Mucin Deposition Research Articles

Pulsed dye laser as an excellent choice of treatment for lupus tumidus: a prospective study

Clinical manifestations of cutaneous lupus erythematosus (CLE) vary widely, and different subtypes of this autoimmune disease exist. Tumidus subtype (LT) has been recently separated from the chronic subtypes and is now considered an independent entity due to its particular clinical and histological features. Different treatments are usually prescribed for CLE. Our group has experience of CLE effectively treated with pulsed dye laser (PDL). It was our impression that better responses were achieved in the LT subtype, but no controlled prospective studies with PDL have been specifically performed. A prospective study was performed on 10 patients with histologically confirmed LT treated with PDL. All patients were treated with 595 nm PDL using the 10 mm spot size at 0.5 ms pulse width and a fluence of 8 J/cm(2). Biopsies were taken before and 4 weeks after treatment and were stained with haematoxylin-eosin. Evaluation after PDL treatment showed clinical improvement in all of them without side-effects and reduction of the dermal lymphocytic infiltrate in 9/10 of the patients. Epidermal changes were absent in all patients. Mucin deposition persisted only in one patient. However, 50% of the patients developed new lesions nearby or distant to the treated zones. PDL therapy is an effective and fast treatment option for acute flares of LT; however, it does not prevent recurrences. A histological improvement has been confirmed in this study. Purpura seems to be necessary to achieve a good response.

A case of pachydermodactyly in a 16 year-old male

Background Pachydermodactyly is a rare benign superficial fibromatosis, aetiology unknown, characterized by asymmetric painless swelling of the proximal interphalangeal (PIF) joints of the hands, most frequently in adolescent males. Radiographs and MRI show only soft tissue thickening. Histological exam reveals epidermic hiperplasia, collagen fibers and fibroblast proliferation and increased mucin deposition.

Scleroderma Renal Crisis-Like Acute Renal Failure Associated With Mucopolysaccharide Accumulation in Renal Vessels in a Patient With Scleromyxedema

Scleromyxedema is a systemic disease characterized by lichenoid papules, nodules, and plaques on the skin and often diffuse skin induration resembling the cutaneous involvement of systemic sclerosis. The systemic involvement affects the musculoskeletal, pulmonary, cardiovascular, gastrointestinal, and central nervous systems, and the disorder is commonly associated with a paraproteinemia. Involvement of the kidney is rare and not considered a feature of the disease. Here, we describe an unusual case of scleromyxedema complicated by the development of scleroderma renal crisis-like acute renal failure with a marked intimal deposition of mucin, mucopolysaccharides, and hyaluronic acid in the intrarenal vessels.

Successful Treatment of Subacute Lupus Erythematosus With Ustekinumab

A 58-year-old woman presented with a history of subacute cutaneous lupus erythematosus (SCLE) for the past 6 years, associated with photosensitivity, arthralgia, and fatigue. There was no evidence of systemic lupus erythematosus (SLE) or any history of exposure to drugs known to induce SCLE. Previous treatments included hydroxychloroquine sulfate, dapsone, prednisone, azathioprine, mycophenolate mofetil, topical corticosteroids, and tacrolimus 0.1% ointment, with limited benefit. Examination of the skin revealed erythematous and variably hyperkeratotic plaques (Figure, A) covering 70% of the body surface area. Laboratory test results were positive for SS-A/Ro and antinuclear antibodies (titer 1:640) but negative for SS-B/La and double-stranded DNA antibodies. Histopathologic analysis demonstrated vacuolar interface dermatitis, with a superficial and middermal perivascular and periadnexal inflammatory infiltrate consisting of mononuclear cells. There were faint deposits of mucin. Direct immunofluorescence revealed focal cytoid bodies and IgG and fibrin deposits at the dermoepidermal junction. This constellation of findings was consistent with the diagnosis of SCLE. THERAPEUTIC CHALLENGE

Lúpus eritematoso crônico discoide nas linhas de Blaschko

Linear chronic discoid lupus erythematosus is a rare manifestation of cutaneous lupus in which erythematous, atrophic, dyschromic lesions are located along the lines of Blaschko. This report describes the case of a 15-year old boy with a 2-year history of discoid, erythematous, hyper and hypopigmented lesions with central atrophy, situated along the lines of Blaschko on his right arm. Histopathology showed epidermal atrophy, hyperkeratosis, follicular plugging, thickening of the basement membrane zone, and superficial and deep chronic perivascular and periadnexal inflammatory infiltrate, with dermal mucin deposit, thus confirming diagnosis. A total of 14 cases have been described of this variant, the onset of which is often in childhood. There is no difference in incidence between genders. Lesions most commonly develop on the face. There have been no reports of any association with systemic disease.

Histopathology and Immunohistochemistry of Cutaneous Lupus Erythematosus after Pulsed Dye Laser Treatment

Cutaneous lupus erythematosus (CLE) is a heterogeneous autoimmune disorder with a wide range of skin manifestations. Current treatment options include topical and systemic approaches. Few controlled prospective studies have been performed using the pulsed dye laser (PDL). Based on previous experience that supported the efficacy of PDL treatment of CLE, we decided to study the histological changes induced by PDL. A prospective study was performed on nine patients with histologically confirmed CLE treated with PDL. Biopsies were taken before, immediately after, and 4 weeks after treatment and stained with hematoxylin and eosin and with commercially available antibodies to intercellular adhesion molecule (ICAM)-1 and vascular cell adhesion molecule (VCAM)-1. Evaluation after PDL treatment showed a significant reduction of the dermal lymphocytic infiltrate in six of nine patients (66.7%) and an important reduction of the basal damage in six of seven patients (85.7%). Other epidermal changes improved in four of six patients (66.7%). Mucin deposition persisted in two patients. ICAM and VCAM expression was reduced in seven of seven patients (100%) and five of six patients (83.3%) (p<.05). Clinical improvement was present in eight of nine patients (88.9%), without side effects. PDL therapy is an effective treatment for CLE. Immunohistologic improvement has been confirmed in this study.

A novel case of follicular mucinosis after autologous stem-cell transplantation for multiple myeloma

Follicular mucinosis is a rare inflammatory disorder of unknown aetiology, characterized by mucin deposition in hair follicles and sebaceous glands. FM can occur as a benign idiopathic primary disorder or secondary to malignant lymphoproliferative processes, most notably mycosis fungoides. We report a novel case of FM developing after autologous stem-cell transplantation for multiple myeloma, a correlation not previously reported in the literature.

Localized Papular Mucinosis With IgA Nephropathy

Papular mucinosis is an idiopathic cutaneous mucinosis. It includes 3 clinicopathologic subsets according to the recent Rongioletti classification: a generalized papular form, a localized form, and atypical forms. The localized form is subdivided into 5 subtypes. The atypical form does not meet the criteria for either the generalized papular form or the localized forms and includes localized forms with monoclonal gammopathy and/or systemic symptoms. We describe a 37-year-old man who had multiple small, waxy papular lesions on the neck for 4 years. One year after the lesions first appeared, he developed IgA nephropathy. No paraproteinemia or thyroid dysfunction was observed. Histologic examination of the skin lesions showed mucin deposits in the upper dermis. Alcian blue-positive mucin was also found in the renal interstitium and around some vessels and capillary loops. A diagnosis of localized papular mucinosis with IgA nephropathy (atypical form) was made after the biopsy. The patient showed an unusual manifestation of localized papular mucinosis with the lesions confined to the neck and IgA nephropathy. Alcian blue staining is a useful way to determine kidney involvement of papular mucinosis.

Lipedematous alopecia with mucinosis: report of the first case in Taiwan

Lipedematous alopecia is an uncommon disease that mainly affects African American and Egyptian women. This report is of an 18-year-old Taiwanese woman who presented with asymptomatic boggy and thickened scalp for 10 years. In the last 6 months, there was diffuse hair loss on the affected scalp without scarring. Histopathologically, there were increased thickness of subcutaneous fat layer, mild perivascular lymphocytic infiltration, and separated collagen bundles in the dermis. Alcian blue stain demonstrated mucin deposition in the dermis and subcutis, whereas magnetic resonance imaging showed thickened scalp with expanded subcutaneous fat layer. The clinical findings and imaging study established the diagnosis of lipedematous alopecia. The pathogenesis and disease etiology remain unclear. The coexistence of mucin is extremely rare and its significance should be further investigated.

Primary cutaneous marginal zone B-cell lymphoma with anetoderma: spontaneous involution plus de novo clonal expansion

We describe a rare case of primary cutaneous marginal zone B-cell lymphoma (PCMZL) in a 33-year-old male affected by hepatitis B virus (HBV)-related chronic active hepatitis. The patient presented with multiple cutaneous papulonodular lesions characterized by polymorphic immunomorphology, with inverse pattern of immunoglobulin light chain restriction in two different lesions, coupled with mucin deposition and anetoderma in a third lesion. The involution of lesions of PCMZL with secondary anetoderma has been previously described, but this has not been reported in the context of multiple lesions showing differing immunoglobulin light chain restriction. We speculate that these findings may be secondary to a variable interaction between a chronic antigenic stimulus (i.e. HBV) and its specific immune response.

Cutaneous Manifestations of Thyroid Disease: A Case of Thyroid-Induced Myxedema

The cutaneous manifestations of thyroid disease can be present in the hair, nails, and local or diffuse locations throughout the skin. Traditionally, thyroid-associated mucin deposition is present in a pretibial location on bilateral lower legs. We present a case of a growing plaque on the lower back of a 10-year-old girl, whose appearance coincided with a recent diagnosis of Hashimoto's thyroiditis.

Mucin-containing Rokitansky-Aschoff Sinuses With Extracellular Mucin Deposits

The American Journal of Surgical Pathology 35(1):p 153-154, January 2011. | DOI: 10.1097/PAS.0b013e3182011b98

Myxoid intravascular fasciitis

Nodular fasciitis is a common reactive proliferation of soft tissue. It is composed of high density of fibroblasts and myofibroblasts, and sometimes shows a sarcomatoid appearance histologically. A rare variant of nodular fasciitis, intravascular fasciitis can be found in the veins or arteries. Histologically, intravascular fasciitis has been described that resembles nodular fasciitis with a less prominent mucoid matrix. Here, we report a case of intravascular fasciitis with prominent mucin deposition. Clinically, it presented as multiple nodules on the leg of the patient. Histologically, it showed proliferation of spindle cells in a vein of the dermis and Alcian blue staining confirmed prominent mucin deposition between the spindle cells. Immunohistochemistry studies showed the spindle cells were positive for vimentin, actin and smooth muscle actin, but negative for keratin, S100 protein, desmin, CD31 and CD34. Based on the clinical and histopathological properties, a diagnosis of myxoid intravascular fasciitis was made.

Palmar Fasciitis and Polyarthritis Syndrome in Patients with Ovarian Cancer—A Case Report and Review of the Literature

Palmar Fasciitis and Polyarthritis Syndrome in Patients with Ovarian Cancer—A Case Report and Review of the Literature

Concomitant occurrence of patch granuloma annulare and classical granuloma annulare

Granuloma annulare (GA) is characterized clinically as annularly-distributed, erythematous papules on the extremities in children and adolescents. GA is recognized histologically as palisading granulomas with central degenerated collagen and mucin deposits. Here, we present a case of concomitant occurrence of patch GA (PGA), the most rare type of GA, and classical GA in a patient. A 60-year-old man was referred to our hospital for asymptomatic eruptions on the upper arms, forearms, right flank and right lateral chest. Clinical examination revealed annular erythematous plaques composed of numerous small papules on bilateral upper arms and forearms. Moreover, an indurative, exudative erythematous to violaceous plaque was present on the right lateral chest and right flank. Histopathology of the former was compatible with palisade-type GA, and the latter interstitial-type GA. This is the first report of PGA concomitant with "classical" annular papular lesions.

Peritumoral clefting in basal cell carcinoma: correlation of in vivo reflectance confocal microscopy and routine histology

Histopathologically, basal cell carcinoma (BCC) is characterized by basaloid tumor nodules of varying size showing peripheral palisading of cells and nuclei, and separation from surrounding stroma by optically empty appearing clefts. These are usually regarded as an artifact, occurring during routine tissue processing. Recently, reflectance confocal microscopy (RCM) has been applied for noninvasive, in vivo evaluation of BCC. Besides other features, small areas of low refractility separating tumor islands from the surrounding tissue can be observed in vivo, suggesting that the presence of amorphous material like mucin might be the causal factor for these clefts. A total of 13 BCCs were studied by RCM and histopathological techniques. Staining was performed with Alcian blue for the detection of peritumoral mucin. Correlation between RCM images and histopathological samples was studied, and the diameter of hyporefractile areas on RCM as well as the thickness of peritumoral mucin was measured. Good correlation was seen between dark areas on RCM and thickness of peritumoral mucin with a mean diameter of 14 µm (RCM) and 11.44 µm (histopathology), respectively. Pearson correlation coefficient was 0.605 (p < 0.0001). Our results show that the peritumoral cleft-like spaces seen in BCC on histopathology exist in vivo, and correspond to the peritumoral mucin deposition.

Extensive Fat Necrosis With Lipomembranous Changes and Calcification in Lupus Erythematosus Panniculitis Is Not Necessarily Associated With Systemic Lupus Erythematosus

Biswas A, 2007, EUR J DERMATOL, V17, P550; Fraga J, 2008, DERMATOL CLIN, V26, P453, DOI 10.1016-j.det.2008.06.002; FRAGA J, 2008, DERMATOL CLIN, V26, pR6; Martens PB, 1999, J RHEUMATOL, V26, P68; Ng PPL, 2002, INT J DERMATOL, V41, P488, DOI 10.1046-j.1365-4362.2002.01510.x; Suda T, 2007, EUR J DERMATOL, V17, P86; TUFFANELLI DL, 1982, HAWAII MED J, V41, P394; TUFFANEL.DL, 1971, ARCH DERMATOL, V103, P231, DOI 10.1001-archderm.103.3.231; Watanabe T, 1996, BRIT J DERMATOL, V134, P123, DOI 10.1111-j.1365-2133.1996.tb07850.x; Yamamoto T, 2007, CLIN EXP DERMATOL, V32, P278, DOI 10.1111-j.1365-2230.2007.02358.x

Caractéristiques cliniques et évolutives du scléromyxœdème : étude rétrospective multicentrique

scleromyxoedema is characterized by dermal mucin deposition associated with a monoclonal gammopathy. This is a rare disease, mostly reported as isolated cases. There is limited data regarding the course and prognosis of the disease. The aim of this study was to determine the clinical characteristics and course of scleromyxoedema. this was a retrospective study in patients from five French university hospitals between 1987 and 2007. Data were collected using a standardized questionnaire. The inclusion criteria were based on the disease diagnosis criteria proposed by Rongioletti and Rebora: (1) generalized, papular and sclerodermiform skin eruption, (2) mucin deposition in the dermis, fibroblastic proliferation and skin fibrosis, (3) presence of a monoclonal gammopathy, (4) absence of thyroid disease. eight patients were included. The mean age at disease onset was 51.5 years (range: 35-67). The mean time from primary symptoms and diagnosis was 41.6 months (range: 4-120). Seven patients had extra-cutaneous involvement: four with peripheral neuropathy and three with interstitial pneumonia. The mean follow-up time was 9 years. Four patients improved: two experienced partial remission and two complete remission. Complete remission was obtained under treatment with dexamethasone (one patient) and thalidomide (one patient). One patient presented a myeloma and one patient presented encephalopathy leading to death. our study shows the frequency of extra-cutaneous involvement and shows that complete remission occurs in some patients.

Successful Treatment of Necrobiotic Xanthogranuloma With Intravenous Immunoglobulin

Necrobiotic xanthogranuloma (NXG) is a rare systemic disease first described by Kossard and Winkelmann in 1980, and about 100 cases have been reported since then. It is clinically characterized by indurated yellowish to red-orange or brown papules or nodules that grow into larger and in some cases very extensive plaques covering the face (especially with periorbital distribution), trunk, and extremities. Lesions are nearly always asymptomatic, but secondarily they may become ulcerated. Moreover, pathologic changes in internal organs have been documented, including giant-cell myocardial disease. Because involvement of the heart seems to be relatively frequent, routine echocardiography and dynamic cardiac imaging are recommended in all patients. Necrobiotic xanthogranuloma also may involve other organs including the lung, larynx, pharynx, skeletal muscle, kidney, spleen, ovary, and intestine. Histopathologically, NXG is characterized by a granulomatous inflammation in the dermis extending into the subcutaneous fat. Dense infiltrates of macrophages with some foreign body–type giant cells and foamy macrophages are accompanied by areas of degenerated collagen and a moderate lymphocytic infiltrate in some cases with plasma cells. Mucin deposition or cholesterol clefts can be found. The underlying pathogenesis of the disease remains unknown, although in up to 80% of the patients, an association with paraproteinemia, especially monoclonal IgGproteinemia, can be found. Less often, bone marrow examination shows multiple myeloma. No first-line NXG therapy has been established. The recommended therapies of corticosteroids (intralesional and/or systemic), alkylating agents (such as cyclophosphamide, melphalan, or chlorambucil), interferon alfa, antimetabolites, antimicrobial treatment, and plasmapheresis have shown inconsistent success. To our knowledge, this is the first report of successful treatment of NXG with intravenous immunoglobulin (IVIg).

Cutaneous Focal Mucinosis Arising From the Chin

Cutaneous focal mucinosis is a type of degenerative-inflammatory dermal mucinoses characterized with asymptomatic, single, dermal mucin deposition. Because of its rarity, it is often mistaken clinically for other disorders such as sebaceous cyst, fibroma, myxoma, and xanthoma. In this study, we will discuss a case of cutaneous focal mucinosis arising from the chin of a 27-year-old man.