New and emerging conditions of acquired cutaneous mucinoses in adults.

Cutaneous mucinoses are a heterogenous group of conditions, characterized by the deposition of glycosaminoglycans (mucin) in the dermis, follicles, or in the epidermis. Major cutaneous mucinoses include lichen myxedematosus, scleredema, mucinoses associated with thyroid disease, reticular erythematous mucinosis, papulonodular mucinosis associated with connective tissue diseases, and cutaneous focal mucinosis. The aim of this review is to provide an update of what has currently been reported in the last 30-year literature about several new or emerging conditions of acquired cutaneous mucinoses in adults. Two new clinico-pathologic entities have been described: (i) Obesity-associated lymphedematous mucinosis and pretibial stasis mucinosis; (OACM) (ii) Nodular mucinosis of the breast (NMB). Two relatively new disease categories encompassing cutaneous mucinoses with a common pathogenetic mechanism have been identified: (i) Cutaneous mucinoses associated with drug exposure including biologic therapy, anti-colony-stimulating factor 1 receptor (CSF1R) and subcutaneous intralesional interferons (toxic dermal mucinoses); (ii) Cutaneous mucinosis following physical agents including mechanical traumas and after knee replacement.

Reticular erythematous mucinosis: Literature review and case report of a 24-year-old patient with systemic erythematosus lupus.

Reticular erythematous mucinosis (REM) was first described 50 years ago, but only around 100 case reports in English have been published. Its relation with other inflammatory skin disorders is still being debated. We report a case of REM, including the clinical and histopathological findings. Also, a systematic review of 94 English-language reported cases is provided. The described criteria for clinical and histopathological diagnosis are highlighted in order to REM can be confidently diagnosed.

Oral focal mucinosis: A multi-institutional study and literature review.

BACKGROUND: Oral focal mucinosis (OFM) is a rare benign condition of unknown etiology, considered the oral counterpart of cutaneous focal mucinosis. We report the clinicopathologic features of 21 cases of OFM in conjunction with a review of the literature. METHODS: Clinical data were collected from the records of five oral and maxillofacial pathology services. All cases were evaluated by hematoxylin and eosin staining, histochemistry, and immunohistochemistry (vimentin, S-100, α-SMA, CD34, and mast cell). RESULTS: The series comprised 14 females (66.7%) and seven males (33.3%), with a mean age of 48.2 ± 20.7 years (range: 8-77 years) and a 2:1 female-to-male ratio. Most of the lesions affected the gingiva (n = 6, 28.6%) and presented clinically as asymptomatic sessile or pedunculated nodules with fibrous or hyperplasic appearance. All cases were negative for S-100 protein, CD34, and α-SMA and positive for Alcian blue staining. Conservative surgical excision was the treatment in all cases, and there was only one recurrence. CONCLUSION: OFM is a rare benign disorder that is often clinically misdiagnosed as reactive lesions or benign proliferative processes. Dermatologists and pathologists should consider OFM in the differential diagnosis of soft tissue lesions in the oral cavity, mainly located in the gingiva.

Dermal Mucinosis in Chronic Sclerodermoid Graft-Versus-Host Disease.

Dermal mucinosis is characterized by the deposition of glycosaminoglycans (mucin), either focally or diffusely within the dermis. This may occur as a primary idiopathic disorder or secondary to several dermatoses, most notably lupus erythematous, scleroderma, and dermatomyositis. The authors present an unusual finding of dermal mucinosis in association with chronic sclerodermoid graft-versus-host disease.

[Dermatological manifestations of monoclonal gammopathies: contribution of cutaneous histopathology]. / Manifestations dermatologiques des gammapathies monoclonales : apport de l'histopathologie cutanée.

Skin manifestations associated with monoclonal gammapathy are common and can present with various clinical and pathological aspects. They can be the first events leading to the diagnosis of monoclonal gammapathy. They may be present either as specific lesions, including lymphoplasmacytic or pure plasma cell neoplastic infiltrates and monoclonal immunoglobulin deposits, or as non-specific dermatitis, such as leukocytoclastic vasculitis, neutrophilic dermatoses, mucinoses or xanthomatosis, giving little clues for the diagnosis of the underlying disease.

Papulonodular mucinosis in a patient with systemic lupus erythematosus and antiphospholipid syndrome.

The skin is one of the target organs most commonly affected in lupus erythematosus (LE) and a wide range of cutaneous changes have been described in LE patients. Papulonodular mucinosis (PNM) in particular is an uncommon cutaneous manifestation of LE. We discuss the case of a 26-year-old Senegalese woman with systemic LE and antiphospholipid syndrome (APS) who presented with pruritic papules on her back and extremities that appeared when she was on vacation in Africa and non-compliant with medications. Histopathologic examination was consistent with PNM. The patient was treated with mycophenolate mofetil and hydroxychloroquine, with subjective relief in pruritis at 6-week follow-up. To our knowledge, this is the first case of PNM presenting in a patient with both SLE and APS. Whether APS contributes to the pathogenesis of PNM is currently unknown.

Oral focal mucinosis associated with surgically assisted rapid maxillary expansion.

Oral focal mucinosis is a rare soft-tissue lesion that might result from the overproduction of hyaluronic acid by fibroblasts. The lesions are commonly found on the gingiva and palate; however, other sites, such as the tongue, have also been reported. The diagnosis of oral focal mucinosis is based on histologic analysis, and treatment involves surgical excision. Recurrences of lesions have not been reported. This article presents a patient with oral focal mucinosis that might be associated with surgically assisted rapid maxillary expansion.

Reticular erythematous mucinosis: a review of patients' characteristics, associated conditions, therapy and outcome in 25 cases.

BACKGROUND: Reticular erythematous mucinosis (REM) is an uncommon disease, the nosology and specific characteristics of which are controversial because most reports deal with single cases or small series. OBJECTIVES: To describe the characteristics of patients with REM regarding demographics, clinical and pathological features, comorbidities, treatment and course. METHODS: A retrospective and prospective study was conducted on 25 patients diagnosed with REM in the setting of university-affiliated dermatology departments and dermatopathology centres. RESULTS: Of the 25 patients with REM, 16 were women (sex ratio 2 : 1) and the mean age was 46 years. The roles of sun exposure and oral contraceptives were ambiguous. Associated diseases included hypertension (n = 4), malignancies (n = 3), autoimmune diseases (n = 3) and Borrelia infection (n = 1). Immunological studies (including serology and direct immunofluorescence) were noncontributory. The response to antimalarial treatment was good in > 80% of cases. Worsening or recurrence of the lesion after treatment discontinuation, or in the course of the disease, occurred in 31% of patients. CONCLUSIONS: We present the largest REM case series to date. The reticular pattern with involvement of the midline of the chest and back, the predilection for middle-aged women, the controversial relationship with photosensitivity and the possible association with other conditions such as malignancies and thyroid dysfunctions are the main characteristics that makes REM a recognizable disease.

Intestinal Submucosal Mucinosis in a Patient With Systemic Lupus Erythematosus: A Case Report.

Systemic lupus erythematosus (SLE) is an autoimmune disease with various clinical presentations. Mucin deposition is a characteristic finding in skin lesions, but it is rare in other organs. We present a case with erythematous patches from the terminal ileum to the anus in an SLE patient. Diffuse colitis was diagnosed clinically. However, in addition to inflammatory cell infiltration, there was abundant mucinous material deposition in the submucosa. The mucinous material was positive for Alcian blue staining (pH 2.5) and was sensitive to hyaluronidase digestion. These findings are similar to those of cutaneous mucinosis in SLE patients. This is thought to be the first case of gastrointestinal tract mucinosis in SLE reported in the literature.

Nodular mucinosis in Graves disease mimicking gouty tophi.

We report a patient who had Graves disease and recently developed subcutaneous nodules over both lower limbs. Initially, gouty tophi were suspected by the endocrinologist so he was referred to the rheumatology clinic. However, a series of studies, including soft tissue ultrasonography, magnetic resonance image, and biopsy revealed nodular mucinosis resulting from Graves dermopathy. Nodular mucinosis is an unusual form of myxedema which mimics tophaceous gout, and should be considered in patients with Graves disease presenting with subcutaneous nodules.

Cutaneous Mucinosis Associated with Beckwith-Wiedemann Syndrome.

A 37-year-old man presented with firm, skin-colored papules and nodules on his back and chest, which had been appearing during the past 7 years (Figure 1a). The patient denied any associated pruritus, pain, or ulcerations. Further history revealed he had a repaired omphalocele during childhood. Physical examination revealed a large body habitus, with asymmetric overgrowth of the right extremities when compared to the left. In addition, the patient had bilateral anterior linear earlobe creases, preauricular pits, and posterior helical pits (Figure 1b). There was no evidence of rheumatologic and endocrine disorders or paraproteinemia.

Obesity-associated lymphoedematous mucinosis.

BACKGROUND: Mucin deposition on the shins is considered as an indicator of pretibial myxoedema, which is typically seen in patients with Graves' disease. OBJECTIVE: The purpose of this study was to report the clinical and histopathological features of a group of patients with pretibial mucinosis in the absence of thyroid disease. METHODS: Five patients are included in this series and studied both clinically and histologically and compared with similar cases in the literature. RESULTS: All patients were middle aged or elderly. Four patients were women. They were characterized clinically by morbid obesity and bilateral lower extremity pitting oedema sparing the feet. Semitranslucent papules and/or nodules and sometimes vesicles were found on the shins. Characteristic histological features include (i) hyperorthokeratosis with epidermal atrophy and effacement of the rete ridge pattern, (ii) oedema in the papillary and upper part of the reticular dermis with mucin deposition stained positively with alcian blue and colloidal iron, (iii) angioplasia in the upper part of dermis with upward-running, increased and thickened capillary vessels and (iv) variable fibrosis in the reticular dermis with separation of collagen bundles and increased stellate or linear fibroblasts. A hypocaloric diet was given in two cases, and an important weight loss was observed, which was accompanied by a marked improvement of the pretibial mucinosis. CONCLUSIONS: Pretibial mucinosis is a histological feature associated with morbid obesity and lymphoedematous features of the legs that should be distinguished from true pretibial myxoedema. The term of 'obesity-associated lymphoedematous mucinosis' seems to be appropriate for this condition.

Papulonodular mucinosis indicating systemic lupus erythematosus.

We describe an 18-year-old girl with systemic lupus erythematosus (SLE) who had cutaneous papulonodular mucinosis (PNM) as the first sign of SLE. She presented with multiple flesh-coloured papules on the face, abdomen and limbs. Histological examination of a biopsy taken from a papule showed diffuse deposition of mucin throughout the dermis, and direct immunofluorescence of lesional skin showed a dermoepidermal junction band composed of IgG, IgM and C3, consistent with PNM. Investigations showed that that the patient had leucopenia, positive antinuclear and anti-double-stranded DNA antibodies and lupus nephritis. PMN can be an unusual clinical presentation of SLE.

Reticular Erythematous Mucinosis: A Rare Cutaneous.

Reticular erythematous mucinosis (REM) is a rare form of primary cutaneous mucinosis, most often involving the midline of the upper chest or back in middle-aged women. REM bears clinical and histopathologic resemblance to lupus erythematosus tumidus (LET), dermatomyositis, scleredema, and lichen myxedematosus. Early recognition and diagnosis of REM is particularly relevant to exclude the abovementioned diseases, as REM is more benign and has fewer systemic consequences.

Stasis Mucinosis: Insights Into Euthyroid Localized Mucinosis.

INTRODUCTION: Stasis mucinosis and obesity-associated lymphedematous mucinosis (OALM) have been described as 2 discrete diagnostic entities. CASE REPORT: A morbidly obese African American man in his late 60s presented to the emergency room with a 3-month history of swelling of both lower extremities and secondary changes suggestive of lymphedema and venous dermatitis. On physical examination, the patient had severe edema with multiple raised areas of verruciform skin changes and varicosities, diffuse induration, erythema, and scaling. He also had an open wound in his left hallux. The skin biopsy found mucinosis. A diagnosis of stasis mucinosis was rendered. He had normal thyroid function test laboratory results. CONCLUSIONS: The authors suggest stasis mucinosis and OALM represent the spectrum of euthyroid mucin depositional disease in varying clinical settings.