Liquen mixedematoso atípico asociado a mieloma múltiple / Atypical lichen myxedematosus associated to multiple myeloma

Resumen El liquen mixedematoso (LM) representa un grupo de enfermedades cutáneas raras, el cual se encuentra dentro de las mucinosis crónicas. Anteriormente descrita como escleromixedema localizado, sin embargo, a diferencia de éste, por lo general no tiene compromiso sistémico. Dentro de los subtipos, se encuentra el LM atípico, el cual es infrecuente y hay pocos casos reportados asociados a mieloma múltiple (MM). Se presenta el caso de un paciente masculino con MM positivo para cadenas lambda, con cuadro clínico de inicio agudo, en quien se realizó diagnóstico de LM atípico; recibió manejo con corticoide tópico con mejoría de las lesiones al mes de tratamiento.

Abstract Lichen myxedematous (LM) represents a group of rare skin diseases, which is found within the chronic mucinoses. Previously described as localized scleromyxedema, however, unlike localized scleromyxedema, it usually does not have systemic involvement. Among the subtypes, there is atypical LM, which is infrequent and there are few reported cases associated with multiple myeloma (MM). We present the case of a male patient with MM positive for lambda chains, with acute onset clinical picture, who was diagnosed with atypical LM; he received management with topical corticosteroid with improvement of the lesions after one month of treatment.

Escleromixedema. Reporte de un caso / Scleromyxedema. Case report

Resumen Se presenta una paciente femenina con erupción papulosa generalizada que compromete cara, tronco y cuatro miembros. En el examen físico se visualizaengrosamientoy oscurecimiento de la piel. Se realiza el estudio integral y el correspondiente diagnóstico diferencial.El estudio histopatológico cutáneo exhibió un incremento excesivo de mucina intersticial, actividad fibroblástica y engrosamiento de los haces de colágeno. Se arriba al diagnóstico de escleromixedema debido a las manifestaciones cutáneas características. Se constata compromiso extracutáneo en ausencia de gammapatía monoclonal. Se indica prednisona, talidomida ehidroxicloroquina con excelente evolución.

Abstract A female patient presents with a generalized papular rash involving face, trunk, and four limbs. The skin is thickened and darkened, forming yellowish erythematous plaques that are linearly arranged papules. It is assumed as a generalized sclerodermiform syndrome and a comprehensive study and corresponding differential diagnosis is performed. The histopathological study of the skin showed an excessive increase of interstitial mucin, fibroblast activity and thickening of collagen bundles. The characteristic clinical expression and the histopathological study added to the extra cutaneous involvement lead to the diagnosis of scleromyxedema. There was no evidence of monoclonal gammopathy. Prednisone, thalidomide and hydroxychloroquine are indicated with excellent evolution.

Papulonodular mucinosis in the primary onset of juvenile systemic lupus erythematosus: An unusual association / Mucinosis papulonodular en el inicio de lupus eritematoso sistêmico juvenil: asociación inusual

ABSTRACT Cutaneous mucinosis is a group of conditions characterized by the abnormal deposition of mucin in the skin. They can be primary, which in turn can be inflammatory-degenerative, and hamartomatous-neoplastic; or secondary. Papulonodular mucinosis is part of the group of dermal inflammatory-degenerative primary mucinosis. Its association with autoimmune connective tissue diseases has been described, especially with systemic lupus erythematosus, but it is considered an unusual manifestation of this disease. The clinical case is presented of an 11 year-old girl who, at the onset of systemic lupus erythematosus, presented with skin lesions for which the histopathological diagnosis corresponded to mucinosis.

RESUMEN Las mucinosis cutáneas son un grupo de condiciones caracterizadas por el depósito anormal de mucina en la piel. Pueden ser primarias, que a su vez pueden ser inflamatorias-degenerativas (dérmicas o foliculares) y hamartomatosas-neoplásicas; o secundarias. La mucinosis papulonodular forma parte de las mucinosis primarias inflamatorias-degenerativas dérmicas. Se ha descrito su asociación con enfermedades autoinmunes del tejido conectivo, especialmente con el lupus eritematoso sistémico, pero se considera una manifestación inusual de esta enfermedad. Se presenta el caso clínico de una niña de 11 años, quien al inicio del lupus eritematoso sistémico presentaba lesiones en la piel cuyo diagnóstico histopatológico correspondió a mucinosis.

Mucinosis linfedematosa asociada a obesidad. reporte de un caso y revisión de la literatura / Lymphedematous mucinosis associated with obesity. a case report and review of the literature

RESUMEN: La mucinosis linfedematosa asociada a obesidad es una dermatosis de reciente reconocimiento que corresponde al grupo de mucinosis cutáneas focales. Presenta un curso clínico caracterizado por la aparición progresiva de lesiones asintomáticas en las piernas de pacientes que concomitantemente cursan con obesidad y linfedema secundario en miembros inferiores.Su diagnóstico implica reconocer otros tipos de mucinosis cutáneas y realizar el diagnóstico diferencial respectivo, de acuerdo con el contexto clínico en el que se presenten las lesiones. Una vez establecido el diagnóstico, su manejo terapéutico debe ir dirigido a lograr una disminución significativa en el peso.

ABSTRACT: Lymphedematous mucinosis associated with obesity is a recently recognized dermatosis that corresponds to the group of focal cutaneous mucinosis. It presents a characteristic clinical course consisting of the progressive and asymptomatic appearance of lesions in the legs of adults who concomitantly have obesity and lymphedema secondary in the lower limbs, as in the clinical case that we will discuss. After having made the differential diagnosis with other possible types of cutaneous mucinosis, according to the clinical context in which the lesions occur, therapeutic management should be aimed at achieving a significant decrease in weight.

Oral focal mucinosis of the hard palate and gingiva

Oral focal mucinosis (OFM) is an uncommon, asymptomatic, submucosal, slow-growing nodule representing a counterpart of the cutaneous focal mucinosis (CFM). OFM has a female predilection with the highest prevalence in the fifth decade of life. About 68% of OFMs occur in the gingiva and 14% in the palate. We present the case of a 41-year-old woman presenting a progressively growing mass on the palate, since the last 8 months. The diagnostic workup led to the diagnosis of an unusual OFM with the clinical presentation involving the gingiva and hard palate. This case report discusses the clinical and histopathological differential diagnosis.

Congenital Melanocytic Nevus with Secondary Cutaneous Mucinosis in a Child / 대한피부과학회지

No abstract available.

Mucinosis. Presentación de caso / Mucinosis. Presentation of case

Mucinosis (REM síndrome), es una rara enfermedad cutánea, descrita hace más de 30 años por Steigleder, afectando predominantemente a mujeres de edad avanzada. Clínicamente presenta áreas con eritema persistente, pápulas que pueden confluir, formando placas con escamas. La etiología aún no está bien esclarecida, pero diversos factores como la luz ultravioleta, trastornos inmunológicos, infecciones virales, han sido relacionados o asociados con la inducción de la misma. Se presenta un reporte de caso de un paciente masculino de 20 años, con antecedentes de haber padecido de Condilomas Acuminados, además presentó otras lesiones cutáneas caracterizadas por placas alopécicas, con discreta infiltración en número de 10 en cuero cabelludo y lesiones en placas, infiltradas, de 3 a 5 centímetros de diámetro en número de 2, de bordes precisos en tercio inferior central de la espalda. Se realizó biopsia de piel, donde se corrobora el diagnóstico de Mucinosis, posteriormente el paciente ingresó en el servicio de Nefrología en el hospital de Cárdenas con diagnóstico de Insuficiencia Renal Crónica (AU).

Mucinosis (REMsyndrome), he is a rare cutaneous, described disease he does over 30 years for Steigleder, affecting predominantly women late in years. Clinically he presents areas with persistent erythema, pápulas that they can converge, forming plates with scales. The etiology not yet is very illustrious, but various factors like the ultraviolet light, immunogenic upsets, viral infections, they have been related or associated with the induction of the same. He encounters a report of case of a masculine patient of 20 years, with background to have suffered from Condylomas Acuminados, besides he presented another cutaneous injuries characterized by plates alopécicas, with discreet infiltration in number of 10 in scalp and injuries in plates, spies, of 3 a 5 cms of diameter in number of 2, of precise borders in inferior central third part of the back. The patient accomplished biopsy of skin himself, where Mucinosis's diagnosis is corroborated, at a later time he entered in Nefrología's service at Cárdenas's hospital with diagnosis of renal chronic Insuficiencia (AU).

Mucinose folicular primária ­ relato de caso / Primary follicular mucinosis ­ case report

De etiologia desconhecida, a mucinose folicular primária ou idiopática se caracteriza clinicamente como afecção inflamatória, com placas mais ou menos infiltradas e descamativas, com ou sem perda de pelos. Na sua forma secundária, costuma apresentar lesões mais numerosas e difusas, com morfologia variá- vel, desde placas até nódulos ulcerados. É representada por depósitos localizados ou difusos de mucina na pele ou nos folículos pilosos. Objetivo: Apresentar um caso incomum de mucinose folicular primária, de importância da diferenciação com a forma secundária de mucinose folicular, discutir os aspectos clínicos e histopatológicos utilizados no diagnóstico, suas características e classificações, bem como as possíveis escolhas terapêuticas.(AU)

Of unknown etiology, acute follicular mucinosis is clinically characterized as an inflammatory disease coursing with more or less infiltrated and scaly plaques, with or without hair loss. In the chronic form, it usually has more numerous and diffuse lesions with variable morphology, from plaques to ulcerated nodules. It is characterized by localized or diffuse deposits of mucin in the skin or hair follicles. Objective: To present an unusual case of primary follicular mucinosis, the importance of differentiation from the secondary form of follicular mucinosis, to discuss the clinical and histopathological aspects used in the diagnosis, its characteristics and classifications as well as the possible therapeutic choices.(AU)

Nodular Cutaneous Mucinosis as an Initial Presentation of Systemic Lupus Erythematosus / 대한피부과학회지

It is well recognized that the deposition of mucin occurs in areas of eruption in patients with lupus erythematosus. However, nodular cutaneous mucinosis is regarded as a rare distinctive cutaneous mucinosis in which the mucin deposition occurs in areas other than the sites of eruption in patients with systemic lupus erythematosus and manifests as a clinically specific lesion. Although several cases have been described in the literature, there have been only two reported cases in Korean literature. Thus, we report an interesting case of nodular cutaneous mucinosis that occurred in a young man. A 16-year-old man visited our clinic presenting with multiple soft subcutaneous nodules on his back for 6 months. He denied any other symptoms. Histological examination showed diffuse mucin deposition throughout the superficial and mid-reticular dermis. The abnormal laboratory values were as follows: a positive antinuclear antibody, increased anti-dsDNA, and reduced WBC count and C3 levels. He was diagnosed with nodular cutaneous mucinosis with systemic lupus erythematosus and treated with oral hydroxychloroquine and methylprednisolone. Our report is of interest owing to the rarity of developing nodular cutaneous mucinosis as an initial presentation of systemic lupus erythematosus.

Mucinosis folicular primaria: presentación de un caso clínico tratado con tacrolimus unguënto al 0.1% / Primary follicular mucinosis: a case report treated with tacrolimus ointment 0.1 %

La mucinosis folicular se caracteriza por depósito anómalo de mucina, en los folículos pilosos y glándulas sebáceas. Puede corresponder a un trastorno idiopático benigno y auto-limitado, especialmente en niños o representar un cuadro secundario a un trastorno mielo-proliferativo, generalmente un linfoma cutáneo-T. Presentamos el caso de una niña de diez años de edad, con varias placas eritemato-blanquecinas circulares, ocasionalmente pruriginosas, en cara, cuello y tronco, además de alopecia difusa de cinco meses de evolución. No hay ataque al estado general. El estudio histopatológico reportó un cuadro compatible con mucinosis folicular.

Follicular mucinosis is characterized by mucin deposits within the hair follicles and sebaceous glands. It can occur as a primary idiopathic benign disorder; it can; or it can arise as a secondary symptom of a malignant disorder, most commonly mycosis fungoide. Youth and localization in a single area used to be regarded as indicative of the benign nature of follicular mucinosis. We report the case of a ten years old girl who presented with a five months history of a multiple, slightly pruritic, erythematous patches on her face, neck and chest. Different local treatments were ineffective. Overall, she was healthy. Histopathological examination of the lesion showed the typical histological picture of follicular mucinosis.

A Case of Self-healing Juvenile Cutaneous Mucinosis / 대한피부과학회지

Self-healing juvenile cutaneous mucinosis (SHJCM) is a rare disorder of unknown origin that affects healthy children. It is characterized by multiplication of transient papules and nodules on the head and periarticular area. Histopathologically, lesions show mucin deposition in the dermis or subcutis. A 9-year-old male patient presented with multiple skin-colored papules and nodules on the face and both hands. These papules and nodules had appeared over the preceding months and had been increasing in number. He was otherwise healthy and had no underlying systemic disorders. Skin biopsy in the right thenar nodule revealed deposition of amorphous material stained positively with Alcian blue (pH 2.5) within the dermis and subcutis. Spontaneous resolution occurred over several months without sequelae.

A Case of Cutaneous Focal Mucinosis on the Fingertip / 대한피부과학회지

No abstract available.

Follicular mucinosis: an important differential diagnosis of leprosy in an endemic area

Primary follicular mucinosis is a rare dermatosis characterized by the accumulation of mucin in the follicular epithelium and sebaceous glands. Clinically, it is characterized by the presence of papules or well-circumscribed and infiltrated plaques. In this paper, we report the case of a female patient, seven years old, evolving for three months with an asymptomatic, erythematous and infiltrated plaque located in the chin region. The research of thermal, pain and tactile sensitivity was inconclusive. Histological findings confirmed the diagnosis of follicular mucinosis. There was regression of the lesion with the use of medium potency topical corticosteroids for 20 days. The pathogenesis of follicular mucinosis remains unknown, being in some cases associated with lymphoproliferative disorders. In endemic areas of leprosy, isolated and infiltrated follicular mucinosis lesions should be further differentiated from leprosy.

Mucinosis folicular primaria: simulando una tiña corporal / Primary follicular mucinosis: simulating a tinea corporis

La mucinosis folicular se caracteriza por depósito anómalo de mucina en los folículos pilosos y glándulas sebáceas. Puede corresponder a un trastorno idiopático benigno y autolimitado especialmente en niños, o representar un cuadro secundario a un trastorno mieloproliferativo, generalmente un linfoma cutáneo T. Presentamos el caso de una niña de 10 años de edad con varias placas eritematoblanquecinas circulares ocasionalmente pruriginosas en cara, cuello y tronco, además de alopecia difusa de 5 meses de evolución. No hay ataque al estado general. El estudio histopatológico reportó un cuadro compatible con mucinosis folicular.

Follicular mucinosis is characterized by mucin deposits within the hair follicles and sebaceous glands. It can occur as a primary idiopathic benign disorder; it can; or it can arise as a secondary symptom of a malignant disorder, most commonly mycosis fungoides. Youth and localization in a single area used to be regarded as indicative of the benign nature of follicular mucinosis. We report the case of a 10 -year old girl who presented with a 5- months history of a multiple, slightly pruritic, erythematous patches on her face, neck and chest. Different local treatments were ineffective. Overall, she was healthy. Histopathological examination of the lesion showed the typical histological picture of follicular mucinosis.

Alopecia mucinosa: relato de um caso com revisão de literatura e proposta dos achados dermatocópicos / Mucinous alopecia (follicular mucinosis): case report, review of the literature and dermatoscopic consideration

Doença rara, descrita por Pinkus em 1957, a alopecia mucinosa pertence às alopecias cicatriciais. É assim denominada pelo acúmulo de mucina e linfócitos na porção infundibular do aparelho pilossebáceo, promovendo destruição total e irreversível do local afetado. De etiologia desconhecida, clinicamente se manifesta em placas, geralmente normocrômicas, bem demarcadas, composta por pápulas com evidente proeminência folicular. A ausência de sintomas é a regra. Sua classificação como micose fungoide incipiente ainda não é consenso. Com pico bimodal de incidência costuma manifestar-se em crianças ou idosos, através de diferentes quadros clínicos, prognósticos e associações. Não existem, até o presente momento, estudos de dermatoscopia nessa rara entidade. Descrevemos uma apresentação atípica da doença e propomos seus achados dermatoscópicos.

Follicular mucinosis - Case report

Follicular mucinosis, also known as alopecia mucinosa, is a cutaneous mucinosis histologically characterized by accumulation of dermal type mucin in the pilosebaceous follicle and sebaceous glands. It presents in two forms, a primary or idiopathic form and a secondary form associated with various benign or malignant processes. Among the malignant processes, the main association is with mycosis fungoides. The frequent overlap of clinical, histopathological, immunohistochemical and molecular biology characteristics makes the correct classification of these conditions difficult, therefore a long follow-up of all cases is recommended. We report the case of an adolescent with disseminated lesions and discuss the difficulty of early identification of secondary follicular mucinosis associated with cutaneous lymphoma.

A Case of Cutaneous Horn-Like Cutaneous Focal Mucinosis / 대한피부과학회지

No abstract available.

Generalized papular and sclerodermoid eruption: Scleromyxedema.

Scleromyxedema (SM) is a rare chronic progressive and highly intractable cutaneous disease with unknown etiology, affecting both genders equally between 30 and 50 years. The disease is characterized with mucin deposits in the skin and/or other organs. In fact it is a clinicopathological subset of lichen myxedematosus (LM) according to a new classification. Sclerodermiform plaques and lichenoid papules are characteristic cutaneous lesions. An elevation of IgG λ (lambda) chain exists in most cases and extracutaneous involvement occurring with variable systemic findings is also detected. Generalized form is quite difficult to treat and may even be fatal. Herein, we present a male patient with typical features of generalized papular and sclerodermoid LM variety and with benign outcome by isotretinoin.

Mucinosis folicular: a propósito de dos casos / Follicular mucinosis: with regard to two cases

Las lesiones de Mucinosis folicular suelen localizarse en cabeza y más raramente extenderse a cuello, tronco y extremidades. Los hallazgos histológicos se caracterizan por la degeneración reticular del folículo pilosebáceo y el depósito de glicosaminoglicanos (mucina). Se presenta un caso de Mucinosis folicular generalizada, en una paciente de sexo femenino de 35 años de edad. Al examen dermatológico se visualizaban múltiples pápulas eritematosas, con predominio en tórax región posterior y cuero cabelludo, acompañadas de placas localizadas en pliegues y alopecia difusa. El segundo caso es de una paciente de sexo femenino de 26 años de edad. A la inspección presentaba placa eritematosa y nódulos dolorosos. Se destaca la necesidad de seguimiento clínico e histológico, ante la posibilidad de transformación a linfoma cutáneo.

The injuries of Follicular mucinosis usually are located in head or more rarely to become general to neck, trunk and extremities. The histological findings are characterized by the reticular degeneration of the pilosebaceous follicle and the deposit of glicosaminoglicans (mucin). A case of generalized Follicular mucinosis in a patient of feminine sex of 35 years of age appears. The dermatological examination revealed manifold papules with predominance in later thorax erythematous region and hairy leather, accompanied by plates that were located in you fold and diffuse alopecia. The second case is a patient of feminine sex of 26 years of age. The dermatological examination erythematous plate with painful nodules to the palpation was observed. The necessity of clinical and histological pursuit stands out before the cutaneous possibility of transformation to lymphoma.

Oral focal mucinosis: report of two cases / Mucinose oral focal: relato de dois casos

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.