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.

Lichen myxedematosus: a rare group of cutaneous mucinosis

Abstract: Cutaneous mucinoses are a heterogeneous group of dermatoses in which excess deposition of mucin in the dermis gives the skin a waxy appearance, with papules and plaques that can vary from self-healing mucinosis to even disrupting the normal shape of a patient's face, conferring a leonine facies, or be part of life threatening diseases like scleromyxedema. This review will describe the most recent classification on lichen myxedematosus in the generalized (scleromyxedema) and the localized forms, as well as the different organ systems involved in scleromyxedema, diagnostic workup, current management, and prognosis.

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.

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)

Case for diagnosis. Lichen myxedematosus

Abstract Scleromyxedema or lichen myxedematosus is a rare papular mucinosis of chronic and progressive course and unknown etiology. It is commonly associated with monoclonal gammopathy and may show extracutaneous manifestations, affecting the heart, lung, kidney, and nerves. The diagnosis is based on four criteria: generalized papular and sclerodermoid lesions; mucin deposition, fibroblast proliferation, and fibrosis in the histopathology; monoclonal gammopathy; and no thyroid disorders. This article reports the case of a scleromyxedema patient with a recent history of acute myocardial infarction and monoclonal gammopathy.

Acral papular mucinosis: a new case of this rare entity

Abstract Acral persistent papular mucinosis (APPM) is a rare subtype of localized lichen myxedematosus. It consists of small papules localized exclusively on the back of the hands, wrists and extensor aspects of distal forearms with no other clinical or laboratory manifestations. The lesions tend to persist and may increase slowly in number. Histologically, hematoxylin-eosin and Alcian blue staining demonstrate mucin accumulation in the upper reticular dermis with separation of collagen fibers as a result of hyaluronic acid deposition. Treatment is rarely necessary due to the absence of symptoms. We present a 27-year-old healthy woman with asymptomatic papules on her upper extremities, which adequately meet clinical and pathological criteria of acral papular mucinosis.

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.

Scleredema associated with Sjögren’s syndrome

Scleredema adultorum of Buschke is a rare disorder characterized by diffuse swelling and non-pitting induration of the skin usually involving the face, neck, arms and upper trunk. It has been associated with previous infectious diseases, diabetes, paraproteinemia and, more rarely, malignant neoplasms or autoimmune disorders. We report the case of a 30-year-old man who presented with a 2-year history of scleredema. Further investigation led to the diagnosis of primary Sjögren’s syndrome. The association between scleredema and autoimmune disorders has been rarely seen. To our knowledge, there are no other reports describing the association between primary Sjögren’s syndrome and scleredema adultorum of Buschke.

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.

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.

Síndrome REM associada a lúpus eritematoso sistêmico e hipotireoidismo / REM syndrome associated with systemic lupus erythematosus and hypotiroidism

A mucinose eritematosa reticulada é síndrome crônica e rara de etiologia desconhecida que afeta adultos jovens e de meia idade, principalmente do sexo feminino. Clinicamente é caracterizada por máculas eritematosas reticulares, pápulas e placas localizadas de forma simétrica em área central do tórax e dorso. Em aproximadamente 20 por cento dos casos pode estar associada com várias doenças, especialmente auto-imunes. Os autores apresentam um caso de mucinose eritematosa reticulada associada a lúpus eritematoso sistêmico e hipotireoidismo.

Reticular erythematous mucinosis is a chronic and rare syndrome of unknow aetiology that affects young adult and midle-aged women. Clinical presentation is characterized by macular and reticulated erythema, papula and plaques on the central chest and upper back of simmetrical form. In approximately 20 percent of the cases may be associated with a variety of disorders, especially auto-immune diseases. The authors present a case of reticular erythematous mucinoses associated with systemic lupus erythematosus and hypothiroidism.