RESUMEN
Lichen myxedematosus (LM) is a chronic cutaneous mucinosis that can present as a localized skin lesion or as a generalized systemic disease termed scleromyxedema. The differential diagnosis is determined by a combination of clinical presentation, serological studies, and histopathological examination. Currently, well-established and accepted histopathological features to distinguish localized LM from scleromyxedema have not been elucidated. Our recent publication, together with a retrospective literature review, suggests that the presence of groups of light chain-restricted plasma cells represents a distinct histopathological clue for the diagnosis of localized LM. In this report, we provide two additional cases of localized LM with lambda light chain-restricted plasma cells, together with clinical and histopathological findings that are similar to our previous publication. These cases support our theory that the light chain-restricted plasmacytic microenvironment is primarily attributed to the pathogenesis of localized LM. Therefore, we consider these cases to constitute a clinically and pathologically new variant of localized LM and name it primary localized cutaneous LM with light chain-restricted plasma cells.
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Células Plasmáticas , Escleromixedema , Humanos , Células Plasmáticas/patología , Células Plasmáticas/inmunología , Escleromixedema/patología , Escleromixedema/diagnóstico , Femenino , Masculino , Persona de Mediana Edad , Diagnóstico Diferencial , Adulto , Cadenas lambda de Inmunoglobulina , AncianoRESUMEN
BACKGROUND: Acral persistent papular mucinosis (APPM) is a rare idiopathic subtype of localized lichen myxedematosus. To date, there have been 40 APPM cases reported worldwide; however, only 7 cases have been reported in the Korean literature. CASE SUMMARY: A 70-year-old man was referred to our hospital with a solitary pinkish nodule on the dorsum of his right hand. Despite the absence of symptoms, the patient wanted to know the exact diagnosis; thus, a biopsy was performed. Histopathological examination of a biopsy specimen obtained from the nodule on the dorsum of his hand revealed orthokeratotic hyperkeratosis with patchy parakeratosis, prominent hypergranulosis, and diffuse dissecting mucinous deposition between collagen bundles, along with some bland-looking spindle cells throughout the dermis. The nodule was histologically diagnosed as an APPM, and an intralesional triamcinolone injection (2.5 mg/mL) was started every 2 wk. After three sessions of treatment, the patient showed marked improvements. CONCLUSION: To the best of our knowledge, this is the first case of a Korean APPM presenting as a solitary nodule that showed a marked response to triamcinolone intralesional injection. Since it is a rare disease, we report this case to contribute to future research on the pathogenesis and treatment of APPM.
RESUMEN
Lichen myxedematosus (LM) is an idiopathic cutaneous mucinosis disorder, and monoclonal gammopathy of undetermined significance (MGUS) is a preneoplastic plasma cell disease with a monoclonal increase in globulin. Patients with LM combined with monoclonal gammopathy are normally diagnosed with scleromyxedema. However, we report a case of generalized papules combined with MGUS in a 78-year-old man who was eventually diagnosed with atypical or intermediate forms of LM because it only involved the skin, and the pathological type was not consistent with scleromyxedema. Few cases of atypical or intermediate forms of LM have been reported, so the course of atypical or intermediate forms of LM is unpredictable. We report the diagnosis and treatment of a case of atypical forms of LM to discuss the current understanding of the disease, hoping to provide a reference for clinical research on this disease.
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Localized lichen myxedematosus (LM) is a rare, idiopathic mucinosis characterized by dermal mucin deposition and variable fibroblast proliferation. Nodular lichen myxedematosus, a clinicopathologic subtype of localized LM, is exceedingly rare in pediatric patients with only three prior cases reported. Understanding of LM in pediatric patients is limited by the rarity of the disease, and diagnosis is complicated by overlapping clinical and histopathologic features. There is no standardized treatment for localized LM and treatment is largely dictated by a patient's desire to minimize cosmetic disfigurement. This case series reports two additional patients with juvenile nodular lichen myxedematosus, highlights the limitations of existing diagnostic criteria, and describes successful treatment of one patient with intralesional triamcinolone.
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Scleromyxedema is an uncommon and progressive fibromucinous disorder characterized by disseminated papular eruption with histological features of dermal mucin deposition. The skin changes associated with this disease are highly visible and they tend to affect the patient's quality of life. We report a case of a 50-year-old male patient that presented a 3-year-old history of disseminated asymptomatic firm papules-associated systemic symptoms. Medical treatment with oral corticosteroid and thalidomide was indicated and surgical treatment on residual facial folds was performed, with an excellent outcome.
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The skin manifestations of monoclonal(M)-proteinemia are rare and present in patients with monoclonal gammopathy of undetermined significance (MGUS), smoldering plasma cell myeloma (SMM) and multiple myeloma (MM). In this study, we reported 4 cases with M-proteinemia-related rare skin lesions, including pyoderma gangrenosum (PG), erythema elevatum diutinum (EED), cutis laxa (CL) and lichen myxedematosus(LM). These skin lesions are specific, where the potential mechanism was immune-mediated paraneoplastic syndrome rather than direct plasma cell infiltration. Anti-plasma cell treatment was effective in treating skin lesions. The clinical outcome of MM-related skin changes was correlated to tumor control, whereas the prognosis of MGUS or SMM related skin lesions was favorable. Skin involvement in M-proteinemia is extremely rare and less well-known, which greatly impairs quality of life. The diagnosis and treatment of these 4 cases support the need for futher study.
RESUMEN
Scleromyxedema and lichen myxedematosus (LM) are rare disorders that fall along the spectrum of primary cutaneous mucinoses. Scleromyxedema is a systemic form that classically presents with generalized waxy papules, sclerodermoid eruption, and monoclonal gammopathy; LM is a localized form limited to the skin that classically presents with white, firm, waxy papules and lacks monoclonal gammopathy. According to diagnostic criteria established in 2001, the diagnosis of both conditions requires absence of thyroid disease. However, atypical cases that lack monoclonal gammopathy and that present with hypothyroidism have been reported, suggesting that these criteria may require revision. First, we report a case of a 58-year-old female with a history of Hashimoto thyroiditis and biopsy-proven scleromyxedema responsive to intravenous immunoglobulin therapy with delayed presentation of monoclonal gammopathy. Next, we report a case of a 54-year-old female with a history of hypothyroidism, Hodgkin's lymphoma in remission after radiation and chemotherapy, and concurrent rheumatoid arthritis, with biopsy-proven LM temporarily responsive to systemic steroids. Our cases demonstrate that patients with papular mucinoses can have a multitude of concurrent and prior rheumatologic and endocrine conditions, including thyroid disease, which should not preclude a diagnosis of scleromyxedema and LM.
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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.
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Escleromixedema , Diagnóstico Diferencial , Humanos , Escleromixedema/diagnóstico , Escleromixedema/patología , Piel/patologíaRESUMEN
Lichen myxedematosus is an idiopathic, cutaneous mucinosis with 2 clinicopathologic subsets. There is the generalised papular and sclerodermoid form, more properly termed scleromyxedema, and the localised papular form. We report the first case, to our knowledge, of lichen myxedematosus in association with rheumatoid arthritis as well as a case in association with dermatomyositis. An up-to-date literature review on cutaneous mucinoses and connective tissue diseases, excluding the common association of primary and secondary mucinoses with systemic lupus erythematosus, was also performed.
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Enfermedades del Tejido Conjuntivo , Mucinosis , Humanos , Masculino , Persona de Mediana Edad , Escleromixedema , Piel/patologíaRESUMEN
A 41-year-old man was referred to our outpatient department with a diagnosis of urticaria with angioedema of 3 months duration. On examination, he had generalized coalescent waxy papules and diffuse periorbital swelling. Systemic examination was unremarkable except for limited finger flexion. Serum electrophoresis and thyroid function tests were normal. Histopathological examination showed normal epidermis and intradermal mucin deposition, which was diagnostic of lichen myxedematosus (LM). The patient showed prompt response to melphalan. Here, we report this case of atypical LM because the patient had generalized eruption with normal thyroid function along with the absence of monoclonal gammopathy.
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BACKGROUND: Few histologic studies describe the histopathologic aspects of scleromyxedema. OBJECTIVE: We sought to describe the histopathologic and immunohistochemical features of scleromyxedema in a large series of patients. METHODS: We studied all the cases with scleromyxedema diagnosed between 2000 and 2014 at participating centers. Sections with hematoxylin-eosin and special stains were examined. Immunohistochemistry for CD3, CD4, CD8, CD20, CD68, and factor XIIIa was performed in 10 cases. RESULTS: A total of 44 skin biopsy specimens from 34 patients were reviewed. Two different histopathologic patterns were observed: the classic microscopic triad (dermal mucin deposition, fibroblast proliferation, fibrosis) was identified in 34 specimens, whereas an interstitial granuloma annulare-like pattern was found in 10 specimens. A superficial perivascular infiltrate with T lymphocytes was found in all specimens whereas an interstitial proliferation of CD68(+) epithelioid cells was identified in the 10 specimens with an interstitial granuloma annulare-like pattern. Elastic fibers were largely lost, explaining the redundant folds of the disease. LIMITATIONS: This was a retrospective study. CONCLUSIONS: Scleromyxedema shows 2 histopathologic patterns, including the classic type with the microscopic triad of mucin, fibroblast proliferation and fibrosis, and an interstitial granuloma annulare-like pattern. Recognition of these histologic presentations expands the spectrum of scleromyxedema and highlights the difficulty in diagnosing this disabling condition in the absence of a clinicopathological correlation.
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Antígenos CD/análisis , Factor XIIIa/análisis , Escleromixedema/patología , Piel/patología , Adulto , Anciano , Anciano de 80 o más Años , Antígenos CD20/análisis , Antígenos de Diferenciación Mielomonocítica/análisis , Complejo CD3/análisis , Antígenos CD4/análisis , Linfocitos T CD4-Positivos/química , Linfocitos T CD4-Positivos/patología , Antígenos CD8/análisis , Linfocitos T CD8-positivos/química , Linfocitos T CD8-positivos/patología , Citoprotección , Femenino , Fibroblastos/patología , Fibrosis , Histiocitos/química , Histiocitos/patología , Humanos , Inmunohistoquímica , Masculino , Persona de Mediana Edad , Mucinas , Estudios Retrospectivos , Escleromixedema/inmunología , Piel/químicaRESUMEN
BACKGROUND: Cutis laxa-like features were observed in a subset of patients with scleromyxedema. Given this observation, clinical and histopathologic features of scleromyxedema were reviewed in correlation with elastic tissue staining. METHODS: We retrospectively reviewed clinical records and histopathologic features from patients with scleromyxedema seen at our institution from 1992 through 2013. We also evaluated available skin biopsies with an elastin stain and assessed whether dermal elastin fibers were diminished in density or were fragmented (or both). RESULTS: Nineteen patients with scleromyxedema and 34 skin biopsies were identified. Alcian blue (mucin) stain was used to grade mucin deposition as weakly positive (24%), positive (44%) and markedly positive (32%). Eight patients (42%) had clinical findings of cutis laxa, which were often observed in conjunction with areas of papular eruption or induration. Elastic tissue fibers were normal in 9 of 34 skin specimens (26%), 18 of 34 specimens (53%) had diminished elastic fiber density and 7 of 34 (21%) had markedly decreased density. The elastic tissue was fragmented in 25 specimens (74%). CONCLUSIONS: A cutis laxa-like clinical presentation and decreased elastic tissue density on skin biopsy were consistent findings. Dermatologists and dermatopathologists should be aware of these previously unreported clinical and histopathologic findings.
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Cutis Laxo , Dermis , Elastina/metabolismo , Escleromixedema , Biopsia , Cutis Laxo/metabolismo , Cutis Laxo/patología , Dermis/metabolismo , Dermis/patología , Femenino , Humanos , Masculino , Persona de Mediana Edad , Estudios Retrospectivos , Escleromixedema/metabolismo , Escleromixedema/patologíaRESUMEN
Localized papular mucinosis is a type of mucinosis induced by several different causes. However, to the best of our knowledge, prior radiation therapy has not been reported to be related to papular mucinosis. We present a case of a 47-year-old woman who had undergone an operation for a breast carcinoma 2 years earlier and received local radiotherapy in the affected breast. Currently, she presents multiple erythematous papules that are caused by abundant dermal mucin deposits. We discuss some potential differential diagnoses.
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Neoplasias de la Mama/radioterapia , Traumatismos por Radiación/etiología , Escleromixedema/etiología , Neoplasias de la Mama/cirugía , Diagnóstico Diferencial , Femenino , Humanos , Persona de Mediana Edad , Traumatismos por Radiación/diagnóstico , Traumatismos por Radiación/patología , Escleromixedema/diagnóstico , Escleromixedema/patologíaRESUMEN
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.
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Adulto , Femenino , Humanos , Brazo , Fibroblastos , Inmunoglobulina G , Corea (Geográfico) , Pierna , Luz , Mucinas , Cuello , Paraproteinemias , Escleromixedema , Enfermedades de la TiroidesRESUMEN
An 18-year-old Japanese woman noticed a progressive appearance of nodules on both forearms and on the left cubital fossa and left thigh one year before her initial consultation at our department. Physical examination showed elastic hard, slightly elevated, shiny and yellowish to skin-colored nodules of 6 to 10 mm in size on her extremities. From laboratory and histopathological findings, nodular-type lichen myxedematosus was diagnosed and nodules showed complete remission following local injection of triamcinolone acetonide.
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Acral persistent papular mucinosis (APPM) is a rare subtype of localized lichen myxedematosus, which involves only the extensor surfaces of the hands, wrists and the distal part of forearms. Some subtypes of lichen myxedematosus including APPM have been reported with increased frequency in a patient with human immunodeficiency virus (HIV) infection. We describe a 31 year-old male patient with an HIV infection who presented with discrete, flesh-colored papules on the extensor surfaces of the wrists and the distal part of the forearms. Histopathologic findings revealed focal deposition of mucin in papillary and mid dermis that spared a small grenz zone. The lesions showed spontaneous regression after starting highly active antiretroviral therapy (HAART). We report a rare case of APPM in a patient with HIV infection which showed spontaneous regression with HAART, suggesting that APPM may represent one of the cutaneous markers of HIV infection.
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Adulto , Humanos , Masculino , Síndrome de Inmunodeficiencia Adquirida , Terapia Antirretroviral Altamente Activa , Dermis , Antebrazo , Mano , Infecciones por VIH , VIH , Mucinas , Escleromixedema , MuñecaRESUMEN
Lichen myxedematosus (synonym, papular mucinosis) is a disorder characterized by lichenoid papules, nodules and plaques due to dermal mucin deposition, and a variable degree of fibrosis without thyroid dysfunction. Discrete lichen myxedematosus is a subtype of the localized lichen myxedematosus. Clinically, firm, smooth, waxy or flesh-colored papules measuring 2 to 5 mm in diameter, numbering just a few to hundreds, and involving limbs and trunk. The lesions progress slowly without systemic symptoms and rarely resolve spontaneously. Histologically, the upper and mid dermis shows edema and diffuse or focal mucinous deposit under normal epidermis. Fibroblast proliferation is variable, but there is neither collagen deposition nor sclerosis. On experiencing a case of discrete grouped papules on the arm and thigh of a sixty three year-old women, we present it as discrete lichen myxedematosus.