Mucinosis nodular de la mama / Nodular mucinosis of the breast

La mucinosis nodular de la mama es una rara entidad de histogénesis incierta y con comportamiento benigno. Suele presentarse como una tumoración asintomática en torno a pezón o areola y localizada en dermis o tejido celular subcutáneo. Presentamos un caso de mucinosis nodular típica. Histológicamente se define por estar constituida por lagos irregulares de mucina negativos con PAS pero positivos con hierro coloidal y azul alcián. La mucina se acompaña de estroma fibroso con un escaso componente de células fusiformes. Algunos autores sugieren un posible origen miofibroblástico. No obstante no se han realizado estudios moleculares de la mucinosis nodular. El diagnóstico diferencial se ha de hacer principalmente con lesiones mamarias benignas y malignas de estroma mucinoso. En la literatura se describen 13 casos de mucinosis nodular

Nodular mucinosis of the breast is a rare entity of uncertain histogenesis and with benign behavior. It usually presents as an asymptomatic tumor around the nipple or areola and located in the dermis or subcutaneous cellular tissue. We present a case of typical nodular mucinosis. Histologically it is defined as being composed of irregular lakes of mucin negative with PAS but positive with colloidal iron and alcian blue. Mucin is accompanied by fibrous stroma with a scarce component of fusiform cells. Some authors suggest a possible myofibroblastic origin. However, molecular studies of nodular mucinosis have not been performed. The differential diagnosis must be made mainly with benign and malignant mammary lesions of mucinous stroma. In the literature, 13 cases of nodular mucinosis are described

Alopecia e hipoestesia en linfocitosis de células T foliculotrópica / Alopecia and Hypoesthesia in Folliculotropic T-Cell Lymphocytosis

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Clinicopathological study of oral focal mucinosis: a retrospective case series

Background: Oral focal mucinosis (OFM) is a rare soft tissue lesion of unknown etiology that exhibits tumor-like growth. It is considered the oral counterpart of cutaneous focal mucinosis or cutaneous myxoid cyst. This is a retrospective study of oral OFM diagnosed over a period of 42 years at an oral pathology service. Material and Methods: Clinical, histopathological and immunohistochemical data were analyzed. Alcian blue staining and S-100 immunohistochemistry were performed. Results: Eleven cases were retrieved (4:1 female-to-male ratio). The mean age was 44 years. The gingiva was the most affected site. The main clinical presentation was sessile or pedunculated lesions of fibrous or hyperplasic appearance, most of them asymptomatic. Positive Alcian blue staining and absence of S-100 protein were observed in all specimens, which supported the histological diagnosis of OFM. Surgical excision was the treatment of choice. Conclusions: Although rare, this study supports the inclusion of OFM in the differential diagnosis of intraoral myxoid lesions

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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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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)

Mucinosis y vasculitis a nivel uterino asociadas a lupus eritematoso / Uterine mucinosis and vasculitis associated with lupus erythematous

Uterine mucinosis and vasculitis associated with lupus erythematous(AU)

The association of cutaneous papulonodular mucinosis with lupus erythematosus has long been recognized. Massive cutaneous deposits of mucin and periorbital mucinosis have also been reported in patients with lupus. Recently, a report was published of two cases of mucin deposits in the myometrium of patients with lupus, denominated "myxoidosis" by the authors. A case is presented of vasculitis and diffuse, predominately myometrial, mucinosis, in the uterus of a patient with lupus erythematosus. The term "mucinosis" and not "myxoidosis" is recommended, even when referring to the uterus, in order to unify the terminology of pathological findings that have similar histological and histochemical features to the cutaneous lesions found in lupus erythematosus(AU)

Mucinosis eritematosa reticular: respuesta parcial al tratamiento con tacrolimus tópico / Reticular erythematous mucinosis: partial response to treatment with topical tacrolimus

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Manifestaciones cutáneas del síndrome tóxico por aceite de colza adulterado / Skin Manifestations of Toxic Syndrome Due to Denatured Rapeseed Oil

En este artículo se efectuó una amplia descripción clínico-patológica y cronológica de las manifestaciones cutáneas del síndrome tóxico por aceite de colza adulterado o síndrome tóxico por aceite. Esta nueva enfermedad se produjo en España en 1981, debida a la ingesta de aceite de colza destinado a uso industrial, que había sido coloreado con anilinas y posteriormente decolorado de forma fraudulenta y vendido como aceite de oliva. En total se produjeron unos 20.000 casos y unos 400 fallecimientos. Existía un predominio por el sexo femenino, sobre todo en la fase tardía. En la fase aguda las manifestaciones cutáneas predominantes fueron exantemas toxoalérgicos, con un patrón dermatopatológico de erupción alérgica urticariforme. Alrededor de un 25% de los casos desarrolló posteriormente un aspecto edematoso de la piel, con trastornos de la pigmentación, que demostró estar relacionado con mucinosis dérmica. Finalmente se produjo un cuadro esclerodermiforme peculiar, que tendió a mejorar de forma espontánea. La presencia constante de mastocitos en todas las biopsias y el desarrollo de mastocitosis en varios pacientes sugirieron un papel importante del mastocito en la patogenia del cuadro, que luego ha sido ratificada en otros procesos esclerodermiformes. En 1989 apareció en EE.UU. el síndrome mialgia-eosinofilia, relacionado con sustancias tóxicas presentes en suplementos alimentarios de triptófano y que compartía muchos aspectos con el síndrome tóxico por aceite. Esto corroboró la existencia de mucinosis y cuadros esclerodermiformes de origen tóxico (AU)

This article offered an extensive description of the clinical and pathological features and time-course of the skin manifestations of toxic syndrome caused by denatured rapeseed oil, also known as toxic oil syndrome. This new condition occurred in Spain in 1981 and was due to the ingestion of rapeseed oil intended for industrial use that had been denatured with anilines and subsequently refined and sold fraudulently as olive oil. In total, 20 000 cases and 400 deaths were reported. The disease affected mainly women, particularly in the late stages. In the acute phase, the predominant skin manifestations were toxic-allergic rashes reminiscent of allergic urticaria in the dermatopathologic study. In approximately 25% of cases, the patients’ skin subsequently took on an edematous appearance, with pigmentary abnormalities shown to be related to cutaneous mucinosis. Finally, a characteristic sclerodermatous condition would develop that tended to improve spontaneously. The constant presence of mast cells in all biopsies and the development of mastocytosis in several patients pointed to an important role for these cells in the pathogenesis of the condition. This was subsequently confirmed in other sclerodermatous processes. In 1989, eosinophilia-myalgia syndrome caused by toxins present in tryptophan food supplements was reported in the United States. This syndrome resembled toxic oil syndrome in many ways and demonstrated that mucinosis and toxic sclerodermatous processes do exist (AU)

Lesiones ampollosas como forma de presentación de una enfermedad de injerto contra huésped crónica / Chronic graft-versus-host disease presenting as bullous lesions

La enfermedad de injerto contra huésped (EICH) continúa siendo la primera causa de morbilidad y mortalidad en los enfermos con trasplante de médula ósea, de modo que un tratamiento precoz es importante para reducir la gravedad y las consecuencias de esta complicación. Las lesiones cutáneas son en muchas ocasiones la forma de presentación de una EICH y anuncian la afectación visceral. Presentamos el caso de una paciente de 45 años con mieloma múltiple a la que se realizó primero un trasplante autólogo y posteriormente un trasplante alogénico de médula ósea con precursores hematopoyéticos. Desarrolló lesiones ampollosas con eliminación de líquido en el abdomen y los miembros inferiores. Se realizó una biopsia que fue compatible con una EICH y se aumentó el tratamiento inmunodepresor. La paciente posteriormente presentó lesiones liquenoides orales, síndrome seco y las lesiones ampollosas que evolucionaron a úlceras dolorosas, que cicatrizaron con un intenso aspecto esclerodermiforme e importante hiperpigmentación. Las lesiones ampollosas son una forma de presentación excepcional en la EICH crónica, y en estos casos la sospecha inicial del diagnóstico puede ser difícil, sobre todo cuando se presentan de forma aislada sin afectación de órganos internos (AU)

Graft-vs-host disease is still the leading cause of morbidity and mortality in patients undergoing bone marrow transplantation. It is important to start treatment early to reduce the severity and consequences of this complication. Cutaneous lesions are often the presenting compliant of graft-vs-host disease and presage visceral involvement. We present the case of a 45-year-old woman with multiple myeloma who underwent autologous and subsequently allogeneic bone marrow transplantation with hematopoietic precursors. She developed bullous lesions with fluid elimination on the abdomen and legs. Biopsy findings were compatible with graft-vs-host disease and immunosuppressive therapy was increased. She subsequently presented oral lichenoid lesions and sicca syndrome. The bullous lesions progressed to painful ulcers that healed leaving highly sclerodermatous skin with substantial hyperpigmentation. Bullous lesions are a rare form of presentation of chronic graft-vs-host disease. In such cases, the diagnosis may not be suspected initially, particularly when the lesions are isolated and internal organs are not involved (AU)

Pápulas en el dorso de los dedos de las manos / Papules on the Dorsum of the Fingers

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

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Tacrolimus en enfermedades diferentes a la dermatitis atópica / Tacrolimus in diseases other than atopic dermatitis

El tacrolimus tópico es un inmunosupresor que actúa mediante la inhibición de la calcineurina y, por tanto, de las células T, lo cual produce una disminución en la producción de interleucinas, del factor estimulador de colonias granulocíticas, del interferón alfa y del factor de necrosis tumoral. A pesar de que el uso del tacrolimus tópico sólo está indicado para el tratamiento de la dermatitis atópica moderada o severa, su efecto inmunosupresor y su menor número de efectos secundarios respecto a los corticoides tópicos, ha provocado el aumento de su utilización en otro tipo de enfermedades inflamatorias dermatológicas. La finalidad de este artículo es revisar el uso del tacrolimus en este grupo de enfermedades diferentes a la dermatitis atópica y cuyo uso no está autorizado en la ficha técnica del fármaco (AU)

Topical tacrolimus is an immunosuppressant that acts through the inhibition of calcineurin and thus of the T cells. This causes a decrease in the production of interleukins, the granulocyte colony stimulating factor, alpha interferon and tumor necrosis factor. Although the use of topical tacrolimus is only indicated for the treatment of moderate or severe atopic dermatitis, its immunosuppressant effect and fewer side effects regarding topical corticosteroids have lead to the increase of its use in other types of inflammatory skin diseases. The purpose of this article is to review the use of tacrolimus in this group of diseases other than atopic dermatitis, this use not being authorized within the data sheet of the drug (AU)

Mucinose papular: manifestação incomum de dermatomiosite amiopática / Papular mucinosis: uncommon presentation of amyopathic dermatomyositis

Os autores relatam um caso de dermatomiosite amiopática com manifestação cutânea incomum do tipo mucinose papular. O diagnóstico de dermatomiositefoi baseado na clínica e no exame histopatológico cutâneo e a forma amiopática foi definida pela ausência de fraqueza muscular, exameslaboratoriais normais e pela ausência de sinais de miosite na eletroneuromiografia e na biópsia de músculo. A dermatomiosite amiopática representaum subtipo raro da doença. Um diagnóstico precoce depende do reconhecimento dos achados cutâneos clássicos e das manifestações raras, entre asquais a mucinose cutânea. Um correto diagnóstico do paciente colabora para o seu adequado manejo

The authors report a case of amyopathic dermatomyositis with uncommon papular mucinosis. The dermatomyositis diagnosis was based on clinicalfindings and skin biopsy and amyophathic presentation was defined by no clinical evidence of muscle weakness, normal laboratory results, electromyogramand muscle biopsy. Amyopathic dermatomyositis is a rare subtype of the disease. A prompt diagnosis of dermatomyositis depends on recognitionof classic cutaneous findings and rare presentations such as papular mucinosis. An accurate patient diagnosis contributes for the correct treatment

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

Mucinosis papulosa acral persistente / Acral persistent papular mucinosis

Presentamos un caso de mucinosis papulosa acral persistente (MPAP). Se trata de una mujer de 69 años que presenta pápulas del mismo color de la piel de 2-4mm en el dorso de las manos y las muñecas. En el estudio histopatológico aparece un depósito de mucina focal en la dermis papilar y reticular superior. La MPAP es un tipo de mucinosis que presenta un curso benigno y persistente, sin asociación a alteraciones sistémicas. Existe controversia sobre si se trata de una nueva entidad o un subtipo de la forma discreta papular de liquen mixedematoso

We present a case of acral papular persistent mucinosis(APPM). A 69 years old woman had 2-4mm flesh-colored papules on the back of her hands and extensor wrist. Examination of biopsy specimen showed focal mucin deposition in papillary and upper reticular dermis. APPM is a mucinosis subtype which has a benign and persistent course, without systemic involvement. Controversy exist as to wether this subtype should be considered a new entity o as a variant of the discrete papular form of lichen myxedematosus

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

Mucinosis eritematosa reticular / Reticular erythematous mucinosis

La mucinocis eritematosa reticular (MER) es una rara entidad que se caracteriza por placas eritematosas infiltradas en zonas de exposición de tórax y espalda. Se ha relacionado con exposición solar, alteraciones hormonales, procesos reumatológicos y cáncer de mama. Una mujer de 28 años presentaba una placa eritematosa infiltrada, de 4 años de evolución en la mama izquierda, que con el paso del tiempo fue extendiéndose por ambas mamas. El estudio histopatológico demostró la existencia de un infiltrado linfocitario perivascular y perianexial, que se acompañaba de evidentes depósitos de mucina en dermis superior y media. Se realizó tratamiento con colchicina e hidroxicloroquina, con regresión parcial de las lesiones (AU)