Primary localized cutaneous lichen myxedematosus with light chain-restricted plasma cells: A distinct variant of the localized form of lichen myxedematosus.

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.

Nodular-Type Lichen Myxedematosus: A Rare Variant With Exclusive Involvement of the Hands-Report of Two Cases With a Comprehensive Review of the Literature.

ABSTRACT: Lichen myxedematosus (LM) is an uncommon cutaneous mucinosis characterized by the deposition of mucin and fibroblast proliferation in the dermis. This condition can be classified into 2 forms: a diffuse/generalized LM, also known as scleromyxedema, associated with monoclonal gammopathy and systemic implications, and a localized form, primarily affecting the skin. Within the localized form, nodular-type LM is a rare variant presenting as firm, skin-colored to pinkish mucinous nodules. In this article, we report 2 new cases of nodular-type LM with exclusive involvement of the hands and provide a comprehensive review of the diagnosis, histopathological aspects, and therapeutic considerations of this rare condition.

Consensus statement on the diagnosis and treatment of sclerosing diseases of the skin, Part 2: Scleromyxoedema and scleroedema.

The term 'sclerosing diseases of the skin' comprises specific dermatological entities, which have fibrotic changes of the skin in common. These diseases mostly manifest in different clinical subtypes according to cutaneous and extracutaneous involvement and can sometimes be difficult to distinguish from each other. The present consensus provides an update to the 2017 European Dermatology Forum Guidelines, focusing on characteristic clinical and histopathological features, diagnostic scores and the serum autoantibodies most useful for differential diagnosis. In addition, updated strategies for the first- and advanced-line therapy of sclerosing skin diseases are addressed in detail. Part 2 of this consensus provides clinicians with an overview of the diagnosis and treatment of scleromyxoedema and scleroedema (of Buschke).

Small lymphocytic lymphoma presenting as chronic diffuse lip swelling.

Although rare, small lymphocytic lymphoma can present as chronic lip swelling and papules, thus mimicking the features of orofacial granulomatosis, a chronic inflammatory disorder characterized by subepithelial noncaseating granulomas, or papular mucinosis, characterized by localized dermal mucin deposition of mucin. When assessing lip swelling, one must carefully consider the clinical clues and have a low threshold to perform a diagnostic tissue biopsy, preventing delays in treatment or progression of the lymphoma.

Scleromyxedema histopathologically mimicking hypercellular fibrous papules (angiofibomas): Case report of an unusual histopathological presentation.

Scleromyxedema (SMX) is an inflammatory condition of unknown etiology strongly associated with monoclonal gammopathy. Classical histopathology of SMX is characterized with the triad of diffuse mucin deposits, increased amount of collagen, and presence of stellate fibroblasts. Herein, we report an unusual histopathological variant of SMX in a 41-year-old female with lesions of the nose histopathologically mimicking cellular angiofibromas. The dome-shaped papules were characterized by increased collagen bundles and fascicles of spindle cells. Widened vessels were seen at the periphery of the proliferation. Cells expressed CD68. Factor XIIIa was expressed only by dendritic cells. The mucin was highlighted with colloidal iron. In sum, we draw attention to this unusual variant of SMX, which should be suspected in a setting of multiple "angiofibromas/fibrous papules" on the face with presence of mucin.

Scleromyxedema with multiple systemic involvement: Successful treatment with intravenous immunoglobulin.

Scleromyxedema is a rare connective tissue disorder characterized by a generalized lichenoid eruption and sclerodermoid induration with histologic features of dermal mucin deposition. A 44-year-old man presented with a 3-year history of generalized progressive skin thickening and sclerosis. He had diffuse skin-colored to erythematous firm papules coalescing into indurated plaques over his whole body. He had been diagnosed with scleromyxedema from a skin biopsy with monoclonal gammopathy of undetermined significance (MGUS) at another tertiary hospital 3 years earlier. He had been treated with systemic corticosteroids and methotrexate, but his systemic symptoms (dyspnea, dysphagia, skin swelling, and induration) had worsened over the past year, so he visited our clinic seeking further evaluation and management. The patient received high-dose intravenous immunoglobulin (IVIG) therapy once a month in combination with systemic corticosteroids. After three courses of IVIG, his cutaneous symptoms and dyspnea had improved dramatically. Herein we report a case of scleromyxedema with systemic involvement with significant improvement following IVIG therapy.

Scleromyxedema in a 21 year old female patient with acute lymphoblastic leukemia: a case report.

BACKGROUND: Scleromyxedema is a rare, para-neoplastic, chronic, progressive condition of the Lichen myxedematosus (LM) family. The clinical picture consists of generalized confluent papular eruptions with possible systemic manifestations, which may be fatal as it still constitutes a therapeutic dilemma. Histologically, it is characterized by dermal mucin deposition, fibroblast proliferation with fibrosis, with monoclonal gammopathy in the absence of thyroid disease. Some atypical forms of the disease were reported in the literature, but none were reported in acute leukemia. CASE PRESENTATION: Herein, we report a case of a 21 years old female patient, known case of acute lymphoblastic leukemia (ALL), who developed numerous hyper-pigmented erythematous papules and plaques, mainly over her thighs, lower abdomen, and sub-mammary flexures. Histopathology of skin lesions confirmed the diagnosis of atypical scleromyxedema. Her symptoms significantly improved with the use of high dose intravenous immunoglobulin (IVIG). CONCLUSIONS: Despite that scleromyxedema is associated with many hematologic disorders, it is very rarely associated with acute lymphoblastic leukemia, and a high index of suspicion is needed for diagnosis. IVIG remains a reasonable management of such a disabling disease.

Lichen Myxedematosus: Case Report and Review of Literature.

Lichen myxedematosus (LM) is an idiopathic cutaneous mucinosis, commonly described as localized scleromyxedema. In contrast to scleromyxedema, there is typically no systemic involvement. Treatment options are limited and spontaneous resolution has been reported. We present the case of a 66-year-old Hispanic male referred by his primary care physician for evaluation of asymptomatic dark spots on his trunk and extremities present for about one-year. Physical exam revealed smooth, brown hyperpigmented papules coalescing into plaques on the trunk. Multiple well-demarcated oval dark brown plaques measuring 3 cm in size were located on the upper back, peri-umbilical area, bilateral lower extremities, and buttocks. A diagnosis of lichen myxedematosus was made based on histologic features observed in the dermis. There are 5 subtypes of LM: a discrete papular form, acral persistent papular mucinosis, self-healing papular mucinosis, papular mucinosis of infancy, and a pure nodular form. Occasional patients with LM have atypical features or features intermediate between scleromyxedema and localized LM. We present a case of atypical LM with mixed features of the different subtypes. Herein we will review the varied clinical presentations of LM and highlight the distinguishing features of scleromyxedema. J Drugs Dermatol. 2020;19(3): 320-322 doi:10.36849/JDD.2020.4864.

Scleromyxedema.

Scleromyxedema is a rare, cutaneous deposition disorder from the group of mucinoses, which can affect multiple organs and is virtually always associated with a monoclonal gammopathy. Cutaneous manifestations are usually generalized, 2 to 3 mm sized, dome-shaped or flat-topped, waxy, slightly red to skin-colored papules and sclerodermoid indurations. Neurological, rheumatological, cardiovascular, gastrointestinal, respiratory tract, renal and ophthalmologic manifestations can occur, with decreasing frequency. A serious and potentially lethal complication is the dermato-neuro syndrome which manifests with flu-like prodromes followed by fever, convulsions and coma. Untreated, scleromyxedema usually takes an unpredictable and potentially lethal progressive disease course over several years. According to a widely acknowledged classification by Rongioletti a diagnosis of scleromyxedema can be rendered when (1) generalized, papular and sclerodermoid eruption, (2) a histological triad of mucin deposition, fibroblast proliferation and fibrosis, and (3) monoclonal gammopathy are present, and (4) thyroid disease is absent. Apart from the classic microscopic triad, an interstitial granuloma annulare like pattern was also described. The pathogenesis of scleromyxedema is unknown. A potential role for various, as yet unknown serum factors has been discussed. An unequivocal causal relationship between paraproteinemia and disease manifestations could not be established to date. High dose intravenous immunoglobulins (IVIg) are the first-line treatment of choice according to the most recent European guidelines.

An atypical presentation of discrete papular mucinosis in the genitalia mimicking a molluscum contagiosum infection.

Discrete papular mucinosis is a rare variant of primary cutaneous mucinosis. Involvement of genitalia is extremely rare and can mimic molluscum contagiosum. We report the second case of a papular mucinosis with an exclusive genital involvement.

[Mimetics of systemic sclerosis]. / Imitatoren der systemischen Sklerose.

BACKGROUND: Systemic sclerosis (SSc) is characterized by heterogeneous clinical symptoms. Peripheral skin fibrosis can be a common symptom. Nevertheless, a variety of diseases with different etiologies are associated with a thickening of the skin and make the initial diagnosis of systemic sclerosis more difficult. OBJECTIVE: The different disease entities that can lead to dermal fibrosis should be differentiated. An earlier diagnosis of SSc would therefore be facilitated. METHODS: A literature search was carried out for clinical pictures that can be associated with skin fibrosis. The clinical picture, the etiology and the treatment of the individual diseases are described. RESULTS: Diseases that can mimic the cutaneous symptoms of SSc include morphea, scleroderma, diabetic cheirarthritis, scleromyxedema, nephrogenic systemic fibrosis and eosinophilic fasciitis. The characteristic pronounced skin involvement, an accompanying Raynaud's phenomenon, capillary microscopy, histopathology and antinuclear antibodies help to enable a differentiation of SSc from its mimics. CONCLUSION: An early differential diagnostic distinction between SSc and other sclerosing diseases is important due to SSc-associated and potentially life-threatening systemic organ involvement. If a diagnosis of SSc has been made, a critical and organ-specific evaluation with respect to pulmonary, gastrointestinal, renal and cardiac involvement is mandatory and should be repeated at regular intervals.

Interstitial Granulomatous Variant of Scleromyxedema-A Diagnostic Pitfall.

Scleromyxedema is a rare disorder where patients may develop systemic manifestations such as monoclonal gammopathy, inflammatory polyarthritis, and esophageal and neurological dysfunction. Histologically, there may be atypical variants of scleromyxedema showing features resembling interstitial granuloma annulare. We report an unusual case of scleromyxedema with interstitial granulomatous pattern and highlight potential diagnostic pitfalls when encountered with such a variant.

[Scleromyxedema]. / Skleromyxödem.

Scleromyxedema is a rare disorder that frequently affects multiple extracutaneous organ systems and is usually associated with monoclonal gammopathy. The pathogenesis of scleromyxedema is unknown. The clinical course is chronic and progressive and can lead to marked morbidity or death. The skin findings consist of multiple waxy papules and indurated plaques. Progressive skin involvement can lead to decreased mobility of the mouth and joints. Extracutaneous manifestations occur in the musculoskeletal or cardiovascular system, in the gastrointestinal or respiratory tract, or in the kidneys. There are no approved or evidence-based treatment options available for scleromyxedema. High-dose immunoglobulins are considered the treatment of choice, followed by lenalidomide (or thalidomide) and systemic glucocorticosteroids, or in severe cases even autologous hematopoetic stem cell transplantation. Long-term maintenance treatment is usually required and close clinical follow-up is necessary as recurrence of scleromyxedema is common after withdrawal of an effective therapy.

Cutaneous mucinosis of infancy: report of a rare case and review of the literature.

Cutaneous mucinosis of infancy (CMI) is a rare dermatologic condition, first reported in 1980 and currently classified within the complex group of papular mucinoses. We report a case of CMI and review the prior 13 cases in the literature. The patient was a 5-year-old girl who presented with asymptomatic dermal papules and plaques on her leg and back with no overlying color change. These lesions were first noticed during infancy and had become slightly more evident over time. The patient had a history of birthmarks and eczema. Her family history included eczema, allergies, photosensitivity, and Graves disease. Pre-biopsy clinical differential diagnosis included connective tissue nevus, granuloma annulare, myofibroma, lipofibroma, and lymphangioma. Biopsies revealed significant increase in interstitial mucin within the reticular and mid dermis, without significant sclerosis or fibroblastic proliferation. The relatively quiescent pattern of interstitial mucinosis with slight fibrocyte hyperplasia presenting as dermal papules-plaques on the trunk and extremities was most consistent with a diagnosis of CMI. We report another case of CMI in an otherwise healthy patient. Our patient is unique as she is the first CMI patient with a family history of Graves disease, although our patient appeared euthyroid. We will also review the literature on this rare entity.

[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.