[Scleromyxedema]. / Skleromyxödem.

Scleromyxedema or generalized diffuse lichen myxoedematosus is a rare mucinosis that is associated with monoclonal gammopathy and which frequently affects multiple extracutaneous organ systems. The pathogenesis of scleromyxedema has not been fully elucidated, but includes stimulation of glycosaminoglycan synthesis. The clinical course of scleromyxedema is chronic and often progressive, leading to severe morbidity and even death. The characteristic skin findings encompass multiple waxy papules often on indurated plaques, while thickening of skin leads to conspicuous folds on glabella and dorsal aspects of finger joints. Microscopical manifestations are dermal deposits of glycosaminoglycans between collagen bundles in reticular dermis, increased numbers of fibroblasts and fibrosis as well as loss of elastic fibers. Progressive skin involvement results in decreased mobility of the mouth and joints and even contractures. Extracutaneous manifestations occur in the musculoskeletal or cardiovascular system, in the gastrointestinal or respiratory tract, in the kidneys or in the central and peripheral nervous system. There are no in-label or evidence-based treatments available for scleromyxedema, but by expert consensus high-dose immunoglobulins are considered as treatment of choice, followed in case of insufficient efficacy by systemic glucocorticosteroids and then lenalidomide or thalidomide. In severe and refractory cases, autologous hematopoietic stem cell transplantation has been performed. Long-term maintenance treatment is usually required to prevent recurrences. Close interdisciplinary follow-up is recommended.

Scleromyxedema in an adult following Sinopharm BBIBP-CorV vaccination: An extremely rare phenomenon.

Introduction: The Sinopharm BBIBP-CorV vaccine produces a variety of cutaneous adverse effects. Scleromyxedema is a mucinous connective tissue disorder that causes skin thickness and sclerodermoid changes. According to our findings, this is the first case of scleromyxedema induced by the Sinopharm immunization. Case description: We discuss the case of a 75-year-old woman who acquired progressive thickening of the skin in her limbs and trunk after getting the Sinopharm vaccination. Examination, laboratory testing, and a biopsy were used to verify scleromyxedema diagnosis. Intravenous immunoglobulins, mycophenolate mofetil, and prednisolone were used in the treatment of the patient. The outcomes from the 4-month follow-up were reassuring. Conclusion: This study emphasizes the need of considering scleromyxedema as a connective tissue pathology in patients who have recently received Sinopharm vaccine and have similar cutaneous signs.

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.

Reticular Erythematous Mucinosis: Exclusive Involvement of the Axillary Region.

Reticular erythematous mucinosis is a primary cutaneous mucinosis characterized by the presence of erythematous macules, papules, or plaques coalescing into a reticular pattern that typically involves the midline of the chest or back in middle-aged women. Because of this peculiar location, it is also called midline mucinosis. Although atypical disease localizations have been described in the literature, these have always been accompanied by midline involvement. We report a case of reticular erythematous mucinosis with exclusive involvement of the axillary region.

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.

Scleromyxedema and lichen myxedematosus: Is it associated with viral hepatitis?

Scleromyxedema (SM) was previously known to be associated with monoclonal gammopathy. The association of SM and its counterpart lichen myxedematosus (LM) with chronic hepatitis has rarely been reported. We retrospectively reviewed medical records and histopathological reports of consecutive patients who presented at our department with the diagnosis of SM or LM from January 2001 to September 2017. The patients' demographic details, cutaneous presentation, associated underlying diseases and hepatitic profile were studied and compared with previous published cases. In all, 28 patients were enrolled, including one SM, 19 LM and eight atypical LM. Of the patients, 50% (n = 14/28) had hepatitis. Of these, 21.4% (n = 6/28) had hepatitis C, 10.7% (n = 3/28) hepatitis B, 7.1% (n = 2/28) concurrent hepatitis B and C, whereas 10.7% (n = 3/28) had alcoholic liver disease. The prevalence of hepatitis C in our patients was 6.5-times higher than that of the general population (28.6% vs 4.4%) and the prevalence of hepatitis B was similar (17.9% vs 17.3%). Polyclonal gammopathy was found in 28.6% (n = 8/28) of the patients and monoclonal gammopathy was found in 7.1% (n = 2/28). The extent of clonality did not correlate with disease severity. Our study did not notice a significant association with monoclonal gammopathy but the prevalence of hepatitis C was found to increase 6.5-times in these patients compared with the general population. We recommend dermatologists to be aware of hepatitis investigations in such patients and future studies are warranted to understand the mechanism behind such association.

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.

[Scleromyxedema as a systemic disease of glycosaminoglycan accumulation]. / Skleromiksedema kak sistemnaia bolezn' nakopleniia glikozaminoglikanov.

Scleromyxedema is a rare mucinosis with a primary skin lesion due to diffuse mucin deposition, sclerosis, and lichenoid eruptions in the absence of hypothyroidism. The paper describes scleromyxedema cases and gives recommendations for the histological diagnosis of the disease by histochemical reactions to detect acid and neutral glycosaminoglycans.

Trauma-induced Focal Nodular Mucinoses: A Rare Entity.

Cutaneous mucinoses refer to disorders with abnormal accumulation of mucin in the skin. Mucin is an amorphous material that is a part of the extracellular matrix in dermis. Cutaneous mucinoses could be primary or secondary. The latter is associated with systemic disorders such as autoimmune diseases, diabetes mellitus, paraproteinemia, or altered thyroid function. Here, we report an adult female patient with trauma-induced cutaneous focal nodular mucinoses on left-sided scapular region. Histology showed replacement of collagen bundles by mucinous deposits in superficial and mid-dermis. Mucinous substance was present in peri-eccrine location on Alcian blue staining.

[Is scleromyxedema a skin problem or systemic pathological process?] / Skleromiksedema - dermatologicheskaia problema ili sistemnyi patologicheskii protsess?

Scleromyxedema is regarded as a rare cutaneous mucinosis from a group of lichen myxedematosus characterized by diffuse mucin deposition, sclerosis, and lichenoid eruptions in the absence of thyroid disease. The paper discusses the pathogenesis of the disease and histological changes in tissues. It underlines the need for using histochemical tests to identify acidic and neutral glycosaminoglycans and gives a differential diagnosis of this disease.

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)

Scleromyxedema without Paraproteinemia: Treatment with Thalidomide and Prednisolone.

Scleromyxedema is a rare and distinctive variant of cutaneous mucinoses of unknown etiology. It is presenting with generalized papular eruption and sclerodermoid induration. Numerous treatment modalities have been reported to produce partial or permanent responses. This study reports on a case of scleromyxedema without paraproteinemia in a subject who experienced a partial response to thalidomide and prednisolone.

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.