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lupus profundus

Shricharith Shetty, Raghavendra Rao, R Ranjini Kudva, Kumudhini Subramanian
Alopecia areata (AA) over scalp is known to present in various shapes and extents of hair loss. Typically it presents as circumscribed patches of alopecia with underlying skin remaining normal. We describe a rare variant of AA presenting in linear band-like form. Only four cases of linear alopecia have been reported in medical literature till today, all four being diagnosed as lupus erythematosus profundus.
July 2016: International Journal of Trichology
Toshihiro Hosokawa, Ryo Oda, Shogo Toyama, Daigo Taniguchi, Daisaku Tokunaga, Hiroyoshi Fujiwara, Toshikazu Kubo
INTRODUCTION: Spontaneous flexor tendon rupture is usually caused by trauma, systemic diseases, or carpal bone and joint disorders. Here we report a case of spontaneous flexor tendon rupture occurring in a systemic lupus erythematosus (SLE) patient following nonunion of the hamate hook after an insufficiency fracture, and which was also associated with tendon degeneration caused by SLE. CASE PRESENTATION: A 57-year-old woman was diagnosed with SLE 22 years ago and being treated with oral prednisolone...
2016: International Journal of Surgery Case Reports
Mary Vineetha, Seena Palakkal, K Sobhanakumari, M I Celine
Lupus mastitis is an uncommon presentation of lupus erythematosus profundus (LE profundus), characterized by inflammation of the subcutaneous fat. LE profundus occurring as initial manifestation of LE is rare. We report a case where the patient presented with lupus mastitis and years later, she developed disseminated discoid LE (DLE) and polyarteritis nodosa (PAN). PAN and DLE are connective tissue diseases with different etiologies, clinical, immunological, and histopathological features. One connective tissue disease evolving into another is rare, and the reasons remain unexplained...
March 2016: Indian Journal of Dermatology
Carolyn J Shiau, Marie S Abi Daoud, Se Mang Wong, Richard I Crawford
The diagnosis of panniculitis is a relatively rare occurrence for many practising pathologists. The smaller subset of lymphocyte-predominant panniculitis is further complicated by the diagnostic consideration of T cell lymphoma involving the subcutaneous tissue, mimicking inflammatory causes of panniculitis. Accurate classification of the panniculitis is crucial to direct clinical management as treatment options may vary from non-medical therapy to immunosuppressive agents to aggressive chemotherapy. Many diseases show significant overlap in clinical and histological features, making the process of determining a specific diagnosis very challenging...
December 2015: Journal of Clinical Pathology
Hisashi Nomura, Shohei Egami, Tomoaki Yokoyama, Makoto Sugiura
A lot of diseases, including lupus profundus, morphea, lipodystrophy, and Parry-Romberg syndrome, may manifest progressive hemifacial atrophy. These diseases usually progress slowly and rapid progression of atrophy is extremely rare. We report a case of elderly-onset rapid progression of hemifacial atrophy only in three weeks. Our case did not meet variable differential diagnoses. We discuss the clinical character of the patient against the past of literature and suppose it may be a new clinical entity.
2015: Case Reports in Dermatological Medicine
Claudia Ileana Sáenz-Corral, María Elisa Vega-Memíje, Eduwiges Martínez-Luna, Juan Carlos Cuevas-González, Alma Angélica Rodríguez-Carreón, Juan José Bollain-Y-Goytia de la Rosa, Felipe de Jesús Torres Del Muro, Esperanza Avalos-Díaz
INTRODUCTION: Lupus erythematosus is a multisystemic disease that is characterized by autoantibody production and immune complex deposition in such tissues as the mucosa, joints, the central nervous system, and skin. Cutaneous lupus erythematosus is categorized as acute, subacute, and chronic. Chronic cutaneous lupus erythematosus comprises discoid lupus erythematosus (DLE) and lupus profundus (LP). AIM: To analyze the expression of proapoptotic molecules in patients with lupus erythematosus discoid and lupus profundus...
2015: International Journal of Clinical and Experimental Pathology
L Arrico, A Abbouda, I Abicca, R Malagola
Ocular complications associated with cutaneous lupus erythematosus (CLE) are less studied compared with those ones associated with systemic lupus erythematosus (SLE). The main ocular sites involved in patients affected by discoid lupus erythematosus (DLE) are eyelids followed by orbit and periorbit, the least being cornea. The most common complications are blepharitis usually affecting the lower lid and associated with some type of lid lesion such as plaque or erythematosus patches and madarosis. Few cases with LE profundus (LEP) and ocular complications are reported, but they are associated with orbital inflammatory syndrome and severe complications...
2015: Journal of Ophthalmology
Aneta Szczerkowska-Dobosz, Barbara Olszewska, Małgorzata Lemańska, Dorota Purzycka-Bohdan, Roman Nowicki
Facial lipoatrophy refers to the loss of subcutaneous fat tissue presenting by flattening or indentation of convex contour of the face. Facial lipoatrophy is a feature of the normal ageing process. It may be also a manifestation of chronic diseases, most frequently it affects HIV-infected individuals treated with highly active antiretroviral therapy (HAART) and may constitute a complication of connective tissue diseases, like lupus erythematosus profundus or morphea. Early recognition and treatment of the active stage of connective tissue diseases is of essential significance in prevention of subsequent scarring and atrophy lesions...
April 2015: Postȩpy Dermatologii i Alergologii
M Ogawa, Y Muro, K Sugiura, A Sakakibara, M Akiyama
Lupus erythematosus profundus (LEP), which is a variant of chronic cutaneous lupus erythematosus (CLE), is seen in approximately 2∼3% of CLE patients, and only 10% to 20% of LEP patients present with systemic LE (SLE). LEP shows subcutaneous nodules with or without discoid LE (DLE). Linear LEP, a very rare variant of LEP, was first reported in 1991 in Japanese and in 1998 in English. Since LEP sometimes leaves skin depressions or scars as a result of atrophy of adipose tissue, early and adequate treatments are necessary...
October 2015: Lupus
Francesca Bosisio, Sebastiana Boi, Valentina Caputo, Concetta Chiarelli, Fergus Oliver, Roberto Ricci, Lorenzo Cerroni
Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is characterized by panniculitic infiltrates that may be difficult to distinguish from inflammatory disorders, particularly lupus erythematosus profundus (LEP). We report on 11 patients (M:F=5:6; median age: 49 y; range: 20 to 75 y) presenting with lobular panniculitic infiltrates showing histopathologic features of both SPTCL and LEP in different parts of the same biopsy specimen. The areas showing aspects of SPTCL revealed dense infiltrates of small and medium-sized, atypical α/β T-cytotoxic lymphocytes with focal rimming of the adipocytes and high proliferation...
February 2015: American Journal of Surgical Pathology
Yukari Kawakami, Makoto Kunisada, Chikako Nishigori
No abstract text is available yet for this article.
November 2014: Acta Dermato-venereologica
Azusa Miyashita, Satoshi Fukushima, Takamitsu Makino, Yuichiro Yoshino, Junji Yamashita, Noritoshi Honda, Jun Aoi, Asako Ichihara, Masatoshi Jinnin, Yuji Inoue, Hironobu Ihn
Lupus erythematosus profundus is a rare inflammatory disorder of subcutaneous fat in patients with lupus ery-thematosus. Previous reports suggested that plasmacytoid dendritic cells, which expressed CD123 and CD303 antigens, play a central proinflammatory role in the patho-genesis of lupus erythematosus. To find the factors that determine the response to treatment, we analysed 23 skin specimens from the patients with lupus erythematosus profundus. The patients with considerable lymphocytic inflammation with high percentages of CD123+ cells in dermis and subcutaneous fat significantly responded to the systemic corticosteroid therapies...
September 2014: Acta Dermato-venereologica
L G Okon, V P Werth
Cutaneous lupus erythematosus (CLE) encompasses a wide range of dermatologic manifestations, which may or may not be associated with the development of systemic disease. Cutaneous lupus is divided into several sub-types, including acute CLE (ACLE), sub-acute CLE (SCLE) and chronic CLE (CCLE). CCLE includes discoid lupus erythematosus (DLE), LE profundus (LEP), chilblain cutaneous lupus and lupus tumidus. The diagnosis of these diseases requires proper classification of the sub-type, through a combination of physical examination, laboratory studies, histology, antibody serology and occasionally direct immunofluorescence, while ensuring to exclude systemic disease...
June 2013: Best Practice & Research. Clinical Rheumatology
Mitsuaki Ishida, Muneo Iwai, Keiko Yoshida, Akiko Kagotani, Keiko Hodohara, Hidetoshi Okabe
Lupus erythematosus (LE) can cause various cutaneous lesions including panniculitis (LE profundus), but salivary gland involvement has been extremely rare in patients with LE. Herein, we report the first documented case of systemic LE with prominent mucoid degeneration and lymphoplasmacytic infiltration in the parotid gland. A 38-year-old Japanese male with histories of autoimmune hemolytic anemia and systemic LE presented with a swelling of the bilateral cervical region. A physical examination revealed a swelling of the bilateral parotid gland and erythema of the right cheek...
2013: International Journal of Clinical and Experimental Pathology
Fátima Moreno-Suárez, Agueda Pulpillo-Ruiz
Lupus erythematosus panniculitis (LEP) or lupus profundus (LP) is a clinical variant of lupus eryhematosus that involves the deep dermis and the subcutaneous fat and is associated with tender subcutaneous nodules or plaques, with occasional ulceration, atrophy, and scarring. The management of this entity can be difficult because of the lack of response to conventional treatments, such as systemic steroids and antimalarials. The two patients of this study presented LP refractory to several therapies that demonstrated a remarkable improve to the infusion of the anti-CD20 monoclonal antibody, rituximab at a dosage of 375 mg/m²/week...
September 2013: Dermatologic Therapy
Katherine L Baquerizo, Daniel J Teague, Lindsay C Strowd, Joseph L Jorizzo, Omar P Sangueza
No abstract text is available yet for this article.
July 2014: American Journal of Dermatopathology
David P Arps, Rajiv M Patel
Lupus profundus is a rare manifestation of cutaneous lupus erythematosus, seen in 1% to 3% of patients. It most commonly presents in association with classic chronic cutaneous lesions of discoid lupus erythematosus; however, such lesions, as well as a clinical history of lupus erythematosus, may be lacking. The differential diagnosis for lymphocytic lobular panniculitides is broad; however, the consideration of subcutaneous panniculitis-like T-cell lymphoma is most critical. Recently, there have been several reports emphasizing the overlapping histomorphologic features between lupus profundus and subcutaneous panniculitis-like T-cell lymphoma...
September 2013: Archives of Pathology & Laboratory Medicine
Rahul Jayaram, Michelle C de Souza, Mehmet Manisali
Unilateral parotid swelling or mass in the lupus erythematosus profundus person is a rare or under reported clinical scenario in existing literature. This is a case of a 49-year-old man with such presentation for whom medical management of the underlying condition led to the resolution of the swelling. Lupus erythematosus profundus must be considered as a differential diagnosis of unexplained parotid swelling to prevent incorrect diagnosis, unnecessary surgical intervention (eg, parotidectomy) and the morbidity that comes with it (eg, facial nerve weakness, Frey's syndrome etc)...
2013: BMJ Case Reports
Lin Yi, Shi Qun, Zheng Wenjie, Zhang Wen, Li Jian, Zhao Yan, Zhang Fengchun
This study aims to investigate the association between subcutaneous panniculitis-like T-cell lymphoma (SPTCL) or cutaneous gamma/delta T-cell lymphoma (CGDTCL) and a variety of manifestations that mimic autoimmune disorders. A retrospective chart review was made for 11 patients who were initially diagnosed as autoimmune diseases but finally turned out to be SPTCL or CGDTCL. Eleven patients were initially diagnosed with erythema nodosum, nodular panniculitis, lupus erythematosus profundus, systemic vasculitis, dermatomyositis, or pyoderma gangrenosum...
August 2013: Clinical Rheumatology
Luigi Valdatta, Mario Cherubino, Federico Tamborini, Igor Pellegatta, Francesca Maggiulli
Facial lipoatrophy is one of the most difficult complication in the patients with lupus profundus. In this paper, we present a case of a 55-year-old woman affected by lupus profundus, with a grade V lipoatrophy, treated with lipofilling technique. No complications were observed and results at 12 months were stable, natural, and symmetric.
2012: Case Reports in Dermatological Medicine
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