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.

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.

Scleromyxedema: clinical diagnosis and autopsy findings

Abstract Scleromyxedema is a rare chronic cutaneous mucinosis of unknown etiology. It is characterized by papular eruption and scleroderma with microscopic evidence of mucin deposition, fibroblast proliferation, and fibrosis. Most patients with scleromyxedema have monoclonal gammopathy and systemic manifestations resulting in significant morbidity and mortality. Several types of treatment have been reported with partial or inconsistent responses. Despite showing unpredictable evolution, systemic consequences of scleromyxedema and treatment side effects may result in death. We describe a rare case of a patient with scleromyxedema without paraproteinemia with systemic involvement that evolved to death despite treatment with cyclophosphamide.

Respuesta a la talidomida en escleromixedema / Response to thalidomide in scleromyxedema

El escleromixedema es una enfermedad poco frecuente caracterizada por una hiperproliferación de fibroblastos con depósito dérmico incrementado de mucina, que en la mayoría de los casos se asocia con una gammapatía monoclonal de significado incierto. Han sido comunicados diversos tratamientos, con resultados inconsistentes. Esto, sumado a la rareza de la enfermedad y a la falta de ensayos clínicos controlados aleatorios, da lugar a opciones terapéuticas derivadas de informes anecdóticos. Se describe el caso de una paciente de 52 años con diagnóstico de escleromixedema que desarrolló una gammapatía monoclonal, tratada con talidomida con buena respuesta clínica y de laboratorio. Es importante remarcar la necesidad de realizar un seguimiento clínico a largo plazo en estos pacientes, por el riesgo de evolución hacia mieloma múltiple y aparición de complicaciones relacionadas con los tratamientos sistémicos.

The scleromyxedema is a rare condition characterized by hyperproliferation of fibroblasts with increased dermal deposition of mucin and frequently associated with monoclonal gammopathy of undetermined significance. Various treatments have been reported, with inconsistent results. In addition, the rarity of the disease and the lack of randomized controlled trials results in treatment options derived from anecdotal reports. We describe the case of a 52 year-old female patient diagnosed with scleromyxedema who developed a monoclonal gammopathy, with adequate response to thalidomide. The follow up of these patients is important due to the risk of progression to multiple myeloma and complications related to systemic treatments.

Scleromyxedema with Monoclonal Gammopathy / 대한피부과학회지

Scleromyxedema is a rare disorder characterized by generalized papular and sclerodermoid eruption, increased fibroblast proliferation, mucin deposition, and monoclonal gammopathy in the absence of thyroid disease. It is a generalized subtype of lichen myxedematosus. A paraproteinemia, typically an IgG lambda, is observed in more than 80% of patients with scleromyxedema. Here, we report a 38-year-old woman with a 1-year history of a progressively spreading of eruption of small papules on the entire body, including the face, neck, arms, legs, and trunk. Laboratory tests were within normal limits, except lambda light chain monoclonal gammopathy. We administered oral retinoid and topical steroid with slight clinical improvement. To our knowledge, this is the first reported case of scleromyxedema in Korea.

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.

Mucinose papulosa associada ao hipotireoidismo / Papular mucinosis associated with hypothyroidism

A mucinose papulosa ou líquen mixedematoso é doença idiopática, manifestada por pápulas, nódulos ou placas por depósito de mucina na derme, na ausência de doença tireoidiana. Apresentamos um caso atípico, com lesões exuberantes em placas, associado ao hipotireoidismo subclínico. Nas mucinoses cutâneas há quantidade anormal de mucina na pele. Tradicionalmente as classificações consideram critério diagnóstico para mucinose papulosa a ausência de doença tireoidiana. Poucos casos associados ao hipotireoidismo são descritos e, assim como o nosso, constituem líquen mixedematoso atípico, sugerindo que a presença de doença tireoidiana não deva ser critério de exclusão para essa doença.

Papular mucinosis or lichen myxedematosus is an idiopathic disorder characterized by papules, nodules or plaques caused by mucin deposition in the dermis, in the absence of thyroid disease. The present report describes an atypical case with exuberant lesions in the form of plaques, associated with subclinical hypothyroidism. In cutaneous mucinosis, there is an abnormal quantity of mucin in the dermis. In traditional classifications of papular mucinosis, an absence of thyroid disease constitutes a diagnostic criterion. A few cases of the disease have been reported in association with hypothyroidism constituting, as in the present case, atypical lichen myxedematosus. It is therefore suggested that the presence of thyroid disease should not be considered an exclusion criterion for the diagnosis of this condition.

A Case of Discrete Papular Mucinosis / 대한피부과학회지

Papular mucinosis, or lichen myxedematosus is one of the rare cutaneous diseases characterized by papular-lichenoid eruptions, in which mucin deposits in the dermis without thyroid dysfunction. A 31-year-old male presented with 3-5mm sized waxy, flesh-colored, multiple firm papules on the upper and lower extremities and trunk for 5 months. He did not have any familial medical history. None of the laboratory findings, including serum protein electrophoresis, was significant. Skin biopsies were performed from papules of his trunk and arm. Histopathological findings showed that the collagen fibers in the upper dermis were loosely arranged and separated by amorphous materials. These materials were all positive for alcian blue, colloidal iron, mucicarmine, and Giemsa staining. This case is compatible with discrete papular mucinosis which is the rare subtype of localized papular mucinosis.

Two Cases of Papular Mucinosis / 대한피부과학회지

Scleromyxedema is a rare fibromucinous disorder that is often difficult to treat and is associated with significant morbidity and mortality. Clinically, it shows white or flesh colored, dome-shaped waxy papules on the hands, arms, face, neck and upper trunk. There are proliferation of fibroblasts and mucin deposition in dermis and presence of serum paraproteins. It should be differentiated from scleroderma because of sclerotic skin changes. Patients were a 70 year old male and a 45 year old female. They showed multiple confluent or linearly arranged waxy papules on the head, neck and extremities. Sclerotic plaques were also seen. Abnormal findings were not detected in laboratory tests and bone marrow biopsy. Histologically, mucin deposition and marked proliferation of fibroblasts were seen in dermis. Therapy was performed with 200 mg/day of cyclophosphamide. Both patients showed good response and had no recurrence. The latter one had shown improvement of skin lesions but subsequently she developed leukocytopenia. Cyclophosphamide was stopped temporarily until the abnormal leukocytes count recovered. During therapy, mild hemiparesis developed. We could not find the cause of the symptom through physical examination and radiologic studies.

Dermatoses that Present with Cutaneous Sclerosis

The diseases which present with cutaneous sclerodermatous changes are scleroderma, eosinophilic fasciitis, mixed connective tissue disease, sclerederma adultorum, scleromyxedema and cutaneous midline mucinosis. This paper reviews the characteristics and differential diagnosis among of the above mentioned diseases.