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.

Clinical, dermoscopic, and histologic aspects of two cases of cutaneous focal mucinosis

Abstract: Cutaneous mucinoses are a complex and diverse group of connective tissue disorders characterized by the accumulation of mucin and/or glycosaminoglycan in the skin and adnexa. Cutaneous focal mucinosis appears as a solitary, asymptomatic, skin-colored to white papule, nodule, or plaque located anywhere on the body or in the oral cavity. It presents mainly in adults and is characterized on histopathology by mucin throughout the upper and mid dermis. We describe the dermoscopy of two cases of cutaneous focal mucinosis. Both lesions presented a nonspecific homogenous whitish pattern; the first case also exhibited a sharply demarcated yellow border.

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.

Mucinous Nevus

Mucinous nevus is an uncommon entity classified as either a cutaneous mucinosis or a connective tissue nevus. The condition presents as grouped papules and coalescent plaques growing in a unilateral or zosteriform manner. The key histopathological feature is a band-like deposition of mucin in the superficial dermis. A 34-year-old male presented with grouped gray-brown papules and confluent plaques exhibiting a zosteriform distribution on the right side of the lower back. The lesions had commenced in childhood. Histological examination revealed mucin deposition in the papillary dermis. Thus, we diagnosed a mucinous nevus. To date, only a few reports of such nevi have been reported in the literature. Therefore we report a rare case of mucinous nevus.

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)

A Rare Case of Nodular Mucinosis of the Breast

No abstract available.

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.

Pretibial mucinosis in an euthyroid patient

Abstract A case of exuberant pretibial mucinosis in a patient with normal thyroid function is reported. A review of literature on possible etiologies other than thyroid disease for the accumulation of mucin in the pretibial area is presented. In the patient described, it is possible that vascular insufficiency is involved. However, this is not the only factor responsible for the accumulation of mucin, since there are still unidentified causes and many patients with vascular diseases do not develop similar injuries.

A Case of Cutaneous Focal Mucinosis on the Fingertip / 대한피부과학회지

No abstract available.

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.

Periadnexal Mucin as an Additional Histopathologic Feature of Chronic Eczematous Dermatitis

BACKGROUND: Cutaneous mucinoses are a heterogeneous group of disorders characterized by an abnormal amount of mucin in the skin. However, the pathomechanism of an excessive mucin deposition in the skin is still unknown. Eczematous dermatitis is sub-classified histologically into acute, subacute, and chronic variants. The characteristic histopathologic findings for chronic eczema are variable. However, periadnexal mucin deposition is not known as a feature of chronic eczema. OBJECTIVE: To evaluate the presence of periadnexal mucin deposition in chronic eczematous dermatitis. METHODS: We analyzed the skin biopsy specimens from 36 patients who were pathologically diagnosed with chronic eczematous dermatitis. Alcian blue, colloidal iron, and periodic acid-Schiff stains were used to evaluate the mucin deposition in histologic sections. Two dermatologists and two dermatopathologists evaluated the degree of mucin deposition using a 4-point scale. RESULTS: Various amounts of mucin deposition were observed in the periadnexal area of patients who were diagnosed with chronic eczema. Mucin deposition was more visible after staining with mucin-specific stains. Evaluation of the staining analysis scores revealed that the staining intensities were significantly higher in patients with chronic eczema than age- and site-matched controls (normal, acute to subacute eczema, and psoriasis vulgaris). CONCLUSION: Periadnexal mucin (secondary mucinoses) may be an additional finding of chronic eczematous dermatitis.

Mucinose oral focal: descrição de um caso clínico / Large oral focal mucinosis: a case report

The aim of this article is to report the case of a 23-year-old female patient that sought dental service for examination of an asymptomatic slow-growing large lump in the mandibular gingiva causing displacement of the right first molar. Excisional biopsy, histopathological analysis, Alcian blue/periodic acid-Schiff (PAS) staining and immunohistochemistry analysis for S-100 were performed. The diagnosis established was oral focal mucinosis. After three years, the patient is still under follow-up, without any recurrences. Although cases of oral focal mucinosis of this particular size are rare, this entity should be considered in the differential diagnosis of oral lesions located in the gingiva...

O objetivo deste artigo é relatar o caso de uma paciente de 23 anos de idade que procurou o serviço odontológico para exame de um grande nódulo de crescimento lento, assintomático, na gengiva mandibular, o que causou deslocamento do primeiro molar direito. Foram realizadas biópsia excisional, análise histopatológica, coloração Alcian blue/periodic acid-Schiff (PAS) e análise imuno-histoquímica para S-100. O diagnóstico de mucinose oral focal foi estabelecido. A paciente ainda está sob acompanhamento, sem recorrência, após três anos. Embora os casos de mucinose oral focal com esse tamanho sejam raros, esta entidade deve ser considerada no diagnóstico diferencial das lesões orais localizadas em gengiva...

A Case of Congenital Mucinous Nevus on the Back / 대한피부과학회지

Mucinous nevus is a rare entity classified as either cutaneous mucinosis or connective tissue nevi. Clinically, grouped papules or plaques grow to form a verrucous or nevoid feature usually on the trunk. It can be present since birth or early childhood. Histologically, it consists of mucin deposits localized in the superficial dermis with or without epidermal changes. An 18-year-old boy presented with asymptomatic multiple grouped brown papules and coalescent plaques on his lower back that had been present since birth. Histological examination showed a band-like mucin deposit in the papillary dermis. To our knowledge, there have been only few reports of mucinous nevus in the literature. Here, we report a case of mucinous nevus present since birth, including a review of relevant literature.

Primary systemic amyloidosis, acquired cutis laxa and cutaneous mucinosis in a patient with multiple myeloma / Amiloidose sistemica primaria, cutis laxa adquirida e mucinose cutanea em paciente com mieloma multiplo

A 57-year-old woman presented with periorbital ecchymoses, laxity in skin folds, polyneuropathy and bilateral carpal tunnel syndrome. A skin biopsy of the axillary lesion demonstrated fragmentation of elastic fibers, but with a negative von Kossa stain, consistent with cutis laxa. The diagnosis of primary systemic amyloidosis was made by the presence of amyloid material in the eyelid using histopathological techniques, besides this, the patient was also diagnosed with purpura, polyneuropathy, bilateral carpal tunnel syndrome and monoclonal gammopathy. She was diagnosed as suffering from multiple myeloma based on the finding of 40% plasma cells in the bone marrow, component M in the urine and anemia. The patient developed blisters with a clear content, confirmed as mucinosis by the histopathological exam. The final diagnoses were: primary systemic amyloidosis, acquired cutis laxa and mucinosis, all related to multiple myeloma.

Mulher de 57 anos, com equimose periorbitária, frouxidão cutânea nas dobras, polineuropatia e síndrome do túnel do carpo bilateral.O exame histopatológico da lesão axilar revelou fragmentação de fibras elásticas, porém a coloração de von Kossa foi negativa;o diagnóstico foi de cútis laxa. Amiloidose sistêmica primária foi confirmada pela presença de material amilóide no exame histopatológico da pálpebra, além de púrpura, polineuropatia, síndrome do túnel do carpo bilateral e gamopatia monoclonal. Foi diagnosticada como portadora de mieloma múltiplo por apresentar 40% de plasmócitos na medula óssea, componente M urinário e anemia. A paciente evoluiu com bolhas de conteúdo citrino, cujo exame histopatológico mostrou mucinose. Os diagnósticos finais foram: amiloidose sistêmica primária, cútis laxa adquirida e mucinose, todos vinculados ao mieloma múltiplo.

Cutaneous mucinosis in mixed connective tissue disease* / Mucinose cutanea na doenca mista do tecido conjuntivo

Cutaneous mucinosis is a group of conditions involving an accumulation of mucin or glycosaminoglycan in the skin and its annexes. It is described in some connective tissue diseases but never in association with mixed connective tissue disease. This report concerns two cases of cutaneous mucinosis in patients with mixed connective tissue disease in remission; one patient presented the papular form, and the other reticular erythematous mucinosis. These are the first cases of mucinosis described in mixed connective tissue disease. Both cases had skin lesions with no other clinical or laboratorial manifestations, with clinical response to azathioprine in one, and to an association of chloroquine and prednisone in the other.

A mucinose cutânea é um grupo de condições em que há um acúmulo de mucina ou glicosaminoglicanos na pele e seus anexos. É descrita em algumas doenças do tecido conjuntivo, porem nunca em associação com doença mista do tecido conjuntivo. Relatamos dois casos de mucinose cutânea em pacientes com doença mista do tecido conjuntivo em remissão, um apresentava-se sob a forma papular e outro sob a forma reticular eritematosa de mucinose. Estes são os primeiros casos de mucinose descritos na doença mista do tecido conjuntivo. Ambos os casos apresentaram o quadro cutâneo de modo isolado, sem nenhuma outra manifestação clínico-laboratorial, havendo resposta à azatioprina em um e à cloroquina associada a prednisona no outro.

Scleredema diabeticorum: A case report

Scleredema is an uncommon condition of unknown etiology that is characterized by dermal mucinosis and mild sclerosis. It is a symmetrical, diffuse, non-pitting induration of the skin commonly associated with an antecedent febrile illness, diabetes mellitus or paraproteinemia.This is the case of an obese, middle-aged adult female with type 2 diabetes mellitus on oral hypoglycemic medication, who presented with an ill-defined, diffuse erythema and non-pitting induration of the skin on the posterior neck and upper back. It has been estimated that as many as 2.5%-14% of diabetic patients have scleredema. This subset of patients may be under-reported as a consequence of subtle onset and under- recognition. Even lithe prognosis of scleredemadiabeticorum is usually benign, it is important that this condition is recognized since it may have systemic involvement that can lead to complications.