 3 months, no recurrence was observed.
Sujets)
Adulte , Femelle , Humains , Biopsie , Collagène , Derme , Fibroblastes , Études de suivi , Acide hyaluronique , Mucinoses , Muqueuse , RécidiveRésumé
A solitary, asymptomatic lesion, clinically resembling a transpare it cyst, has been present for 4 months in the vaginal mucosa of a 43-year-old healthy woman. An xcisional biopsy revealed the lesion to be a focal accumulatition of hyaluronic acid replacing the collagen fibers and fibroblastic proliferation in the dermis consistant with cutaneous focal mucinosis. During the follow-up period for 3 months, no recurrence was observed.
Sujets)
Adulte , Femelle , Humains , Biopsie , Collagène , Derme , Fibroblastes , Études de suivi , Acide hyaluronique , Mucinoses , Muqueuse , RécidiveRésumé
We report a case of multiple cutaneous focal mucinosis in a 22 year-old male, who presented with multiple, asymptomatic, nodular lesions on the left upper trunk and left arm which had persisted for about one year. They were 2 to 17 mm in size, firm, yellowish, dome-shaped elevated, slightly movable papules or nodules which were distributed over the area of the left C3-7 and TI-3 sensory dermatomes. The histopathologic findings showed homogeneous mucinous material in the upper dermis. The material was confirmed to be hyaluronic add by, alcian blue stain. Some of the lesions showed improvement with intmlesional injection of triamcinolone acetonide. Such an entity, to the best of our knowledge, has not yet been reported.