Primary paediatric cutaneous mucinoses.

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.

Diagnosis and treatment of oral focal mucinosis: a case series.

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.

Reticular Erythematous Mucinosis: A Rare Cutaneous.

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.

Mucinosis nodular asociada a gammapatía monoclonal de cadenas ligeras de significado incierto / Nodular mucinosis associated with light-chain monoclonal gammopathy of uncertain significance

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Oral focal mucinosis: a rare case report of two cases.

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.

Oral focal mucinosis: review of the literature and seven additional cases.

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.

Pimecrolimus 1 percent cream and pulsed dye laser in treatment of a patient with reticular erythematous mucinosis syndrome.

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.

Mucinosis papular acral persistente / Acral persistent papular mucinosis

Las mucinosis cutáneas, también denominadas liquen mixedematoso, son un grupo de enfermedades caracterizadas por el depósito de mucina en la piel. Pueden asociarse a enfermedades sistémicas (escleromixedema) o no (mucinosis papulares o liquen mixedematoso localizado), aunque en algunos casos comparten hallazgos comunes (formas intermedias o atípicas). La mucinosis papulosa acral persistente (MPAP) es un tipo de mucinosis papular que se localiza exclusivamente en el dorso de las manos y en la zona distal de los antebrazos y no se asocia a ninguna enfermedad sistémica. Presentamos el caso de una mujer sana de 52 años que presentaba lesiones cutáneas en el dorso de las manos, cuyo estudio histológico confirmó una MPAP

Cutaneous mucinoses, also called lichen myxoedematosus, are a group of diseases characterized by mucin deposit on the skin. They may be associated to systemic diseases (scleromyxedema) or be primary ones (papular mucinosis, localized lichen myxoedematous), although they share common findings (intermediate or atypical forms) in some cases. Acral persistent papular mucinosis (APPM) is a type of papular mucinosis that is located exclusively on the back of the hands and in the distal zone of the forearms and is not associated to any systemic disease. We present the case of a 52-year-old healthy woman who had skin lesions on the back of her hands and whose histological study confirmed an APPM

Generalized mucinosis in a patient with erythroderma.

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.

Diffuse cutaneous mucinoses.

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.

Cutaneous focal mucinosis: a case report.

Cutaneous or dermal mucinoses are a heterogeneous group of connective tissue disorders in which there is an accumulation of mucin in the skin or within the hair follicle. They have a number of different morphologic presentations and can often be confused with other diseases. We report a 12-year-old Chinese girl with a 1-year history of a well-defined, hypopigmented plaque on her chin, consistent with cutaneous focal mucinosis, a rare condition in children.

Koebner phenomenon due to scratch test in scleromyxoedema.

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.

Mucinosis eritematosa reticular: a proposito de dos casos / Reticular eritematous mucinosis: a propouse of too cases

Se presentan dos pacientes con mucinosis eritematosa reticular (M.E.R.) en edad media de la vida. Realizamos una revisión de la literatura, para actualizar conceptos de la clínica y patología

Mucinosis eritematosa reticular: a proposito de dos casos / Reticular eritematous mucinosis: a propouse of too cases

Se presentan dos pacientes con mucinosis eritematosa reticular (M.E.R.) en edad media de la vida. Realizamos una revisión de la literatura, para actualizar conceptos de la clínica y patología(AU)

Treatment of reticular erythematous mucinosis with a large dose of ultraviolet B radiation and steroid impregnated tape.

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.