Papulonodular Mucinosis Research Articles

Refractory papulonodular mucinosis successfully treated with tofacitinib.

Tofacitinib may be a promising treatment option for PNM. To the best of our knowledge, this is the first case report highlighting the use of tofacitinib in the management of PNM.

Mucinous Lupus Alopecia with Papulonodular Mucinosis as a Sole Cutaneous Manifestation of Systemic Lupus Erythematosus

Mucinous Lupus Alopecia with Papulonodular Mucinosis as a Sole Cutaneous Manifestation of Systemic Lupus Erythematosus

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.

Papulonodular mucinosis in the setting of systemic lupus erythematosus – a diagnostic conundrum

Papulonodular mucinosis in the setting of systemic lupus erythematosus – a diagnostic conundrum

A Case of Papulonodular Mucinosis in a Patient With Systemic Lupus Erythematosus

Papulonodular mucinosis is a distinctive lupus erythematosus cutaneous variant that generally occurs in patients with systemic lupus erythematosus. Despite a higher incidence of SLE in women, lupus cutaneous mucinosis occurs more frequently in men. Typically, papulonodular mucinosis appears as asymptomatic, flesh-colored papules and nodules with a propensity for the trunk and upper extremities. Herein, we report a case of papulonodular mucinosis associated with systemic lupus erythematosus in a middle-aged woman. On presentation, patient had multiple flesh-colored papules coalescing into plaques on the trunk and upper extremities. Histological findings of the lesions demonstrated a pale dermis secondary to profuse mucin deposition. Immunofluorescent staining revealed a 'lupus band' with granular deposition of immunoglobulins/complement proteins at the dermal-epidermal junction.

Papulonodular mucinosis, Guillain-Barr\xe9 syndrome and nephrotic syndrome in a patient with systemic lupus erythematosus: a case report

BackgroundAwareness of the spectrum of clinical manifestations of systemic lupus erythematosus (SLE), especially uncommon changes, is essential for diagnosis and effective management of patients.Case presentationA 26-year-old Chinese man with SLE initially manifested cutaneous papulonodular mucinosis and developed acute Guillain-Barré syndrome and class V lupus nephritis 2 years later. His cutaneous nodules had not been idententified for 2 years and were resected by surgical procedures twice until SLE was diagnosed. The kidney biopsy revealed class V lupus nephritis. The patient responded well to a short course of intravenous immunoglobulins and his muscle strength almost completely recovered. So far, he has undergone five cycles of cyclophosphamide combined with hydroxychloroquine and tapering prednisone, resulting in partial remission of lupus nephritis and disappearance of hypocomplementemia.ConclusionWe reported a rare case of male patient with SLE with manifestation of class V lupus nephritis, Guillain-Barré syndrome and papulonodular mucinosis.

Visualization of Epidermal and Dermal Alteration in Papulonodular Mucinosis by Multiphoton Microscopy

Papulonodular mucinosis (PM) is a cutaneous clue to the presence and activity of silent lupus erythematosus (LE), but the exact pathogenesis is still under secret. Moreover, the currently available treatments for PM are not satisfactory. To demonstrate the possibility of multiphoton microscopy (MPM) to trace the pathological state of PM and evaluate the treatment efficacy, epidermal and dermal alteration in skin lesion with PM before and after treatment was examined using MPM. Microstructure of epidermis as well as content and distribution of collagen and elastin in dermis were quantified to characterize the pathological states of PM. The results showed significant morphological difference between skin lesion before and after treatment, indicating the possibility of MPM to assess the therapeutic efficacy. With the advancement on MPM miniaturization and enhancement of contrast and depth of imaging, the MPM technique can be applied in in vivo tracking PM formation and progression, and leading the better understanding the PM pathogenesis and mechanism of response to any treatment, helping to establish novel effective therapies for PM.

Numerous Mucin Nodules in a Patient with Seropositive Wide Spread Discoid Lupus Erythematosus

Numerous Mucin Nodules in a Patient with Seropositive Wide Spread Discoid Lupus Erythematosus

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.

Papulonodular Mucinosis in a Child with Systemic Lupus Erythematosus

Pediatric DermatologyVolume 24, Issue 5 p. 585-586 Papulonodular Mucinosis in a Child with Systemic Lupus Erythematosus CLAUDE BACHMEYER M.D., CLAUDE BACHMEYER M.D. Paris, France e-mail: claude.bachmeyer@tnn.aphp.frSearch for more papers by this authorANNE-MARIE CHESNEAU M.D., ANNE-MARIE CHESNEAU M.D. Paris, France e-mail: claude.bachmeyer@tnn.aphp.frSearch for more papers by this authorCHANTAL LOIRAT M.D., CHANTAL LOIRAT M.D. Paris, France e-mail: claude.bachmeyer@tnn.aphp.frSearch for more papers by this authorDANIELLE ASENSI M.D., DANIELLE ASENSI M.D. Paris, France e-mail: claude.bachmeyer@tnn.aphp.frSearch for more papers by this authorJEAN-MARIE BABINET M.D., JEAN-MARIE BABINET M.D. Paris, France e-mail: claude.bachmeyer@tnn.aphp.frSearch for more papers by this authorLAURENT BLUM M.D., LAURENT BLUM M.D. Paris, France e-mail: claude.bachmeyer@tnn.aphp.frSearch for more papers by this author CLAUDE BACHMEYER M.D., CLAUDE BACHMEYER M.D. Paris, France e-mail: claude.bachmeyer@tnn.aphp.frSearch for more papers by this authorANNE-MARIE CHESNEAU M.D., ANNE-MARIE CHESNEAU M.D. Paris, France e-mail: claude.bachmeyer@tnn.aphp.frSearch for more papers by this authorCHANTAL LOIRAT M.D., CHANTAL LOIRAT M.D. Paris, France e-mail: claude.bachmeyer@tnn.aphp.frSearch for more papers by this authorDANIELLE ASENSI M.D., DANIELLE ASENSI M.D. Paris, France e-mail: claude.bachmeyer@tnn.aphp.frSearch for more papers by this authorJEAN-MARIE BABINET M.D., JEAN-MARIE BABINET M.D. Paris, France e-mail: claude.bachmeyer@tnn.aphp.frSearch for more papers by this authorLAURENT BLUM M.D., LAURENT BLUM M.D. Paris, France e-mail: claude.bachmeyer@tnn.aphp.frSearch for more papers by this author First published: 23 October 2007 https://doi.org/10.1111/j.1525-1470.2007.00538.xCitations: 9Read the full textAboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL Share a linkShare onFacebookTwitterLinked InRedditWechat No abstract is available for this article.Citing Literature Volume24, Issue5September/October 2007Pages 585-586 RelatedInformation

Papulonodular mucinosis in systemic lupus erythematosus

A 41-year-old man with systemic and serological manifestations of systemic lupus erythematosus presented with a diffuse eruption comprising annular plaques. Histopathology revealed diffuse deposition of mucin throughout the dermis, consistent with papulonodular mucinosis. This uncommon entity of unclear pathogenesis has been described in systemic lupus erythematosus, discoid lupus erythematosus, and subacute cutaneous lupus erythematosus.

Type I and type III collagens in cutaneous mucinosis.

Cutaneous mucinoses are a heterogeneous group of diseases characterized by the focal or diffuse dermal deposition of glycosaminoglycans. The histopathologic examination of many cutaneous mucinoses reveals that the collagen fibers are fragmented. We wanted to characterize the type I (COL1) and type III (COL3) collagen distribution in skin biopsy specimens of patients with cutaneous mucinosis. The diagnosis of mucinosis was based on a modification of the classification by Rongioletti and Rebora: four patients had familial papulonodular mucinosis: four had papular mucinosis, one of which was associated with myxedema and one had scleromyxedema; and one had focal mucinosis. We performed anti-type I and type III collagens immunolabeling on frozen sections. Immunofluorescence for COL1 was increased in the superficial dermis of 2/4 familial papulonodular mucinosis, in 5/5 of papular mucinosis, and in scleromyxedema and focal mucinosis cases. The mid-dermis showed intense staining for COL1 at the periphery of collagen bundles and, in three cases of familial papulonodular mucinosis and two cases of papular mucinosis, a lacy appearance. The superficial dermis of familial papulonodular mucinosis specimens and of papular mucinosis + myxedema, scleromyxedema, and focal mucinosis specimens had decreased COL3 staining. The mid-dermis showed a more prominent fibrillar staining at the periphery of the collagen bundles, and two cases of papular mucinosis showed intense labeling for COL3. Both COL1 and COL3 distributions are altered in cutaneous mucinosis. An intense labeling with COL1 is predominantly found in the superficial layer of cutaneous mucinosis. Cases of FTP revealed decreased COL3 reactivity at the superficial layer.

Alopecia Associated with Papulonodular Mucinosis (Tumid) Lesions of Lupus Erythematosus

Nonscarring lesions associated with lupus erythematosus (LE) include the diffuse alopecia associated with acute flares of systemic LE (SLE), frontal alopecia, and alopecia areata. We present a patient with a less often recognized lesion called papulonodular mucinosis or tumid LE who presented with nonscarring patches of alopecia, which clinically resembled alopecia areata. Histopathological examination revealed abundant Alcian blue positive material in the dermis, typical of tumid LE. We believe our patient demonstrates a fourth type of nonscarring alopecia that may occur in patients with LE.

Papulonodular mucinosis associated with systemic lupus erythematosus: Possible mechanisms of increased glycosaminoglycan accumulation

Background: The mechanism for the production of papulonodular mucinosis in patients with lupus erythematosus (LE) is not known. Objective: Our purpose was to determine whether fibroblasts in a patient with LE and papulonodular mucinosis produced more mucin than normal fibroblasts and whether this mucin production could be stimulated by the patient's serum. Methods: Skin fibroblasts from a patient with systemic LE and massive papulonodular mucin deposition, as well as normal fibroblasts, were incubated in the presence of serum from the patient or from a healthy volunteer. The production of glycosaminoglycan by fibroblasts was analyzed. Results: Fibroblasts from the patient produced more glycosaminoglycan than did normal fibroblasts. Glycosaminoglycan production was increased in all cells when incubated in the presence of the patient's serum. Conclusion: Cutaneous mucin deposition in patients with papulonodular LE skin lesions is associated with increased glycosaminoglycan production by dermal fibroblasts. Our preliminary observations suggest glycosaminoglycan production by these fibroblasts appears to be stimulated by a factor, (or factors) in the patient's serum that is yet to be identified.