Localized Papular Mucinosis With IgA Nephropathy A Case Report

Department of Dermatovenereology, West China Hospital, Sichuan University, 37 Guo Xue Xiang, Wang Hou District, Chengdu, People's Republic of China, 610041.
Archives of dermatology (Impact Factor: 4.79). 05/2011; 147(5):599-602. DOI: 10.1001/archdermatol.2011.110
Source: PubMed


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.

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    ABSTRACT: Scleromyxedema is a generalized skin disease mostly associated with monoclonal gammopathy. In its chronic course, it can lead to systemic disorders related to mucin deposits in organs. We describe here specific lymph node involvement, hitherto not reported in scleromyxedema. A 68-year-old man with a 1-year history of micropapular eruption and skin sclerosis involving the neck, trunk, hands, and face was diagnosed with scleromyxedema associated with IgG kappa monoclonal gammopathy. Enlarged mediastinal lymph nodes found on thoracic X-ray and computed tomography scan were hypermetabolic on positron emission tomography. Lymph node biopsy showed partial nodal infiltration by numerous fibroblasts surrounded by mucin and collagen deposits, the same being observed on the skin biopsy. Lymph node and skin lesions both improved after intravenous immunoglobulin and corticosteroid treatment. Lymph node involvement in scleromyxedema should be considered in the etiological diagnosis of hypermetabolic, enlarged lymph nodes, especially if monoclonal gammopathy is associated.
    Archiv für Pathologische Anatomie und Physiologie und für Klinische Medicin 05/2013; 462(6). DOI:10.1007/s00428-013-1424-2 · 2.65 Impact Factor

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