RESUMEN
BACKGROUND: Primary cutaneous mucinoses are a heterogeneous group of diseases characterized by the abnormal deposition of glycosaminoglycans (mucin) in the skin. This is the main histopathological clue resulting in distinctive clinical presentations. They are usually diseases of adult age and are uncommon in children, in whom diagnosis and management are difficult tasks. In particular, the classification of primary cutaneous mucinoses occurring in childhood is a difficult and confusing challenge because of the very limited number of described cases and overlaps in their clinicopathological features in the absence of reports of homogeneous case series. OBJECTIVES: The aim of this review is to help dermatologists and paediatricians to identify patients with cutaneous mucinoses at a paediatric age and to enable institution of appropriate management. A classification of primary paediatric cutaneous mucinoses is also proposed. RESULTS: New investigations have yielded significant insights into a classification of paediatric cutaneous mucinoses. CONCLUSIONS: The current update tries to offer a more meaningful guide to the diagnosis and treatment of these rare diseases in paediatric patients. What's already known about this topic? The nosography of primary cutaneous mucinoses occurring in childhood is confusing because of the very limited number of cases and overlaps in their clinicopathological features in the absence of homogeneous case series. The diagnosis and management of paediatric patients with cutaneous mucinoses are also difficult as these diseases are rarely seen in children. What does this study add? New investigations have yielded significant insights into a classification of paediatric cutaneous mucinoses. The current update tries to offer a more meaningful guide to diagnosis and treatment of these rare diseases in patients of paediatric age.
Asunto(s)
Mucinosis , Administración Cutánea , Adulto , Niño , Glicosaminoglicanos , Humanos , Mucinosis/diagnóstico , Mucinosis/terapia , PielRESUMEN
Oral focal mucinosis (OFM) is a rare lesion first described in 1974, but the aetiology remains unknown. Clinically, OFM presents as an asymptomatic nodular lesion and the similarity of clinical features to other soft tissue injuries makes the diagnosis difficult. The aim of this study was to integrate the demographic, clinical, and histopathological characteristics from previously published cases of OFM into a systematic review. Electronic searches without publication date restriction were performed in the following databases: Embase, PubMed, Medline, Web of Science, and Scopus. Case reports or case series of OFM published in English and presenting enough clinical and histopathological information were included. This systematic review identified 42 studies from 12 countries, comprising 113 cases of OFM. This lesion affected more females than males, usually in the fourth decade of life. The gingiva was the most common anatomical location, followed by the palate. Clinical presentation was most often an asymptomatic nodule. Imaging exams revealed that most cases did not have bone involvement. Surgical removal was the treatment of choice for most cases and only one recurrent case was reported. In conclusion, OFM is an uncommon pathology, and its diagnosis depends on histopathological analysis. The lesion could be included as a differential diagnosis of benign soft tissue lesions of the oral cavity, especially those affecting the gingiva.
Asunto(s)
Enfermedades de la Boca , Mucinosis , Humanos , Diagnóstico Diferencial , Enfermedades de la Boca/diagnóstico , Enfermedades de la Boca/patología , Enfermedades de la Boca/terapia , Mucinosis/diagnóstico , Mucinosis/patología , Mucinosis/terapiaRESUMEN
Oral focal mucinosis (OFM) is a rare, asymptomatic, benign lesion of unknown etiology that usually involves the mandibular gingiva. This article reports on seven patients, six of whom had lesions that involved the gingiva and one that involved the palate. All cases demonstrated the classic lobular, myxomatous mesenchymal tissue with stellate cells. In all cases, immunohistochemical staining for S-100-protein expression was negative, thus excluding the principal differential diagnoses of myxoid neurofibroma and neurothekeoma (nerve sheath myxoma). This article seeks to bring OFM to the attention of clinicians and pathologists who must consider the differential diagnosis of gingival and palatal nodules.
Asunto(s)
Fibroma/patología , Enfermedades de las Encías/patología , Mucosa Bucal/patología , Mucinosis/patología , Adulto , Femenino , Enfermedades de las Encías/terapia , Humanos , Masculino , Persona de Mediana Edad , Mucinosis/terapia , Paladar DuroRESUMEN
Reticular erythematous mucinosis (REM) is a rare form of primary cutaneous mucinosis, most often involving the midline of the upper chest or back in middle-aged women. REM bears clinical and histopathologic resemblance to lupus erythematosus tumidus (LET), dermatomyositis, scleredema, and lichen myxedematosus. Early recognition and diagnosis of REM is particularly relevant to exclude the abovementioned diseases, as REM is more benign and has fewer systemic consequences.
Asunto(s)
Mucinosis/diagnóstico , Enfermedades de la Piel/diagnóstico , Diagnóstico Diferencial , Humanos , Mucinosis/etiología , Mucinosis/terapia , Enfermedades de la Piel/etiología , Enfermedades de la Piel/terapiaRESUMEN
BACKGROUND: Oral focal mucinosis, the oral counterpart of cutaneous focal mucinosis, is a rare disease. As it has no characteristic clinical or radiological features, diagnosis is established by histopathological and immunohistological examination. We present three cases of oral focal mucinosis occurring in the retromolar (which is extremely rare) and gingival regions. CASE PRESENTATION: Case 1 involved a 26-year-old Japanese man with radiolucency in the right retromolar region on panoramic radiograph and computed tomography; no obvious protrusion was observed in the region. This finding was clinically diagnosed as a tumor of the retromolar region. Case 2 involved a 60-year-old Japanese woman. A tumor-like mass of tissue was identified on the buccal gingiva at the maxillary right canine and first premolar region. The lesion measured 7 × 6 mm and exhibited elastic hardness and healthy-colored mucosa. The lesion was diagnosed as an epulis. Case 3 involved a 47-year-old Japanese woman. A tumor-like mass of tissue was identified on the buccal gingiva at the maxillary right canine and first premolar region. The lesion measured 10 × 10 mm and exhibited elastic hardness and redness of the surface mucosa. This lesion was also diagnosed as an epulis. Resection was performed in all three cases, and the lesions were histopathologically diagnosed as oral focal mucinosis. Postoperative courses were uneventful and, thus far, there have been no recurrences. CONCLUSIONS: Although it is difficult to diagnose oral focal mucinosis based on clinical symptoms and imaging findings, the disease should be considered a possibility when diagnosing benign oral tumors. We believe that an emphasis on histopathologic study is essential to confirm the clinical suspicion.
Asunto(s)
Encía/patología , Enfermedades de las Encías/diagnóstico , Neoplasias de la Boca/diagnóstico , Mucinosis/patología , Mixoma/patología , Adulto , Diagnóstico Diferencial , Femenino , Enfermedades de las Encías/patología , Enfermedades de las Encías/terapia , Humanos , Masculino , Persona de Mediana Edad , Mucinosis/terapia , Mixoma/terapia , Procedimientos Quirúrgicos Ortognáticos , Radiografía , Resultado del TratamientoRESUMEN
We report on the efficacy of twice daily application of pimecrolimus 1 percent cream in a 48-year-old woman with reticular erythematous mucinosis (REM) syndrome and compare its results with pulsed dye laser (PDL) on the other side of her chest and back. The patient was previously treated by hydroxychloroquine but only a fair response was observed. After application of 5 months of pimecrolimus, the lesions completely resolved and the result was comparable with the other side of her body treated by pulsed dye laser PDL. Topical pimecrolimus and pulsed dye laser appear to be effective and safe treatments for REM.
Asunto(s)
Eritema/patología , Eritema/terapia , Terapia por Luz de Baja Intensidad/métodos , Mucinosis/patología , Mucinosis/terapia , Tacrolimus/análogos & derivados , Administración Tópica , Biopsia con Aguja , Terapia Combinada , Femenino , Estudios de Seguimiento , Humanos , Inmunohistoquímica , Persona de Mediana Edad , Síndrome , Tacrolimus/uso terapéutico , Resultado del TratamientoRESUMEN
The cutaneous mucinoses are a complex group of dermatologic diseases with local, follicular, or diffuse disease. The diffuse cutaneous mucinoses are remarkable not only for their dermal disease, but also for the numerous systemic manifestations. It is important that the clinical dermatologist be able accurately to diagnose and differentiate scleredema, scleromyxedema, REM, generalized myxedema of hypothyroidism, and pretibial myxedema of hyperthyroidism. Because of the variability of associated systemic manifestations, some with substantial morbidity and mortality, accurate diagnosis is vital for awareness and appropriate management.
Asunto(s)
Mucinosis , Enfermedades de la Piel , Humanos , Hipotiroidismo/complicaciones , Mucinosis/clasificación , Mucinosis/complicaciones , Mucinosis/diagnóstico , Mucinosis/terapia , Mixedema/complicaciones , Mixedema/diagnóstico , Mixedema/patología , Pronóstico , Escleredema del Adulto/complicaciones , Escleredema del Adulto/diagnóstico , Escleredema del Adulto/terapia , Enfermedades de la Piel/clasificación , Enfermedades de la Piel/complicaciones , Enfermedades de la Piel/diagnóstico , Enfermedades de la Piel/terapiaAsunto(s)
Terapia por Láser , Dermatosis de la Pierna/terapia , Mucinosis/terapia , Anciano , Femenino , Humanos , Láseres de GasRESUMEN
To date, there are no successful treatments for reticular erythematous mucinosis (REM) other than antimalarial drugs such as chloroquine; these have many adverse side effects and are prohibited for use in Japan. We report a case of REM improved by a large dose of ultraviolet B (UVB) radiation and a steroid impregnated tape. The large dose of UVB radiation improved the erythema after 5 weeks. Application of steroid impregnated tape to a lesion where a large dose of UVB had been given produced an additive clinical effect. UVB radiation and steroid impregnated tape are considered to be effective treatments for REM.
Asunto(s)
Mucinosis/terapia , Enfermedades de la Piel/terapia , Esteroides/administración & dosificación , Terapia Ultravioleta , Terapia Combinada , Eritema/terapia , Humanos , Masculino , Persona de Mediana Edad , Dosificación RadioterapéuticaRESUMEN
Cutaneous mucinoses are a heterogeneous group of disorders in which mucin accumulates in the skin or in the follicles. Mucin is a gelatinous substance composed of glycosaminoglycanes, especially hyaluronic acid and dermatan sulfate bound to small quantities of chondoitin sulfate and heparin sulfate. Though the causes of mucinoses remain unknown, they can be divided into distinctive cutaneous (primary) mucinoses, in which mucin deposition is the distinctive histological sign resulting in clinically distinctive lesions, and cutaneous disorders, in which mucin deposition is an epiphenomenon (secondary mucinoses). Histologically, mucin is recognized after special staining techniques using alcian blue and colloidal iron. The microscopic localization of the mucin deposit is used to distinguish between dermal and follicular forms of primary mucinoses and between epidermal, dermal and follicular forms of secondary mucinoses. We present here the clinical and histological features of primary cutaneous mucinoses and an updated classification. The main therapeutic schemas are outlined.
Asunto(s)
Mucinosis/diagnóstico , Adulto , Niño , Femenino , Humanos , Masculino , Mucinosis/clasificación , Mucinosis/terapia , Mucinosis Folicular/clasificación , Mucinosis Folicular/diagnóstico , Mucinosis Folicular/terapiaRESUMEN
Species specific diseases, e.g. idiopathic mucinosis of the Chinese Shar Pei are reported by use of 31 clinical cases and 140 biopsy samples. Idiopathic mucinosis is one of the most frequent diseases of this increasing breed in Germany, too. The disease is diagnosed by use of histologic examination of skin biopsies. Therapy is performed by application of corticosteroids and symptomatic treatment.
Asunto(s)
Enfermedades de los Perros/patología , Mucinosis/veterinaria , Piel/patología , Corticoesteroides/uso terapéutico , Factores de Edad , Animales , Biopsia , Enfermedades de los Perros/epidemiología , Enfermedades de los Perros/terapia , Perros , Femenino , Alemania/epidemiología , Endogamia , Masculino , Mucinosis/epidemiología , Mucinosis/patología , Mucinosis/terapia , Especificidad de la EspecieRESUMEN
Oral focal mucinosis (OFM) is a rare soft tissue lesion of unknown etiology. Clinically, it is most commonly found 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 fourth and fifth decade of life, although it has been reported infrequently in children and adolescents. Its diagnosis mainly relies on histological analysis and the treatment involves complete surgical excision. Its recurrence is unreported. The aim of this report of two cases is to describe the clinical and histological presentation and subsequent management of OFM. The cause of OFM remains unknown. The cases presented in this report bring OFM to the attention of anatomical pathologists while considering the differential diagnosis of myxoid lesions of the oral cavity.
Asunto(s)
Tejido Conectivo/patología , Encía/patología , Enfermedades de las Encías/patología , Mucinosis/patología , Mixoma/patología , Adulto , Femenino , Enfermedades de las Encías/terapia , Humanos , Masculino , Mucinosis/terapia , Mixoma/terapiaAsunto(s)
Vendajes , Trajes Gravitatorios , Dermatosis de la Pierna/terapia , Mucinosis/terapia , Anciano , Anciano de 80 o más Años , Humanos , MasculinoAsunto(s)
Mucinosis/diagnóstico , Mucinosis/terapia , Adolescente , Adulto , Anciano , Niño , Femenino , Humanos , Masculino , Persona de Mediana Edad , Mucinosis Folicular , MixedemaRESUMEN
No disponible
Asunto(s)
Humanos , Femenino , Anciano , Mucinosis/complicaciones , Mucinosis/terapia , Paraproteinemias/complicaciones , Paraproteinemias/patología , Eritema/patología , Hiperqueratosis Epidermolítica/complicaciones , Hiperqueratosis Epidermolítica/patología , Papiloma/complicaciones , PronósticoRESUMEN
We describe an 81-year-old Japanese patient with erythroderma overlapping with widespread and symmetrical deposits of mucin in the upper dermis. Clinically, the mucinous lesions on the nape and upper trunk were localized papular mucinosis. Histologically, there was a perivascular infiltrate of lymphohistiocytic cells mingled with plasma cells in the upper dermis but no sclerosis. Immunohistochemical staining revealed that more than 90% of these infiltrating plasma cells produced immunoglobulin lambda-chain. Both the erythroderma and generalized mucinosis responded to topical steroid and PUVA therapy. To the best of our knowledge, this is the first case of erythroderma accompanied by generalized mucinosis.
Asunto(s)
Dermatitis Exfoliativa/patología , Mucinosis/patología , Piel/patología , Administración Cutánea , Anciano de 80 o más Años , Antiinflamatorios/uso terapéutico , Biopsia , Dermatitis Exfoliativa/diagnóstico , Dermatitis Exfoliativa/terapia , Diflucortolona/análogos & derivados , Diflucortolona/uso terapéutico , Humanos , Masculino , Mucinosis/diagnóstico , Mucinosis/terapia , Terapia PUVA/métodos , Resultado del TratamientoRESUMEN
The treatment of scleromyxoedema is notoriously difficult. We present a patient with long-standing diffuse scleromyxoedema associated with functional impairment who developed chronic idiopathic neutropenia complicated by recurrent life-threatening infections. Treatment with recombinant granulocyte--colony stimulating factor led to normalization of the neutrophil count, prevented further systemic infections, and unexpectedly was associated with a striking clinical improvement of her skin disorder and decrease in mucin deposition in the dermis.
Asunto(s)
Factor Estimulante de Colonias de Granulocitos/uso terapéutico , Mucinosis/terapia , Neutropenia/terapia , Esclerodermia Sistémica/terapia , Enfermedad Crónica , Femenino , Humanos , Persona de Mediana Edad , Mucinosis/patología , Proteínas Recombinantes/uso terapéutico , Esclerodermia Sistémica/patologíaRESUMEN
The Koebner phenomenon or isomorphic response was originally described in psoriasis and has subsequently been observed in various other diseases. We report a patient with isomorphic response in scleromyxoedema, a variant of papular mucinosis with diffuse infiltration of the skin. The Koebner phenomenon was due to a scratch test performed 4 weeks before the appearance of streaky, lichenoid infiltrations on the forearms.