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A 53-year-old female initially presented 8 years ago with papules, hardening, and itching on her trunk and limbs that had been present for 20 d. The dermatological condition presented as multiple waxy, small, round papules ranging from skin-colored to orange were observed on the patient's forehead, face, front chest, back, and bilateral arms extending to her hands. The papules exhibited a waxy surface, which were firm, and dome-shaped or flat-topped. The papules and nodules could coalesce to form plaques, and the papules were commonly arranged in a linear pattern, with deep grooves formed locally. Histopathology of the skin lesions on the left abdomen revealed generally normal epidermis, with interstitial mucin, increased fibroblasts, and collagen in the upper reticular dermis. The diagnosis was lichen myxedematosus. After discontinuing glucocorticoid and hydroxychloroquine sulfate, the patient engaged in self-psychological care. Subsequently, the rash gradually subsided, and there has been no recurrence during the 8-year follow-up. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

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.

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.

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. [ABSTRACT FROM AUTHOR]

Discrete papular lichen myxedematosus (DPLM), asubset of localized lichen myxedematosus, is a rarecutaneous mucinosis of unknown etiology. We reporta case of a 57-year-old woman with palmoplantarpsoriasis who developed DPLM 8 weeks after addingustekinumab to a long-term course of methotrexate.The patient had previously failed 2 prior tumor necrosisfactor (TNF) inhibitors, adalimumab and etanercept.This case demonstrates an association between TNFinhibitor and ustekinumab use in a psoriasis patientand localized lichen myxedematosus for the secondtime in the literature. The presented case is of interestbecause of the rare diagnosis of DPLM, especially inassociation with the start of the anti-IL 12/23 agentustekinumab. The appearance of DPLM in this settingsuggests a possible etiology for the disease.

Plaque-like cutaneous mucinosis (PCM) is a rare atypical subtype of Lichen myxedematosus, characterized by hyperpigmented plaques typically found on the midline trunk. We report the case of a 26-year-old woman with a 1-year history of painful, pruritic, hyperpigmented plaques over her spine. Physical examination revealed soft plaques in the midline of the back, and histopathology showed dermal mucin deposition, consistent with PCM. The presented case is notable as the first reported instance of PCM presenting with lesional pain. We propose that mechanical stress, likely due to repeated friction at the site of spinous processes, contributed to the development of pain. The fact that pain lessened after the use of a lumbar support pillow and gaining weight supports our hypothesis. Our case demonstrates the importance of considering PCM in the differential diagnosis of hyperpigmented plaques and highlights the need for further research to elucidate its underlying pathogenesis and optimize treatment strategies., Competing Interests: The author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article., (© The Author(s) 2024.)

Plaque-like cutaneous mucinosis (PCM) is a rare disorder of dermal mucin deposition.  Some patients with PCM will be found to have an associated malignancy. We report the case of a 72-year-old man presenting with new onset pruritic, waxy-appearing erythematous and skin-colored papules and nodules coalescing into plaques on his shoulder, scalp, and forehead. Skin biopsy revealed cutaneous mucinosis. Despite conservative treatment, his skin lesions progressed, and he was found to have an occult malignancy of pancreatobiliary origin. After several months of chemotherapy, his skin lesions showed progressive improvement. To our knowledge, this is the third reported case of paraneoplastic PCM and the first reported to occur in association with underlying adenocarcinoma of pancreatobiliary origin. PCM may occasionally represent a paraneoplastic dermatosis. This case highlights the importance of a search for occult malignancy in such patients.

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.

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.

Background: Papular mucinosis is a variety of mucinosis characterized by excessive production of mucin by fibroblasts and deposition in the dermis. It manifests itself with fleshy papules or plaques in different sites of the body and taking different clinical morphological cutaneous features. Objective: The objective is to report and evaluate the different clinical and histopathological features of the disease in Iraqi patients. Patients and Methods: This is a case series and a clinical descriptive study in which ten patients with papular mucinosis were reported during the period from 2012 through 2019. The age ranged from 4 to 56 years, with seven females and three males. Clinical evaluation regarding histories of the disease and examination was carried out. General investigation was done and skin biopsy for histopathological assessment was conducted. Results: Nine patients were adults, with their age ranging from 20 to 56 years, a mean of around 35 years, and only one 4-year-old child. It is a disease with a female predominance, as observed in 7 (70%) females. The common sites of involvement were the face but the rash may extend to affect the neck and upper arms. The rash appeared in the form of skin-colored or red fleshy papules and plaques or in diffuse erythematous orange peel-like forms. The rash was asymptomatic in most patients. The pathology of the disease clarified the diffuse deposition of mucin in the dermis, as was demonstrated with H&E staining. Conclusion: Papular mucinosis is a rare disease characterized by mucin deposition in the skin affecting mostly adult females. The face is commonly involved together with other areas such as the neck and upper arms, but the trunk and lower limbs are spared. It manifests itself with different clinical morphological cutaneous features. Papular granuloma annulare must be considered as an important differential diagnosis in all cases of papular mucinosis. [ABSTRACT FROM AUTHOR]

A case of myxedematous lichen is reported. A 60 years old male presented with pigmentation and pruritus for 2 years, and aggravated for 1 month. He was admitted to Hainan Fifth People's Hospital on March 6, 2019. Two years ago, there was no obvious inducement for the patient to have plaque on his face, which gradually increased, hardened and appeared dark brown pigmentation. With the gradual development of the disease, trunk skin appeared rough, hypertrophy, hardening and pigmentation, hand joints appeared stiff and affected activity. Special examination showed diffuse yellowish brown patches and plaques on the forehead, which were distributed in a grid shape, and scattered with atrophic hypopigmentation spots. Black and brown patches were scattered on the back, partially fused into pieces. Sclerotic and atrophic white plaques can be seen in the neck. The skin of the front chest and back is slightly thickened, and obviously hardened. It can still be pinched up. The skin lesions have no tenderness. The joints of the fingers are stiff and the movement is limited. Skin biopsy: The trochanterellus of epidermis disappeared, fibroblasts were arranged in the whole dermis, and some of them were disordered. Ashen blue staining showed that there were clumps of positive material deposition between collagen fibers and around hair follicles in the whole dermis, which was consistent with lichen myxedematosus (LM). The patient was treated with thalidomide 50 mg 3 times a day, prednisone 30 mg orally and acitretin 10 mg twice a day orally, combined with twice a week photochemical therapy (PUVA), external application of allantoin ointment, desonide cream, Halometasone Cream. On June 10, 2019, the patient's condition gradually became stable when he came to the follow-up visit, so the drug dosage was reduced to prednisone 15 mg once a day and acitretin 10 mg twice a day, once a week for photochemical therapy, and topical plaster continued. On September 8, 2019, the patient's condition was further improved when he came to the follow-up visit, so the patient's oral medication was reduced to acitretin 10 mg/d, and allantoin ointment was applied externally. On December 1, 2019, the patient's condition had improved significantly (the patient refused to take photos). Continued treatment was given to the case. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

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.

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. [ABSTRACT FROM AUTHOR]

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.

We report the successful treatment of plaque-like sclerodermiform mucinosis using tacrolimus ointment topically. We present a 70-year-old male with a large chronic erythema and hardening of the nuchal skin and shoulder area. Subjective symptoms were a moderate pruritus and a rather disabling stiffness. A biopsy specimen revealed typical features of lichen myxedematosus. In a subsequent clinical examination, no associated illnesses such as hypothyroidism or gammopathy were found. Since no established therapy exists for this condition, and as there was a lack of response to potent topical glucocorticosteroids, tacrolimus 0.03% ointment was used off-label twice daily. Surprisingly, this resulted in a rapid, almost complete clearance of the skin within three weeks of treatment.

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. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

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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., Competing Interests: The authors have declared that no competing interests exist., (Copyright © 2022, Duran-Lemarie et al.)

Scleromyxedema is a generalized form of lichen myxedematosus which is characterized by a papular and sclerodermoid skin eruption resulting from dermal fibroblast proliferation and mucin deposition. The majority of patients with scleromyxedema have a monoclonal gammopathy, and other systemic manifestations are common. Herein we describe a case of the 'dermato-neuro syndrome', a rare and sometimes fatal neurologic manifestation of scleromyxedema which consists of fever, convulsions and coma, often preceded by a flu-like prodrome. In addition, we provide a comprehensive summary of previously published cases of the dermato-neuro syndrome and discuss the current etiopathogenic theories and treatment options for this rare disease. Fleming KE, Virmani D, Sutton E, Langley R, Corbin J, Pasternak S, Walsh NM. Scleromyxedema and the dermato-neuro syndrome: case report and review of the literature. [ABSTRACT FROM AUTHOR]

Scleroderma is a rare systemic autoimmune disease with multiple organ manifestations, including skin fibrosis. The groups of disorders classified as scleroderma mimics share the common thread of skin thickening but are otherwise quite incongruous in terms of underlying disease process and other organ involvement. This article reviews the clinical presentation, etiology, and treatment options available for scleroderma mimics, including morphea, scleredema, diabetic cheiroarthropathy, scleromyxedema, nephrogenic systemic fibrosis, and eosinophilic fasciitis. Through greater understanding of these diseases and the associated extradermal implications, we hope to facilitate recognition of scleroderma and its mimics. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Lichen myxedematosus (LM) is an idiopathic cutaneous mucinosis; its classification dates back to 1953, when Montgomery and Underwood distinguished 4 types of LM. In the literature, the terms LM, papular mucinosis, and scleromyxedema often have been used indiscriminately as synonyms, but most reported cases of LM or papular mucinosis without indication of the subtype appear in fact to be cases of scleromyxedema. Actually, LM includes 2 clinicopathologic subsets: a generalized papular and sclerodermoid form (the only one which should be called scleromyxedema) with systemic, even lethal, manifestations and a localized form, which does not run a disabling course. The localized form is subdivided into 4 subtypes: (1) a discrete papular form involving any site; (2) acral persistent papular mucinosis involving only the extensor surface of the hands and wrists; (3) papular mucinosis of infancy, a pediatric variant of the discrete form or the acral form of persistent papular mucinosis; and (4) nodular form. A third group of atypical or intermediate forms, not meeting the criteria for either scleromyxedema or the localized form, includes cases of (1) scleromyxedema without monoclonal gammopathy, (2) localized forms with monoclonal gammopathy and/or systemic symptoms, (3) localized forms with mixed features of the subtypes, and (4) not well-specified cases. [Copyright &y& Elsevier]

This article presents the diagnosis of acral persistent papular mucinosis (APPM) for a 61-year-old man suffering from flesh-colored papules on the wrists. Patients of this disease present with multiple, asymptomatic, noncoalescing, skin-colored papules that are limited in distribution to the extensor surfaces of the hands and wrists. The pathogenesis of APPM is unknown, and its course is characterized by either no change or an indolent progressive increase in the number and distribution of papules. Spontaneous remission generally does not occur.

This article presents a question of diagnosing a 61-year-old man suffering from flesh-colored papules on the wrist. Skin examination showed approximately 40 discrete skin-colored to translucent, dome-shaped, firm, 2-to 3-mm papules that were symmetrically distributed over the dorsal aspect of the wrists and hands. There was no erythema or induration of the surrounding skin. His medical history was significant only for papillary thyroid carcinoma, which had been diagnosed in 1990, and for which he had been treated with thyroidectomy and radiation therapy. On presentation, his thyroid function was normal and he had no systemic complaints.