Abundant Mucin Deposition Research Articles

Inhibitory effect of pentoxifylline on the synthesis and deposition of glycosaminoglycans in hypothyroid skin

The skin manifestations in hypothyroidism are common but their advanced forms do not have adequate therapy. Along with achievement of euthyroidism, thyroid-associated dermatopathy (TAD) needs an inhibition of the proliferation and activation of fibroblasts, and of the glycosaminoglycans synthesis. Pentoxifylline (PTX) exerts multifaceted effects and has shown properties to inhibit certain constitutive activities of dermal fibroblasts. The aim of this study was to determine the histopathological changes and to establish the amount and distribution of mucin in hypothyroid skin, with or without treatment with PTX. Hypothyroid skin showed abundant diffuse mucin deposition in all dermal compartments in comparison with euthyroid skin. After administration of PTX in dose 100 mg/kg daily, there was a significant decreased amount of mucin in the dermis. The improvement of the dermal histological manifestations, in general demonstrated by narrowing between the dermal collagen fibers and by a decrease in mucin deposition, might be explained with dose-dependent PTX inhibition of the synthesis and accumulation of glycosaminoglycans. The histological results of this study confirmed several clinical observations about the beneficial effect of PTX on clinical skin manifestations in hypothyroid state. Therefore, we may propose PTX as a new potential therapeutic agent for TAD treatment. Further studies are needed to fully establish the effect of PTX on dermal fibroblasts after continuous administration.

Histiocytose mucineuse progressive sporadique

Progressive mucinous histiocytosis is a very rare, benign, non-Langerhans' cell histiocytosis limited to the skin. This disorder has been observed solely in women, with the exception of three cases in male patients, and most cases are hereditary. The hereditary forms begin in childhood, with sporadic cases occurring later, and it is characterized by numerous papules of slow progression. The aetiology and mode of genetic transmission remain unclear. We report one sporadic case of progressive mucinous histiocytosis. A 33-year-old woman presented with a 15-year history of multiple small purplish asymptomatic papules on the outside of both legs. There was no history of similar cases in her family. The dermatologists she consulted suggested a diagnosis of lichen planus. A punch biopsy was performed and revealed dermal accumulation of histiocytes with abundant mucin deposition. Seventeen hereditary cases in seven families, all of which concerned women except for three males, and five sporadic cases have been described to date. The clinical and histological presentation is typical. Histopathology shows dermal accumulation of histiocytes with abundant mucin deposition. The ultrastructural aspect (intracytoplasmic phospholipid deposits) and progressive extension suggest a disorder similar to that observed in lysosomal diseases. However, primary proliferation of macrophages in response to an unknown stimulus cannot be ruled out. At present, there is no treatment.

Immunohistochemical Characterization of Large Intestinal Adenocarcinoma in the Rhesus Macaque (Macaca mulatta).

In rhesus macaques, adenocarcinomas of either the ileocecal junction or colon are common spontaneous tumors in aging populations. The macaque tumors have similar gross and histologic characteristics compared with their human counterpart, but little is known regarding the immunohistochemical expression of proteins that are commonly implicated in the pathogenesis of these tumors in humans. We performed a retrospective review of 22 cases of large intestinal carcinoma in the rhesus macaque and evaluated the expression pattern of a panel of potentially prognostically significant proteins identified from human studies. Histologic characteristics of the tumors included abundant mucin deposition, transmural spread, and lymphatic invasion. All rhesus adenocarcinomas displayed altered expression of 1 or more of CD10, β-catenin, sirtuin 1, cytokeratin 17, and p53 compared with age-matched controls. Zymographic analysis of active matrix metalloproteinases 2 and 9 in the serum from 5 animals failed to reveal statistically significant differences between adenocarcinoma cases and controls. Based on the data presented herein, large intestinal carcinomas in the macaque share many histomorphologic and immunohistochemical similarities to large intestinal tumors in humans. Further validation of this animal model is considered important for the development of novel therapeutics and a better understanding of the pathogenesis.

Adult T-Cell Leukemia-Lymphoma Associated With Follicular Mucinosis

Follicular mucinosis is frequently associated with follicular mycosis fungoides, but its association with adult T-cell leukemia-lymphoma (ATLL) is extremely rare. We report a case of a 50-year-old female patient with a history of ATLL, after multiple treatments, with residual/recurrent skin tumors in the forehead and legs. Biopsy of a skin tumor from the forehead revealed a perifollicular and intrafollicular atypical lymphoid infiltrate with abundant mucin deposition. Immunohistochemical stains showed that the atypical cells were positive for CD3, CD4, and CD25. Reverse transcription polymerase chain reaction performed on the tissue sections confirmed the presence of human T-cell leukemia virus in the biopsies of skin tumors. To our knowledge, this is only the third reported case of a follicular mucinosis in the setting of ATLL.

Improvement in Clinical Symptoms of Scleredema Diabeticorum by Frequency-Modulated Electromagnetic Neural Stimulation: A Case Report

Scleredema diabeticorum is a rare complication of diabetes, characterized by diffuse induration of the skin over the neck, shoulders, and upper trunk and arms, progressively leading to limited upper body motility and impaired respiratory function (1). Etiology is unknown, and histopathological findings include thickened dermis with abundant mucin deposition among large collagen bundles. No treatment is currently available. We report a case of scleredema diabeticorum treated with frequency-modulated electromagnetic neural stimulation (FREMS), a transcutaneous electrotherapy that proved effective and safe in the treatment of symptomatic diabetic neuropathy (2). Putative mechanisms of FREMS include enhancement of microvascular blood flow (3) and vasomotor activity mediated by smooth cells (4) and release of vascular endothelial growth factor. In April 2012, a 55-year-old man with type 1 diabetes since age 11 presented complaining of a progressive thickening and hardening of his skin, deterioration in daily life activities …

Comparative analysis of rosacea and cutaneous lupus erythematosus: Histopathologic features, T-cell subsets, and plasmacytoid dendritic cells

Distinction of rosacea and cutaneous lupus erythematosus (LE) can be challenging because of significant clinical and histologic overlap. A controlled study comparing these conditions is lacking. We compared the histologic features, T-cell subsets, and plasmacytoid dendritic cells in rosacea and LE. Biopsy specimens of rosacea (n= 27) and facial LE (n= 30) were retrospectively reviewed and reacted with Alcian blue and periodic acid-Schiff stains, and CD4, CD8, CD25, and CD123 immunostains. LE demonstrates a lower CD4:CD8 ratio (1.74 vs 2.80, P= .0064), fewer CD4(+)CD25(+) regulatory Tcells (13% vs 31%, P < .0001), and more CD123(+) plasmacytoid dendritic cells (18% vs 6%, P= .0137) than rosacea. The plasmacytoid dendritic cells in LE are more likely to form clusters (P= .0137) and comprise at least 20% of the infiltrate (P= .0340). Also associated with LE are follicular plugging (P= .0039), perineural lymphocytic infiltrate (P= .0211), abundant mucin deposition (P= .0031), and conspicuous basement membrane thickening (P= .0073), whereas Demodex infestation (P= .0064) and sebaceous hyperplasia (P= .0029) are significantly associated with rosacea. Although statistically significant, the immunophenotypic differences are rather small and limited for routine use. The infiltrates in rosacea and LE differ immunophenotypically, and may aid in their distinction in addition to conventional histologic examination.

Allopurinol induced granuloma annulare in a patient of lepromatous leprosy

Granuloma annulare (GA) is a benign, inflammatory dermatosis involving dermis or subcutis with unknown etiology and poorly understood pathology. GA has characteristic histological features of necrobiosis, granuloma formation and abundant mucin deposition. Various predisposing factors, systemic diseases and drugs have been implicated in the etiology. We hereby describe a case of 70 year old male who was a known case of lepromatous leprosy, taking multidrug therapy for 6 months presented with multiple discrete, annular, firm, non tender, smooth surfaced skin colored papular lesions ranging in size from 0.5-1 cm over back for 1 month. There was past history of intake of allopurinol for hyperuricemia which was started 1 year back. There was history of similar lesions 6 months back which healed within 1 month of stopping allopurinol and he started taking the drug for the past 4 months on his own without any medical advice. Histopathological examination showed superficial and deep perivascular lymphocytic infiltrate with numerous histiocytes scattered in the intersititum of reticular dermis and abundant mucin in between the histiocytes. Allopurinol was implicated as an etiological agent and dramatic improvement was seen after stopping the drug for a period of 4 weeks. Naranjo's algorithm showed a probable association with a score of 6. Thus the final diagnosis of allopurinol induced generalised interstitial granuolma annulare was made. Patient was advised to continue antileprotic drugs, low purine diet and avoid allopurinol intake.

Linear lupus panniculitis of the scalp presenting as alopecia along Blaschko’s lines: A distinct variant of lupus panniculitis in East Asians?

A 32-year-old Taiwanese man presented to our clinic with a 6-month history of linear hair loss in a wavy and curved pattern across the parietal and occipital scalp, resembling the distribution of Blaschko's lines. Physical examination showed interfollicular erythema and follicular plugging without skin atrophy or sclerotic change. Histopathology revealed a lymphoplasmacytic infiltrate in the perifollicular dermis and subcutis with abundant mucin deposition, consistent with the diagnosis of lupus panniculitis. Treatment with hydroxychloroquine and local steroid injection resulted in complete hair regrowth but recurrence was noted. Linear lupus panniculitis of the scalp presenting as alopecia along Blaschko's lines had so far been reported exclusively in five East Asians. Apart from classical lupus panniculitis, it had distinct clinical and histopathological features such as younger age of onset, male predominance, reversible clinical course without scarring, fewer associations with systemic lupus erythematosus, exclusive involvement of scalp, sparse inflammatory infiltration, abundant mucin deposition, higher degree of hyaline fat degeneration and negative results of immunofluorescent studies. Therefore, we propose linear lupus panniculitis of the scalp to be a distinct variant of lupus panniculitis and should be included in the differential diagnosis for focal or linear alopecia, especially in East Asians.

Mucin-containing Rokitansky-Aschoff Sinuses With Extracellular Mucin Deposits Simulating Mucinous Carcinoma of the Gallbladder

Rokitansky-Aschoff sinuses (R-As) are epithelial invaginations that extend down the gallbladder wall through the smooth muscle gaps and by this pathway they reach the subserosal connective tissue. We report here 5 cases of mucin-containing R-As that ruptured, and as a result, extracellular mucin escaped into the adjacent stroma. They were compared with 10 mucinous carcinomas of the gallbladder. Three cases of mucin-containing R-As accompanied by abundant extracellular mucin deposits with epithelial strips, glands and papillary structures were misinterpreted as mucinous carcinomas. Because of this, the patients were subjected to a second unnecessary radical surgical procedure. In the remaining 2 cases, the extracellular mucin associated with R-As was acellular and regarded as focal. Three patients were men and 2 women whose ages ranged from 2 to 71 years (average age 52 y). The youngest patient was a 2-year-old boy in whom the mucin-containing R-As were discovered incidentally by magnetic resonance imaging. He was later diagnosed with metachromatic leukodystrophy. The remaining 4 patients complained of right upper quadrant pain and had a thickened gallbladder wall identified by computed tomography and magnetic resonance imaging. There were gallstones in 3 patients. The R-As located in the lamina propria contained mucin but were not cystically dilated whereas those located in the subserosa were mucin-filled and often cystically dilated. Detached fragments of biliary epithelium, small glands, and papillary structures lacking cytologic atypia and mitotic figures were identified in the abundant mucin deposits located in the subserosa of 3 cases. The overlying surface gallbladder epithelium exhibited papillary hyperplasia with focal intestinal metaplasia in 2 patients, one of which had metachromatic leukodystrophy. The lack of reactivity for carcinoembryonic antigen and p53 and the low proliferative activity as measured by MIB-1 labeling index provided additional support to the benign nature of the lesion. Clues to separate mucin-containing R-As from mucinous carcinoma are provided. All 5 patients with mucin-containing R-As are disease free 8 months to 7 years after surgery (median follow-up of 39 mo).

Lupus erythematosus tumidus is a separate subtype of cutaneous lupus erythematosus

Background Lupus erythematosus tumidus (LET) is a rare disease which was first described in 1909 but has not always been considered as a separate entity of cutaneous lupus erythematosus (CLE) in the international literature. Objectives To compare characteristic features of different subtypes of CLE and to analyse whether LET can be distinguished as a separate entity in the classification system of the disease. Methods The study involved 44 patients with CLE, including 24 patients with LET, 12 with discoid lupus erythematosus (DLE) and eight with subacute CLE (SCLE), from two centres in Germany. A core set questionnaire and an SPSS database were designed to enable a consistent statistical analysis. Results Location of skin lesions did not differ significantly between the CLE subtypes; however, the activity score was significantly lower in LET than in DLE (P < 0.01), and the damage score was significantly lower in LET than in SCLE (P < 0.01) and DLE (P < 0.01). Photosensitivity and antinuclear antibodies were confirmed to be different in LET compared with SCLE and DLE but without statistical significance. Moreover, histological analysis of skin biopsy specimens showed that abundant mucin deposition is significantly more present in LET compared with SCLE (P < 0.01) and DLE (P < 0.01) while prominent interface dermatitis and alteration of hair follicles were absent in LET. Conclusions Several significant differences were found between LET and other subtypes of CLE with regard to clinical, histological and laboratory parameters. These data strongly indicate that LET should be defined as a separate entity in the classification of CLE.

Mucinous Eccrine Nevus: A Case Report and Literature Review

Mucinous eccrine nevus (MEN) is a rare variant of eccrine nevus, characterized by a proliferation of normal eccrine structure surrounded by mucin deposits. We report herein the eighth case of mucinous eccrine nevus in the literature, with abundant mucin deposits not only in the stroma surrounding the eccrine glands but also in the superficial dermis. The literature is reviewed.

Linear morphea with secondary mucinosis

Secondary mucin deposition in the skin is a common feature of lupus erythematosus and dermatomyositis. In scleroderma, it occurs uncommonly or in small amount. We describe a 7-year-old boy with progressive, linear, bound-down plaques involving the thighs, lower abdomen and back with no systemic involvement. Histopathology showed features of scleroderma with abundant mucin deposition in the reticular dermis. This report highlights excessive mucin deposition in lesions of morphea.

Sclerosing ‘Mucinous’ Blue Nevus: A Clinical Simulator of Dermatofibroma

Abundant mucin deposition is an unusual finding for melanocytic lesions. This notion, however, should be toned down since mucin may be present in distinct melanocytic lesions. Indeed, following the first description of mucin depositions in neural differentiated naevi by Maize and Ackerman in 1987 1 this phenomenon was repeatedly observed.2,3,4,5 Especially in blue naevi mucin deposition is a peculiar observation. For blue naevi 5 variants are distinguished to avoid the misdiagnosis of melanoma with stromal mucin deposition6: (i) neural nevus, (ii) myxoid intradermal nevus, (iii) alveolar cellular blue nevus, (iiii) ancient melanocytic nevus, (v) sclerosing blue nevus. In 2003, Rongioletti and Innocenzi described the first two examples of sclerosing blue naevi with an abundant mucinous stroma. Herein, we describe the third case of this rare entity. It should be noted, that particularly this uncommon variant of a blue naevus is easily mistaken as desmoplastic-neurotropic melanoma in which the present stromal deposition of mucin is a more typical finding. A specimen from the right lower leg of a 53-year old man was referred to our dermatopathology department with the clinical diagnosis of a dermatofibroma. Clinically, the tumor appeared as a brown lesion with a regular and sharp border. The histopathological work up revealed a symmetrical, well circumscribed papule; the fibro-mucinous stroma contained spindle-shaped and dendritic melanocytes, which sometimes were arranged in whorls (Fig. 1A). Orderly arranged melanocytes in the periphery were characterized by pigmentation, as confirmed by melanin-staining. Melanophages were present as clearly seen in the reaction against CD68. Alcian blue staining confirmed the mucin deposition within the tumor and excluded an intradermal component (Fig. 1B). Immunohistochemical staining with Mart-1 (Fig. 1D) and S-100 confirmed both the nature and the distribution of the melanocytes. Notably no reaction was found against MIB-1 excluding a high proliferation rate (Fig. 1C). Thus all criteria for the diagnosis of a sclerosing mucinous blue naevus were fulfilled, and the differential diagnosis of a malignant melanoma was excluded. Moreover, the clinical diagnosis of a dermatofibroma was ruled out by lack of either epidermal changes or typical infiltrating pattern. Hemosiderin deposits which mimic melanin were excluded by special staining. Figure 1. (A) Fibro-mucinous stroma with spindle-shaped and dendritic melanocytes, which sometimes were arranged in whorls. Melanophages were present (20x Hematoxylin and Eosin). (B) Alcian blue staining confirmed the mucin deposition within the tumor (20x Mucin). ... The cause of mucin deposition in melanocytic lesions remains elusive. It was attributed to a partial involution of the respective lesions. Interestingly, the dermoscopy of the presented sclerotic blue nevus revealed a hypopigmented center; a notion which has been described before.7 In line with this hypothesis it is well established that cellularity of naevi decrease which age, whereupon melanocytes are replaced by fibrous matrix, mucin and sometimes fat.8 With the present case report we like to raise the awareness for this rare entity because of the momentous differential diagnosis. Further reports of such cases would help to learn more about its clinical, dermoscopic and histological features.

Signet-Ring Cell Adenocarcinoma of Sinonasal Tract: An Immunohistochemical Study of the Mucins Profile

Adenocarcinomas of the sinonasal tract are classified into 4 categories: salivary-type, intestinal-type, nonintestinal-type, and metastatic. Signet-ring cell carcinoma is the rarest form of intestinal-type adenocarcinoma. Only isolated cases have been reported in the literature. To evaluate clinical attributes, morphology, and immunohistochemistry in signet-ring cell carcinoma of the sinonasal tract. Seventy-three cases of primary sinonasal adenocarcinomas were retrieved from the files. Only 5 signet-ring cell adenocarcinomas (tumors composed of more than 90% signet-ring cells) were identified. In all cases, clinical data and histologic slides were available and were reviewed. Consecutive tissue sections were immunostained with monoclonal antibodies against MUC2, MUC5AC, MUC5B, MUC6, p53 protein, and MIB-1. Four of our 5 patients were woodworkers. They were treated by surgical excision and radiotherapy. Patient follow-up ranged from 11 to 84 months. Three patients presented an unfavorable evolution and died of metastatic spread. Microscopically, the tumors produced abundant mucin deposits, which accumulated in large extracellular pools. Tumor cells were predominantly signet-ring cells floating in the pools of mucin. The mucins profile was MUC2+, MUC5AC+, MUC5B+, and MUC6-. Eighty percent of cells were immunostained by p53 protein antibody and 60% cells with MIB-1 antibody. The mucin profile is similar to the profile described in digestive tract adenocarcinoma. It is not useful to differentiate between metastatic adenocarcinoma and primary intestinal-type sinonasal adenocarcinoma. Clinical data and immunochemistry with p53 protein and MIB-1 confirm that sinonasal signet-ring cell carcinoma is a high-grade and aggressive tumor.

Nephrogenic Systemic Fibrosis/Nephrogenic Fibrosing Dermopathy: Clinical Aspects

Nephrogenic systemic fibrosis, previously known as nephrogenic fibrosing dermopathy, is a novel fibrosing disorder characterized by prominent cutaneous and systemic fibrosis in patients with renal failure. Although initially descriptions of this disorder was described to be purely cutaneous, numerous subsequent reports have shown remarkable systemic involvement with fibrosis of numerous internal organs, including striated muscles, myocardium, microvasculature, lungs, and others, accompanied by elevation of systemic inflammation markers, such as erythrocyte sedimentation rate and C-reactive protein. The cutaneous manifestations are characterized by erythematous to browny skin induration with a peau d'orange appearance, plaques, nodules, and papules. Histopathologically, there is dermal and subdermal thickening with abundant collagen fibers and mucin deposits and a striking increase in the number of spindle-shaped fibroblastic cells. The disorder is clinically distinct from scleromyxedema, systemic sclerosis, and other cutaneous fibrosing syndromes. The cause is unknown, and currently there is no uniformly effective treatment.

Two sporadic cases of adult‐onset progressive mucinous histiocytosis

Progressive mucinous histiocytosis is a rare, benign, non-Langerhans' cell histiocytosis limited to the skin. Ten cases--all women--in four families and one sporadic case have been described in the literature. The disorder usually begins in childhood and progresses slowly. We report two sporadic cases of adult-onset progressive mucinous histiocytosis in unrelated African-American women, aged 48 and 55 years, respectively, who developed red-brown and flesh-coloured, asymptomatic papules on the face, the arms and the legs without truncal, mucosal or visceral involvement. The lesions showed no spontaneous regression. Both patients lacked associated systemic symptoms, including polyuria, polydipsia or seizures. There was no underlying hyperlipidaemia, paraproteinaemia or lymphoproliferative disease. No family history of similar lesions could be identified. Light microscopy revealed dermal proliferation of spindle-shaped histiocytes with abundant mucin deposition. Electron microscopy demonstrated a high number of myelin figures or zebra bodies in the cytoplasm of histiocytes. On immunohistochemistry, positive staining with macrophage markers--CD68, HAM56 and lysozyme--and factor XIIIa, a transglutaminase present in dermal dendrocytes, and negative staining with Langerhans' cell markers--CD1a and S100--and CD34, a marker present in dermal dendritic cells derived from uncommitted mesenchymal cells, were observed.

Fatal subcutaneous panniculitis‐like T‐cell lymphoma with interface change and dermal mucin, a dead ringer for lupus erythematosus

We report a 48-year-old man who presented with ulcerated plaques and nodules of the lower extremities. Skin biopsies revealed a dense lymphocytic infiltrate involving the dermis and the subcutis in a lobular and septal pattern. No overt cytological atypia was present. Notably, several features resembling lupus erythematosus were present, including vacuolar interface change and abundant dermal mucin deposition. The patient developed pulmonary nodules, and a lung biopsy showed a perivascular and interstitial lymphoid infiltrate without overt atypia. The cutaneous and pulmonary lymphoid infiltrates showed similar immunohistochemical profiles: CD3(+) CD4(-) CD8(+/-) CD56(+). Monoclonal rearrangements of the T-cell receptor gamma gene with similar migration patterns were identified from both locations. The patient developed fatal hemophagocytic syndrome, involving liver, spleen, lymph nodes, and bone marrow. This case is one amongst the rare reports of subcutaneous panniculitis-like T-cell lymphoma with systemic involvement.

Progressive Mucinous Histiocytosis

Progressive mucinous histiocytosis is a rare, benign, non‐Langerhans cell histiocytosis limited to the skin. A total of ten cases, all women, in four families, and one sporadic case have been described in the English literature. The disorder usually begins in childhood and progresses slowly. We report two sporadic cases of adult‐onset progressive mucinous histiocytosis in unrelated African American women, age 48 and 55 years old respectively, who developed red‐brown and flesh‐colored, asymptomatic papules on the face, arms and legs without truncal, mucosal or visceral involvement. The lesions show no spontaneous regression. Both patients lack associated systemic symptoms including polyuria, polydipsia or seizures. There is no underlying hyperlipidemia, paraproteinemia or lymphoproliferative disease. No family history of similar lesions can be identified. Light microscopy reveals dermal proliferation of spindle‐shaped histiocytes with abundant mucin deposition. Electron microscopy demonstrates a high number of myelin‐figures or zebra bodies in the cytoplasm of histiocytes. On immunohistochemistry, positive staining with macrophage markers CD68, HAM56 and lysozyme, and Factor XIIIa, a transglutaminase present in dermal dendrocytes, and negative staining with Langerhans cell markers CD1a and S100, and CD34, a marker present in dermal dendritic cells derived from uncommitted mesenchymal cells, are observed.

Fatal Subcutaneous Panniculitis‐Like T‐Cell Lymphoma (Sptcl) with Interface Change and Dermal Mucin, A Dead‐Ringer for Lupus Erythematosus

We report a 48‐year‐old male who presented with ulcerated plaques and nodules of the lower extremities. Skin biopsies revealed a dense lymphocytic infiltrate involving the dermis and the subcutis in a lobular and septal pattern. No overt cytological atypia was present. Notably, several features resembling lupus erythematosus were present, including vacuolar interface change and abundant dermal mucin deposition. The patient developed pulmonary nodules, and a lung biopsy showed a perivascular and interstitial lymphoid infiltrate without overt atypia. The cutaneous and pulmonary lymphoid infiltrates showed similar immunohistochemical profiles: CD3+CD4−CD8+/−CD56+. Monoclonal rearrangements of T‐cell receptor gamma gene with similar migration patterns were identified from both locations. The patient developed fatal hemophagocytic syndrome, involving liver, spleen, lymph nodes, and bone marrow. This case is amongst rare reports of subcutaneous panniculitis‐like T‐cell Lymphoma (SPTCL) with systemic involvement.

Plaquelike Cutaneous Lupus Mucinosis

To the Editor.— The concept of nodular cutaneous mucinosis with lupus erythematosus (LE) has been proposed as a distinct variant of cutaneous LE because of its specific clinical features and the histologic evidence of mucin deposition. 1-5 Recently, we examined two patients with systemic LE (SLE) in whom large indurated plaquelike lesions developed that measured 10 to 20 cm in diameter on the trunk. These lesions also had a histologically abundant mucin deposition throughout the dermis. The characteristic large plaquelike skin lesions reported here should be classified as a new clinical variant of cutaneous LE. Report of Cases.—Case 1.— A 60-year-old Japanese man with nephrotic syndrome associated with SLE was referred to our department with a large plaquelike skin lesion with a hard cutaneous induration about 10 to 20 cm in diameter on his lower back (Fig 1). The onset of the lesion was unknown and was not preceded by