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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]

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]

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]

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]

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 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]

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.

A patient is described with typical skin lesions of lichen myxedematosus and IgG-type lambda paraproteinemia. Fibroblasts cultured from the skin of the patient and from the skin of control persons were used to study glycosaminoglycan and collagen synthesis; the cultures were labelled with H-glucosamine and H-proline, respectively. Fibroblasts from the patient grew to a cell density which was lower than that of the control fibroblasts. The production of glycosaminoglycans was increased in lichen myxedematosus cultures, so that the ratio of hyaluronic acid to sulphated glucosaminoglycans was higher in the patient's cultures than in control cultures. Collagen production in the patient's cultures was about half of that in control cultures, whereas the ratio of type III to type I collagen was normal. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

A 34-year-old white man developed acute rhabdomyolysis during the treatment of scleromyxedema with interferon alfa. If interferon alfa is to be used as a therapeutic option for scleromyxedema the possibility of rhabdomyolysis should be considered. To the best of our knowledge, this is the first case in which acute rhabdomyolysis was developed during the treatment of scleromyxedema with interferon alfa. [ABSTRACT FROM AUTHOR]