Recommendations in the management of epithelial appendiceal neoplasms and peritoneal dissemination from mucinous tumours (pseudomyxoma peritonei)

The epithelial appendiceal neoplasms are uncommon and are usually detected as an unexpected surgical finding. The general surgeon should be aware of the diversity of its clinical manifestations and biological behaviors along with the significance of the surgical treatment on the progression of the illness and the prognosis of the patients. The operative findings and, especially, tumor histology, determine the type of surgery. Intestinal histologic subtype behaves and should be treated similarly to the right colon neoplasms; while mucinous tumors, often discordant between histology and its aggressiveness, can be treated with a simple appendectomy or require complex oncological surgeries. Mucinous tumors are often associated with the presence of mucin or tumor implants in the abdominal cavity, being the clinical syndrome known as pseudomyxoma peritonei (PMP). PMP tends to present an indolent but deadly evolution and requires a multimodal approach as a single treatment with curative potential: complete cytoreductive surgery plus hyperthermic Intraperitoneal chemotherapy (CCRS ? HIPEC) now considered the standard of care in this pathology. The general surgeon should be aware of the governing principles of the treatment of appendiceal neoplasms with or without peritoneal dissemination, know the therapeutic frontiers in every situation (avoiding unnecessary or counterproductive surgeries) and sending early these patients to specialised centres in the radical management of malignant diseases of the peritoneum in the conditions and with the necessary information to facilitate a possible radical treatment (AU)

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Líquen mixedematoso localizado: relatode caso e revisão de literatura / Localized lichen myxedematosus: a case report and review of the literature

Líquen mixedematoso (LM) localizado é mucinose cutânea idiopática, que pode ser classificada clinicamente em quatro formas: (1) discreta papular,envolvendo qualquer sítio cutâneo; (2) papular persistente acral, envolvendo apenas face extensora de mãos e punhos; (3) papular da infância, que constitui variante da forma discreta ou da forma acral da mucinose papular persistente; e (4) nodular. Relata-se um caso de LM, na sua forma localizada, na face de paciente do sexo masculino (AU)

Localized lichen myxedematosus (LM) is an idiopatic cutaneus mucinosis, whith can be clinically classified into four forms: (1) a discrete popular form involving any site, (2) acral persistent papular mucinosis, involving only the extensor surface of the hands and wrists, (3) papular mucinosis of infancy, a pediatric variant of the discrete form or the acral form of persistent papular mucinosis, and (4) a nodular form. A LM case, in its localized form, is described on the face of a male pacient (AU)

Pápulas en el dorso de las manos / Papules on the Dorsum of the Hands

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[Acral persistent papular mucinosis]. / Mucinosis papular acral persistente.

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.

Mucinosis papular acral persistente: presentación de un caso y revisión de la literatura / Acral popular persisntent mucinosis: case report and review of the literature

La mucinosis papular acral persistente (MPAP) es una forma clínica e histológicamente distinta de mucinosis cutánea. Esta variante afecta principalmente a mujeres, se caracteriza por pápulas de color piel, de 2 a 3 mm de diámetro, en el dorso de las manos y menos frecuentemente en los antebrazos. Habitualmente no se asocia a otras enfermedades. Presentamos una paciente de 47 años que reúne los criterios de esta entidad (AU)

Mucinosis papular acral persistente: presentación de un caso y revisión de la literatura / Acral popular persisntent mucinosis: case report and review of the literature

La mucinosis papular acral persistente (MPAP) es una forma clínica e histológicamente distinta de mucinosis cutánea. Esta variante afecta principalmente a mujeres, se caracteriza por pápulas de color piel, de 2 a 3 mm de diámetro, en el dorso de las manos y menos frecuentemente en los antebrazos. Habitualmente no se asocia a otras enfermedades. Presentamos una paciente de 47 años que reúne los criterios de esta entidad (AU)

Escleromixedema: estudio ultraestructural / Scleromyxedema: ultrastructural study

El escleromixedema es un tipo de mucinosis que presenta unas características clínicas e histopatológicas bien definidas. En este artículo se describen los hallazgos observados con el microscopio electrónico de transmisión de un caso estudiado recientemente. Se trataba de una paciente de 56 años con una erupción de pápulas liquenoides localizadas en la cara, la parte superior del tronco y en los miembros. Se acompañaba de gammapatía IgG l. El estudio con el microscopio óptico mostró los hallazgos típicos de escleromixedema. El estudio de microscopia electrónica puso de manifiesto la existencia de un elevado número de fibroblastos con una gran actividad de síntesis y liberación de fibras de colágena y sustancia mucoide

Scleromyxedema is a type of mucinosis that presents with some well defined clinical and histopathological characteristics. We describe the findings observed with transmission electron microscopy in a case that we recently studied. The patient was a 56-year-old female with a localized eruption of lichenoid papules on the face, upper trunk and limbs. It was accompanied by IgG lambda gammopathy. Optical microscopy showed the findings typical of scleromyxedema. Electron microscopy revealed the existence of a large number of fibroblasts with high activity levels in the synthesis and release of collagen fibers and a mucoid substance

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 mucinoses: microscopic criteria for diagnosis.

The clinical aspects and the histologic features of cutaneous mucinoses have been reviewed and their classification updated. Cutaneous mucinoses are divided into distinctive (primary) cutaneous mucinoses in which the mucin deposit is the main histologic feature resulting in clinically distinctive lesions, and disorders associated with histologic mucin deposition as an additional finding (secondary mucinoses). The former are further divided into degenerative-inflammatory mucinoses, which may be either dermal or follicular, and into neoplastic-hamartomatous mucinoses. Histopathologic diagnosis is particularly difficult for dermal mucinoses and requires clinicopathologic correlation. Three histologic clues, namely the pattern of mucin distribution (diffuse or focal), the level of mucin deposit in the dermis and some additional findings may help diagnosis. Follicular mucinoses have the easiest pattern to recognize histologically, but the distinction between Pinkus' follicular mucinosis and follicular mucinosis with mycosis fungoides is very difficult.Lastly, neoplastic-hamartomatous cutaneous mucinoses include mucinous nevus, a benign hamartoma, and myxoma, which is a benign tumor to be differentiated from reactive cutaneous focal mucinosis.

Cutaneous mucinosis of infancy: is it a real entity or the paediatric form of lichen myxoedematosus (papular mucinosis)?

We describe a girl presenting with a childhood dermal mucinosis in which we had the unique opportunity to find all the transitional histological features of lichen myxoedematosus (papular mucinosis), from its early focal mucin deposition in the reticular dermis to its late findings of interstitial mucin deposition, dermal fibrosis and fibroblast proliferation. Her father reported having had similar lesions when he was a child, which completely disappeared during adolescence. This case, and a re-evaluation of the literature, suggests that cases of cutaneous mucinosis of infancy that are not hamartomatous conditions such as mucinous naevi are in fact the infantile presentation of lichen myxoedematosus (papular mucinosis) and, in addition to other cases in the literature, suggests a genetic and familial factor in lichen myxoedematosus (papular mucinosis).

Updated classification of papular mucinosis, lichen myxedematosus, and scleromyxedema.

Lichen myxedematosus (LM) is an idiopathic cutaneous mucinosis; its classification dates back to 1953, when Montgomery and Underwood distinguished 4 types of LM: a generalized lichenoid eruption, later called scleromyxedema, a discrete papular form, a localized or generalized lichenoid plaque form, and an urticarial plaque form. In the literature, the terms LM, papular mucinosis, and scleromyxedema have been often used indiscriminately as synonyms, but most reported cases of LM or papular mucinosis without indication of the subtype appear in fact to be cases of scleromyxedema. On the basis of personal experience, the anatomoclinical manifestations of published cases of LM, papular mucinosis, and scleromyxedema are reviewed to distinguish clearly between a generalized form with systemic, even lethal, manifestations and a localized form, which does not run a disabling course. LM includes two clinicopathologic subsets: a generalized papular and sclerodermoid form (also called scleromyxedema) and a localized papular form. Diagnosis of scleromyxedema should fulfill the following criteria: (1) generalized papular and sclerodermoid eruption; (2) mucin deposition, fibroblast proliferation, and fibrosis; (3) monoclonal gammopathy; and (4) the absence of thyroid disease. The criteria for localized LM are as follows: (1) papular or nodular/plaque eruption; (2) mucin deposition with variable fibroblast proliferation; and (3) the absence of both monoclonal gammopathy and thyroid disease. The localized form is subdivided into 5 subtypes: (1) a discrete papular form involving any site; (2) acral persistent papular mucinosis involving only the extensor surface of the hands and wrists; (3) self-healing papular mucinosis, of a juvenile and an adult type; (4) papular mucinosis of infancy, a pediatric variant of the discrete form or of acral persistent papular mucinosis; and (5) nodular form. A third group of atypical or intermediate forms, not meeting the criteria for either scleromyxedema or the localized form, includes cases of (1) scleromyxedema without monoclonal gammopathy, (2) localized forms with monoclonal gammopathy and/or systemic symptoms, (3) localized forms with mixed features of the 5 subtypes, and (4) not well-specified cases.

Discrete papular mucinosis-a rare subtype of lichen myxoedematosus.

Lichen myxoedematosus is an uncommon and distinct disease entity characterized by cutaneous mucin deposition which, depending on the distribution and overall skin involvement, can be classified into several subtypes. We now describe the case of a discrete papular type of lichen myxoedematosus in a patient without any conspicious laboratory findings including normal thyroid function and the absence of any abnormal immunglobulins.

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)

Mucinosis cutáneas / Cutaneous mucinosis

La mucina está constituida por ácido hialurónico, que es el principal mucopolisacárido ácido de la dermis en condiciones fisiológicas. Su apariencia es la de un gel transparent y viscoso, y a la microscopia óptica se evidencia con tinciones especiales (azul de alcian a pH 2,5, hierro coloidal y mucicarmín). El aumento de depósito de mucina dérmica se denomina mucinosis cutánea, y si este depósito es el principal elemento observado en la histopatología constituye una mucinosis primaria o idiopática, con características clínicas e histopatológicas propias. Es importante diferenciarlas de aquellas dermatosis en que secundariamente se observa un depósito de mucina (dermatofibroma, granuloma anular, tumores cutáneos, etc.). Las mucinosis cutáneas primarias pueden clasificarse en focales o difusas, según sea la distribución de la mucina, y según sean sus características histopatológicas pueden constituir entidades clínicas bien diferenciadas

[Cutaneous mucinoses]. / Les mucinoses cutanées.

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.