Search

A young woman presented with panniculitis, fever and blood cytopenias, later going on to develop hemophagocytic lymphohistiocytosis. Further evaluation revealed the presence of subcutaneous panniculitis like T-cell lymphoma (SPTCL). With this case we present an approach to panniculitis, a commonly encountered skin manifestation with a wide range of differentials. We discuss the close similarity between lupus erythematous panniculitis (LEP) and lymphoma.

Lupus erythematosus panniculitis (LEP) has been reported to be the first manifestation of systemic lupus erythematosus (SLE)1. Face and upper limbs are most commonly involved; however, extensive LEP in the buttock region or breasts is relatively rare, but also reported2,3. Differential diagnosis such as subcutaneous panniculitis-like T cell lymphoma …

Background: Substantial clinicohistologic overlap exists between lupus erythematosus profundus (LEP) and lymphomas involving the subcutis, including subcutaneous panniculitis-like T-cell lymphoma (SPTCL) and primary cutaneous gamma/delta T-cell lymphoma (GDTCL). Unequivocal markers separating the entities are not established. Objectives: To explore the usefulness of interferon alpha (INF-α)-induced protein, myxovirus resistance protein 1 (MxA), in the differential diagnosis of these entities, as studies show that the expression pattern of MxA follows the distribution of the inflammatory infiltrate in cutaneous lupus, while INF- α is not known to operate in lymphoma.Materials and Methods: MxA immunohistochemistry was performed on skin biopsies from 5 patients with a clinical and histological diagnosis of SPTCL, 9 patients with GDTCL and 9 patients with LEP. Results: In SPTCL and GDTCL, MxA was primarily seen in macrophages and generally did not exceed 20% of the infiltrate. In contrast, a significant portion of the subcutaneous infiltrate was positive for MxA in LEP, with 50% of the infiltrate staining on average. A greater number of macrophages and lymphocytes stained with a greater intensity as well (P

Background: Lupus erythematosus panniculitis (LEp) is an uncommon but distinctive subset of lupus erythematosus (LE). It may develop in patients with discoid or systemic LE or may occur as an isolated phenomenon. Case Report: We describe a case of LEp affecting unusual sites: the parotid gland, eyelid, and scalp. Subsequently, the patient progressed to antinuclear antibody–negative systemic LE. Contexte: Le lupus érythémateux compliqué d'une panniculite (LEp) est une forme rare mais particulière de lupus érythémateux (LE). Il peut se manifester dans le contexte du LE discoïde ou du LE disséminé, ou encore n'être qu'un phénomène isolé. Exposé de cas: Il sera question ici d'un cas de LEp touchant des sièges inhabituels: la glande parotide, les paupières, et le cuir chevelu. L'état du patient a évolué, par la suite, vers un LE disséminé négatif à l'égard des anticorps antinucléaires.

Erdheim-Chester disease is a rare non-Langerhans cell histiocytosis of unknown etiology, the commonest sites of involvement being the long bones, skin, orbit, pituitary and retroperitoneum. Breast involvement is rare, with only four reported cases in the English literature. We present a case of a 78-year-old female presenting with bilateral clinically malignant breast masses, with mammographic and ultrasound findings suggestive of locally advanced bilateral breast cancer. Core biopsies from both breasts showed identical features, with a diffuse xanthomatous infiltrate with scattered Touton-type giant cells and a patchy lymphocytic infiltrate. The cells were CD68 positive, and negative for S100, CD1a and a broad panel of cytokeratins. The patient has a background history of cerebrovascular disease with carotid artery stenosis, and subsequently developed rapid restenosis after carotid endarterectomy. With the combined clinical history and classic histological findings in the breast, a diagnosis of Erdheim-Chester disease was made. This is the fifth case report of Erdheim-Chester disease involving the breast, and only the second case with breast lesions as the presenting symptom. Perivascular infiltration is also a rare but recognized presentation of Erdheim-Chester disease. Histiocytic proliferations including ECD can mimic breast carcinoma clinically, radiologically, and histologically, and should be considered in the differential diagnosis of breast mass lesions.

Lupus erythematosus panniculitis (LEP) is an inflammatory disorder of the subcutaneous fat in patients with lupus erythematosus (LE). It is a rare variant of the disease, which occurs approximately in 1%-3% of patients with cutaneous LE. The purpose of this study was to investigate the clinical, histopathologic, immunophenotypical, and molecular profiles of LEP. We performed a retrospective study of 19 biopsy specimens from 17 patients with LEP. We reviewed their clinical data and reexamined the histopathology. Immunophenotyping and molecular studies were done using sections from paraffin-embedded formalin-fixed tissue. The most common clinical manifestation was a depressed patch on upper arm. Patients showed good response to variable treatment modalities, but, generally, relapse of panniculitis was noted when treatment was discontinued. Histopathologically, most specimens revealed lymphoplasmacytic lobular panniculitis with epidermal and dermal changes of LE, hyaline fat necrosis, and lymphoid follicles. Immunohistochemistry showed a mixture of T and B cells in dermis and subcutis with a slight preponderance of T cell. Although the polymerase chain reaction analysis of the T-cell receptor-gamma gene rearrangement showed a polyclonal smear in 89.5% of cases, a small portion of specimens demonstrated monoclonality. LEP is a chronic recurrent disease with characteristic features. Its diagnosis is often challenging, and a precise diagnosis is achievable only upon elaborate clinicopathologic correlation and integrated interpretation of all diagnostic criteria.

Lupus mastitis (LM) is a rare presentation of lupus erythematosus profundus or lupus panniculitis, an unusual and rare clinical variant of lupus erythematosus itself in which the inflammatory reaction occurs primarily in the deep subcutaneous adipose. Although not required for diagnosis, essentially all cases of LM present with systemic or discoid lupus. The etiology is uncertain. Histologically it is defined by a lymphocytic lobular panniculitis and a characteristic hyaline sclerosis of the adipose tissue. Treatment is primarily medical due to exacerbation of disease by surgical intervention. A high index of suspicion, and familiarity of the histologic findings, is therefore required to make an accurate diagnosis and prevent further unwarranted diagnostic procedures. Herein, we provide a literature-based review of the clinical, radiologic, and pathologic findings of LM and its treatment and prognosis with the addition of a case for the literature.

A light and electron microscopical study on membranocystic lesions (MCL) in a case of lupus erythematosus profundus (LEP) is reported. The patient was a 16-year-old female who presented with subcutaneous nodules on both upper arms. The light microscopic features were consistent with LEP, and the result of an immunofluorescence band test supported this diagnosis. A peculiar finding in this case was MCL in the subcutaneous tissue. Ultrastructurally, these were thin membranes without a tubular structure and tortuous thick membranes composed of minute tubules. The lesions were very similar to the fatty tissue changes in membranous lipodystrophy. On the other hand, the basement membranes of the blood vessels were thickened and multilayered, and the lumina were narrowed by endothelial swelling and thickening of the vessel wall. Our findings suggest that the MCL in LEP result from circulatory disturbance of the fat tissue.

We report a patient with multiple sclerosis (MS) who developed subcutaneous nodules on the face, shoulders and extremities while being treated with interferon (IFN)-β-1b. These nodules fluctuated in parallel with myelopathy, and were diagnosed as lupus erythematosus profundus (LEP) based on histopathological findings. The patient showed no relapse of either neurological symptoms or subcutaneous nodules after cessation of IFN-β-1b. This agent can cause induration and necrosis in the sites of injection but also systemic skin lesions such as LEP ascribable to its immunomodulatory effects.

In the newly revised World Health Organization (WHO)-European Organization for Research and Treatment of Cancer (EORTC) consensus classification for cutaneous lymphomas, cutaneous gamma/delta T-cell lymphoma (CGD-TCL) has been included as a provisional entity. This type of lymphomas, when involving the subcutaneous fat, can mimic both clinically and histologically other more indolent conditions, such as subcutaneous panniculitic T-cell lymphomas (SPTCL) and lupus erythematosus profundus (LEP), and multiple biopsies may be needed to obtain a correct diagnosis. A good correlation of the clinical data with the histopathology and immunohistochemistry are required for diagnosis. Herein, we describe a patient whose initial histopathologic findings ressembled LEP but presented an aggressive clinical course. A new biopsy was performed during the follow-up, and a final diagnosis of CGD-TCL was made.

Panniculitis is an inflammation of the subcutaneous fat. Skin biopsy plays a critical role in the differential diagnosis of panniculitis. The most common approach to diagnosis relies on the differentiation between predominantly septal or lobular panniculitis, followed by the distinction between lesions with and without vasculitis. It is also very important to submit a part of the skin biopsy for microbiological analysis and for T-cell clonal expansion if T-cell lymphoma is suspected. Erythema nodosum, the most frequent septal panniculitis, has many causes and in its typical clinical presentation, does not require skin biopsy. In other panniculitis, diagnosis is based on the integration of the clinical and histological data, which renders a deep cutaneous biopsy necessary. Periarteritis nodosa, a septal panniculitis with vasculitis characterized by subcutaneous nodules and livedo racemosa, can be associated with systemic involvement. Nodular thrombophlebitis needs search for associated coagulopathy, Behçet's disease, periarteritis nodosa or Buerger's disease. Lobular panniculitis are classified according to the nature of cells present in the inflammatory infiltrate. If there is a lymphocytic infiltration, lupus panniculitis is difficult to differentiate from subcutaneous panniculitis-like T-cell lymphoma. If there are histiocytes, it can be a sarcoidosis, a cytophagic histiocytic panniculitis or, in newborn, a subcutaneous fat necrosis. Neutrophilic panniculitis needs careful clinic-pathologic correlation. Treatment of panniculitis can be challenging and is based on the histopathological findings. Frequently, the precise cause of panniculitis cannot be established from the outset, so it is important to follow-up patients and not hesitate to repeat the skin biopsy.

Juvenile systemic lupus erythematosus (JSLE) is a rare multisystem autoimmune disease with broad heterogeneity of clinical manifestations. Diagnosing JSLE is often very challenging. This life-threatening, unpredictable, and relapsing disease, which may affect various organ systems, requires interdisciplinary, lifelong care. Here, we report the case of a 13-year-old patient with JSLE suffering from recurrent arthralgia, lupus panniculitis, and rashes that were successfully treated with hydroxychloroquine and prednisolone.

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. Material and methods: Descriptive study, the study groups comprised 10 cases of LP and 10 cases of DLE, and a control. Skin samples of cases and controls were processed for immunohistochemistry and by TUNEL technique. The database and statistical analysis was performed (statistical test X2) SPSS (Chicago, IL, USA). Results: Apoptotic features were broadly distributed along the skin biopsies in epidermal keratinocytes as well as at dermis. By immunohistochemistry the expression of Fas receptor and Fas-L was higher in the skin of lupus patients compared with controls. We also noted differences in Fas-L, -Fas, and -Bax proteins expression intensity in discoid lupus erythematosus patients in the epidermis, and hair follicles. Conclusions: Fas and Fas-L are expressed similarly in LP and DLE.

We report a case of a 66-year-old woman who presented with multiple painless masses in both breasts. Prior bilateral biopsies were diagnosed as Rosai-Dorfman disease (Sinus Histiocytosis with Massive Lymphadenopathy). A recent lumpectomy specimen revealed a gray-white smooth cut surface with a discrete masslike lesion. The histopathology demonstrated a fibrotic breast parenchyma with foci of dense fibrosis and scattered inconspicuous breast epithelium surrounded by lymphocytes that formed aggregates and follicles with germinal centers. The inflammation was in a periductal, perilobular, and perivascular distribution. In addition, an exuberant inflammatory response with histiocytes and fibroblasts was present. This inflammatory response focally surrounded areas of fat necrosis and formed noncaseating granulomas with rare multinucleated giant cells. This process had infiltrative, ill-defined edges and involved the subcutaneous tissues. The overlying epidermis was normal. The final diagnosis was diabetic mastopathy with an exuberant lymphohistiocytic response. The differential diagnosis included Rosai-Dorfman disease, inflammatory myofibroblastic tumor, granulomatous mastitis, sclerosing lipogranulomatous response/sclerosing lipogranuloma, lupus panniculitis, and rheumatoid nodules. Immunohistochemical studies and flow cytometry confirmed the polyclonal nature of the lymphoid infiltrate. After the histologic evaluation, we inquired if the patient had a history of diabetes mellitus, and learned that she did have type 2 noninsulin-dependent diabetes mellitus. In conclusion, we report a case of diabetic mastopathy that presents with bilateral tumorlike masses and an unusual exuberant lymphohistiocytic response with granuloma formation. The pathologist may not be provided with a history of diabetes mellitus, but the characteristic fibrosis, lymphocytic ductitis/lobulitis, and sclerosing lobulitis with perilobular and perivascular lymphocytic infiltrates should provide clues for an accurate diagnosis, even when an exuberant and an unusual lymphohistiocytic response is present. A timely accurate diagnosis can help limit repeat surgeries in this vulnerable group of patients.

Background Lupus erythematosus profundus (lupus panniculitis) is a rare variant of lupus erythematosus with predominant involvement of the subcutaneous tissue. There are only a few reported series of patients with this condition; none in individuals of African ancestry. The aim of the study was to evaluate clinical, histopathological and laboratory findings in black South African patients with lupus profundus. Patients and methods Ten prospectively observed patients were studied. Skin biopsies were performed, and several laboratory tests routinely employed in patients with lupus erythematosus were carried out. All patients were followed up for at least a year and their response to treatment was assessed. Results All patients were females. Mean age at diagnosis was 28.5 years. Face was the most common site of involvement. In five patients, the periorbital edema was the initial manifestation. Only one patient had systemic lupus, and three patients also showed lesions of discoid lupus. In only 30% of the patients were the ANA titers greater than 1 : 80, and positivity in other lupus tests was rare and inconsistent. In general, patients responded to antimalarials and/or systemic corticosteroids. Conclusion African patients with lupus profundus when compared with other series comprising Asian and white patients showed greater frequency of periorbital edema as the initial manifestation. Review of the literature disclosed that this subset of lupus profundus appears to have a more benign course.

Lupus erythematosus panniculitis is a rare disease characterized by deep subcutaneous nodules, most commonly localized on the upper limbs and face. Unique clinical presentations, such as linear configuration or ‘overlap’ forms between lupus erythematosus panniculitis and localized scleroderma have been reported. We present here the clinical characteristics, course and laboratory findings of 2 patients having linear lupus erythematosus panniculitis with localized scleroderma-like changes. The 2 patients (of the 14 patients with lupus erythematosus panniculitis seen by us since 1990) were females with a young age at the onset of disease (median, 25 years). In 1 case, evolution into systemic lupus erythematosus with severe renal involvement occurred whereas the other patient, who had a spontaneous abortion and exhibited anticardiolipin antibodies, should be followed and screened for the emergence of antiphospholipid syndrome. Thus, the clinical behavior of this variant seems to be more aggressive, as compared with the usual course of lupus erythematosus panniculitis, which is considered to be a benign disease, although some reports have suggested that its prognosis is not always favorable. The linear distribution could be the clinical hallmark of such a unique, ‘sclerodermic’ subset of lupus erythematosus panniculitis.

An 11-year-old girl presented with a one-year history of multiple, hard, slightly painful subcutaneous nodules on her right cheek, upper arms, and buttock. Histology of a skin biopsy specimen showed a lobular panniculitis. Laboratory studies revealed positive ANA, anti-double strand DNA, and elevated muscle enzymes. She was diagnosed as having lupus panniculitis. During hydroxychloroquine treatment, erythema over knuckle joints developed. These unusual clinical and laboratory findings of panniculitis associated connective tissue diseases made it difficult to make a precise diagnosis. We report this unusual case of lupus panniculitis with combined features of dermatomyositis resulting in severe lipoatrophy.

A 49-years-old female admitted to our hospital because of skin eruptions on the extremities in 1985. She had suffered from polyarthralgia, skin eruptions since 1983. Physical examinations revealed discoid lesion, central nervous system involvement, and polyarthritis. Laboratory tests revealed leukopenia, thrombocytopenia, and hypocomplementemia. Antinuclear antibody, ant-DNA antibody, LE test were positive. From these findings, she was diagnosed as systemic lupus erythematosus (SLE). She developed lupus peritonitis in 1990 and 1994, which was successfully treated by steroid pulse therapy. Since then, the activity of SLE was in good control under administration of prednisolone 10 mg/day. Chilblain lupus was seen from 1993, Raynaud's phenomenon from 1996, and she further developed subcutaneous induration on her chest, back and upper extremities in 1999. Skin biopsy findings were compatible with lupus panniculitis. In 2002, erythematous patches with scales were observed on her right hand and left knee, and these skin lesions were histologically diagnosed as psoriasis vulgaris. An autoimmune response similar to SLE is speculated in psoriasis. We describe a rare case of SLE with various skin lesions including psoriasis vulgaris.

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. Here, we introduce an LEP case in which magnetic resonance imaging (MRI) was quite effective in evaluating a lesion that had been considered to be linear DLE.

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. In other areas the infiltrate was composed of nodules of B lymphocytes arranged characteristically at the periphery of the fat lobules and in the septa and showing a low proliferation rate. CD123-positive plasmocytoid dendritic cells arranged in small clusters could be observed in 3 cases. Our observation raises an important question concerning the relationship between SPTCL and LEP. A simple chance overlap of 2 unrelated pathologies seems unlikely, as we could observe these unusual features in 11 cases, much more than mere chance would justify. Three other hypotheses may explain the features observed in our patients: (1) these are examples of SPTCL with focal histologic features mimicking those of LEP; (2) these are examples of LEP with focal atypical histologic features mimicking those of SPTCL; (3) SPTCL and LEP may represent 2 ends of a spectrum, a hypothesis that may be supported by the frequent association of the 2 diseases.

Background Pediatric discoid lupus erythematosus (DLE) is rare. The risk of progression to systemic lupus erythematosus (SLE) is uncertain. Objective We sought to determine the risk of progression of pediatric DLE to SLE and to characterize its phenotype. Methods This was a retrospective review of 40 patients with DLE. Results Six (15%) of 40 patients presented with DLE as a manifestation of concurrent SLE. Of the remaining 34, 9 (26%) eventually met SLE criteria and 15 (44%) developed laboratory abnormalities without meeting SLE criteria. Only 10 (29%) maintained skin-limited disease. The average age at progression to SLE was 11 years, with greatest risk in the first year after DLE diagnosis. Most (89%) patients with SLE met diagnostic criteria with mucocutaneous disease (discoid lesions, malar rash, oral and nasal ulcers, photosensitivity), positive antibodies, and/or cytopenia without developing end-organ damage over 5 years of median follow-up. Limitations The study was retrospective. Conclusions In pediatric patients, DLE carries a significant risk of progression to SLE but may predict a milder phenotype of systemic disease. All patients require careful monitoring for SLE, particularly within the first year of diagnosis.

T regulator lymphocytes (Tregs) play a key role in the maintenance of immune tolerance and in the development of autoimmune diseases. Expression of Foxp3 is specific for Tregs, and can be used for the identification of these cells. This study investigated the variations of Tregs Foxp3+ in the kidney biopsies inflammatory infiltrate of different lupus nephritis classes compared to that of ANCA glomerulonephritis, acute tubulointerstitial nephritis and nephroangiosclerosis. Sections of paraffin-embedded tissue have been stained by immunohistochemistry with anti-CD3 and anti-FoxP3 antibodies. We find that the ratio of FoxP3+/CD3+ cells is significantly lower in patients with lupus nephritis class IV and in patients with vasculitides than in the course of nephroangiosclerosis, tubulointerstitial nephritis and lupus nephritis class V. The data presented herein demonstrate a decrease of FoxP3+ Treg cells in the inflammatory infiltrate of lupus nephritis, particularly during the most active phases of lupus nephritis, as observed in the course of a IV class nephritis.

A 71-year-old Japanese woman presented with erythematous plaques on the eyelids and subcutaneous indurations or nodules with or without overlying erythema on the hands, thigh, and leg. She also had oral ulcers, arthralgia and a low grade fever. Laboratory tests revealed an elevated titer of antinuclear antibody, an increased erythrocyte sedimentation rate and anemia. Skin biopsy specimens from the hand and thigh showed lymphocytic perivascular and periappendageal infiltrates and vacuolar alterations at the basement membrane zone of the skin appendages. Moreover, there was a dense lymphocytic infiltrate deep in the dermis with extension into the subcutaneous fat, which was compatible with the diagnosis of lupus erythematosus profundus. Although the biopsy specimen from the eyelid lesion did not contain the subcutaneous fat, the changes in the dermis were essentially the same as those of the hand and thigh. The eruption as well as the other symptoms promptly responded to oral prednisolone.

Introduction: The diagnosis and classification of lymphocytic lobular panniculitis (LLP) has historically proven to be a difficult challenge. We encountered 32 cases of primary LLP which could be categorized as: 1) lupus erythematosus profundus (LEP) (19 patients); 2) an indeterminate group termed indeterminate lymphocytic lobular panniculitis (ILLP) (6 patients); and 3) subcutaneous T-cell lymphoma (SCTCL) (7 patients). Objective We attempted to better define the subtypes of LLP by morphologic, phenotypic and genotypic features and to correlate those features to clinical presentation and outcome. Method: Skin biopsy material was studied by conventional light microscopy, through immunophenotyping performed on sections from paraffin-embedded, formalin-fixed tissue and in some cases on sections of tissue frozen after receipt in physiological (Michel's) medium, and by polymerase chain reaction single-stranded conformational polymorphism analysis to assess for clonality of T-lymphocytes. Clinical features were correlated to histologic, phenotypic, and genotypic analyses. Results: Patients with LEP had a prior diagnosis of LE or overlying skin changes which light microscopically were characteristic of LE. Patients with ILLP had no concurrent or prior history of LE, no systemic symptoms or cytopenias, and a clinical course not suggestive of lymphoma. Cases of SCTCL showed hemophagocytic syndrome and/or lesional progression with demise attributable to the disease. Lesions in all groups showed proximal extremity predilection. Females predominated in the LEP group. The average age of onset was 38, 40 and 55 years in the LEP, ILLP and SCTCL groups, respectively. Cytopenia was seen in 4 LEP patients; 1 also developed fever. In LEP and ILLP, lesions resolved with hydroxychloroquine and/or steroid therapy, with recurrences following cessation of therapy. In the SCTCL group 4 developed hemophagocytic syndrome, 4 died within 2 years of diagnosis, and 3 went into remission following chemotherapy. The LEP and SCTCL groups manifested histological similarities: dense perieccrine and lobular lymphocytic infiltration, lymphoid atypia, histiocytes with ingested debris, eosinophilic necrosis of the fat lobule and thrombosis. The atypical lymphocytes although pleomorphic did not have a cerebriform morphology. The infiltrate in ILLP had a similar cytomorphology and distribution with variable angioinvasion which in all save one case was of lesser intensity and was not associated with significant fat necrosis or vasculitis. Germinal centers, dermal/subcuticular mucin deposition and an atrophying interface dermatitis with hyperkeratosis and follicular plugging were largely confined to the LEP group. Erythrophagocytosis, characteristic of SCTCL, usually indicated a supervening subcuticular lymphoid dyscrasia when encountered in ILLP and LEP. SCTCL showed a selective loss of CD5 expression with or without diminution in CD7 and monoclonal CD3 expression. Of 4 cases studied, 3 showed a CD8 dominant infiltrate while 2 others exhibited CD56 and CD30 positivity, respectively. All cases of SCTCL with amplifiable DNA showed T-cell clonality. Similar molecular and phenotypic features indicative of subcuticular lymphoid dyscrasia were encountered in cases of LEP and ILLP including a reduction in CD5, CD7, and/or monoclonal CD3 expression, a preponderance of CD8 lymphocytes within the subcutaneous fat and T-cell clonality. These cases showed lymphoid atypia with variable erythrophagocytosis. Cases of phenotypically abnormal and/or clonal LEP showed one or more of local destruction, lesional size progression, fever, and cytopenias, but lesions responded to hydroxychloroquine and/or prednisone therapy and death attributable to panniculitis could not be documented. Cases that were phenotypically normal and without clonality had none of the aforesaid atypical clinical features. Conclusion: Lymphoid atypia, erythrophagocytosis, loss of certain pan T-cell markers, a reduced CD4/8 ratio and TCR rearrangement define subcuticular T-cell lymphoid dyscrasia, including a subset of LEP and ILLP. The subcuticular lymphoid infiltrates represent a spectrum of histologic, immunophenotypic, and molecular abnormalities which range from those which are clearly benign to those which are clearly neoplastic, and also encompasses those cases which defy precise classification into the two aforesaid poles.

Breast and nipple skin is commonly affected by various inflammatory and neoplastic processes. Despite this fact, many physicians are unaware of the spectrum of diseases that can involve this area. Because breast and nipple skin represents a cosmetically, sexually, and functionally important entity to most patients, awareness of these disease entities is invaluable. This article reviews the normal anatomy of the breast, cutaneous manifestations of neoplastic processes that can present in these areas, and common inflammatory diseases of the breast and nipple skin.

Background: Subcutaneous fat necrosis associated with pancreatic disease is a rare event. The clinical cutaneous findings are non-specific erythematous nodules with central softening located predominantly on the lower extremities. The histopathologic features of these lesions are very characteristic and diagnostic. Methods: We present an unusual case of pancreatic panniculitis associated with lupus pancreatitis in a 21-year-old African American female. The patient presented with lower extremity skin nodules, arthralgia, and serositis prior to the diagnosis of systemic lupus and pancreatitis. The skin lesions progressed despite normalization of serum pancreatic enzymes. Following femoral vein catheterization for renal dialysis, she developed a large indurated area over the left lower quadrant, flank, groin, and upper thigh measuring 25 cm. She was treated with repeated debridement, tissue grafts, and hyperbaric oxygen because of a clinical suspicion of necrotizing fasciitis. Results: Examination of skin biopsies and debrided tissue revealed the pathognomonic features of pancreatic panniculitis without any evidence of necrotizing fasciitis. Organisms were not detected by tissue examination or microbiologic cultures. Conclusions: This case illustrates the potential role of vascular trauma in the pathogenesis of pancreatic panniculitis.

Objective To present a patient with systemic lupus erythematosus who developed infarction and melting of the orbit secondary to her systemic disease. Design A case report. Participant A 61-year-old white woman with a 5-year history of systemic lupus erythematosus. Methods The patient presented with left orbital pain, limitation of extraocular movements, and a fistula from the ethmoid sinus to the upper eyelid. A detailed examination with computerized tomography, ultrasound, and a comprehensive medical evaluation with laboratory testing was performed. Histopathologic analysis with special stains of the orbital tissues was also performed. Results Histopathologic examination of the biopsy specimens revealed the features of an inflammatory process involving the orbit, similar to a panniculitis. These include a lymphocytic reaction with a predominance of plasma cells, vasculitis with occlusion, and thickening of the vessel walls, necrosis, and hyalinization of fat. Conclusion This is a unique case in which infarction and melting of the entire orbital structures occurred in the presence of systemic lupus erythematosus. The underlying disease process is a lupus-related panniculitis. The authors stress that this is a very rare entity and that other diseases should be ruled out before entertaining this diagnosis.

We present the case of a 57-year-old woman with a 1-year history of a cutaneous eruption clinically and histopathologically characteristic of Degos disease. In one of the two cutaneous biopsy specimens taken from the trunk lesions, the histopathology consisted of necrosis and sclerosis of the subcutaneous lobules, a finding that to our knowledge has not been previously described in the cutaneous lesions of Degos disease. Two months after cutaneous biopsies were taken, the patient developed ptosis and an episode of acute abdominal pain; intestinal perforation and many characteristic lesions of visceral Degos disease involving the entire small bowel were noted at laparotomy.

Generalized chronic cutaneous lupus including lupus panniculitis in childhood is rare and usually occurs in the setting of genetic complement deficiencies. The association with antiphospholipid syndrome is even more rare. We report a 13-year-old girl with extensive lupus panniculitis since the age of 8 months and no evidence of complement deficiency. She recently developed antiphospholipid syndrome characterized by anticardiolipin antibodies and digital necrosis.

Panniculitis rarely occurs in the course of systemic lupus erythematosus (SLE). When it occurs, it is thought to be mainly lupus erythematosus panniculitis (LEP). Here we describe a 32-year old Japanese woman with chronic SLE, who simultaneously presented facial lymphocytic lobular panniculitis and pancytopenia due to haemophagocytic syndrome. She showed several auto-antibodies against trilineage haematopoetic cells, an anti-cardiolipin antibody, and no evidence of viral infection, indicating that her haemophagocytic syndrome might be autoimmune-associated haemophagocytic syndrome. The panniculitis and haemophagocytic syndrome presented simultaneously, and these manifestations were dramatically improved with corticosteroid therapy; therefore, the lymphocytic lobular panniculitis could be linked to autoimmune-associated haemophagocytic syndrome in this case. Lupus (2007) 16, 363—365.

Background Lupus erythematosus profundus (LEP) is a rare variant lupus erythematosus with unclear etiology characterized by lobular panniculitis. Recently, we observed a case of LEP involving the lower right eyelid. Our immunohistological analyses of lesional skin biopsies revealed a type I IFN signature in the context of cytotoxic lobular panniculitis. Objective Since type I IFNs have been shown to be involved in other cutaneous LE subtypes, especially in chronic discoid LE, we hypothesized that a type I IFN driven immune response might play an important role in the pathogenesis of LEP. Methods In addition to the above case, 9 skin biopsies taken from 5 patients with LEP were analyzed for a type I interferon signature by immunohistochemistry. Furthermore, 8 skin biopsies taken from patients with active chronic discoid LE and 5 biopsies of healthy skin were included for control purposes. The inflammatory infiltrate was characterized using monoclonal antibodies specific for CD3, CD4, CD8, CD20, CD68, and CD123. Subsequently, we analyzed the expression the type I IFN Marker MxA, the cytotoxic molecules granzyme B and Tia1, the chemokine receptor CXCR3 and its ligand, the interferon inducible protein IP10/CXCL10. Results LEP skin lesions were characterized by a lobular panniculitis, dominated by cytotoxic CXCR3 + lymphocytes. Strong MxA expression indicated extensive type I IFN production within the fat lobules. Numerous plasmacytoid dendritic cells appear to be the major source of type I IFNs. Lesional expression of IP10 links the type I IFN production and recruitment of CXCR3 + lymphocytes. Limitations The study was based on histological and immunohistological analyses in a limited number of patients, due to the rareness of the investigated disease. Conclusion Our results demonstrate a type I IFN driven immune response in active LEP skin lesions. We suggest that this type I IFN driven inflammation is responsible for the recruitment of CXCR3 + lymphocytes into fat lobules and enhance their cytotoxic capacity.

Even though benefits of vaccination policies have been widely demonstrated, vaccine injections might be associated with rare side effects. In this setting, the potential role of vaccines, mostly against hepatitis B virus, in the induction of autoimmunity has been a matter of controversy. We report the case of a woman followed for a lupus panniculitis which had been in remission for 3 years, who developed a lupus flare following an anti-hepatitis-B vaccine injection. The topography of recurring lupus lesions, the chronology of the flare and the increase in the antinuclear autoantibody serum level all supported a causal role for vaccination in the relapse of the lupus lesions. We believe that the present case might provide a first observation of lupus panniculitis possibly induced by hepatitis B vaccination, and this should be added to the range of dysimmune manifestations caused by vaccinations.

PubMedID: 15934442 We describe a 16-year old female with lupus erythematosus panniculitis with unusual manifestations. She had noted to have developed erythematous nodules and plaques in the right axilla and inguinal region at the age of one year. These lesions resolved gradually with scar formation. However, new lesions were noted at the same locations in the following years. Some of her lesions at the scalp and the left axillary regions developing within the last two years slowly enlarged showing an annular configuration and subsequently resulted in hair loss. The erythematous border of her lesion in the left axilla consisted of two parallel red lines. Histopathological and direct immunofluorescent findings were consistent with lupus erythematosus panniculitis. Similar clinical findings in the same locations were also observed in the mother. © 2005 Edward Arnold (Publishers) Ltd.

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. After the first infusion, painful lesions had resolved without the appearance of new lesions. Rituximab may be an effective treatment for patients with LP when other therapies are ineffective. To date, there is only one case report of LEP treated with rituximab in the literature.

We report a 43-year-old woman, who underwent therapy with interferon-α for hairy cell leukemia. During interferon-α therapy she developed multiple subcutaneous swellings, accompanied by fever and fatigue. A skin biopsy revealed lobular, T-cell lymphocytic panniculitis. In conjunction with the clinical and immunological findings, the diagnosis of lupus panniculitis was made and interferon-α therapy stopped. Initially, she responded well to oral prednisone and hydroxychloroquine, but after several months she became resistant to it. Her condition worsened, she developed skin ulcers in the inflamed regions. Only with the leukemia-targeted therapy using cladribine and rituximab her skin condition could be controlled, suggesting hairy cell leukemia as an additional trigger of the lupus panniculitis. Our report is the first one to show induction of lupus panniculitis under interferon therapy of hairy cell leukemia and its presumable sustentation by the latter.

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. A biopsy specimen of the cheek demonstrated LE profundus with mucoid material deposition in the dermis. A biopsy specimen of the parotid gland showed lymphoplasmacytic infiltration and prominent mucoid material deposition within the parotid gland as well as mild lymphoplasmacytic infiltration and hyaline fat necrosis in the perisalivary tissue. Mucoid material deposition is one of the characteristic features of LE, however, this is the first case demonstrating mucoid material deposition in the salivary gland. Moreover, albeit extremely rare, lymphoplasmacytic infiltration within the lobules of the salivary gland has also been reported in patients with LE. Therefore, it is important that both lymphoplasmacytic infiltration and mucoid material deposition must be included in the differential diagnostic considerations for salivary gland tumors in patients who had been previously diagnosed as systemic or discoid LE.

Lupus Mastitis (LM) is a rare presentation of lupus panniculitis involving the breast. Because it often presents as a tender palpable mass, a workup for malignancy usually ensues. It is well documented that surgery may worsen the condition; therefore, it is important to consider LM in the differential of a palpable breast mass in patients with systemic lupus erythematosus (SLE). Currently, management of LM remains primarily medical. We discuss the multi-disciplinary work-up of LM, and further describe its appearance on serial Magnetic Resonance (MR) exams.

Clinicopathologic correlation of cutaneous biopsy specimens demonstrating typical lipomembranous fat necrosis was performed. Material from 732 biopsies of various subcutaneous inflammatory disorders seen at our institution in the past 5 years was screened for typical lipomembranous (membranocystic) changes in the panniculus, and 39 specimens from 38 patients with these changes were identified. The most common clinical context in which this condition was observed was in chronic sclerotic plaques of the lower legs associated with venous insufficiency (37% of the total cases). All patients were women, and the majority were obese. Typical lipomembranous fat necrosis was also observed in eight cases (21%) of erythema nodosum, three (8%) of morphea or subcutaneous morphea (or both), two (5%) of lupus panniculitis, two (5%) of necrobiosis lipoidica, and in single cases of polyarteritis nodosa, necrotizing vasculitis, and erysipelas. Six cases (16%) had no definite underlying disease. The mean age of all patients was 57 years (range 32-86 years), and 34 patients (89%) were women. Of the five major categories identified, lipomembranes lining macrocysts and microcysts were most prominent in the venous insufficiency- and morphea-related cases and were much less prominent in erythema nodosum, lupus panniculitis, and necrobiosis lipoidica, which generally showed histopathologic findings typical of these disorders. In addition to lining the macrocystic and microcystic cavities formed in the fat lobules, lipomembranes were prominent in areas of septal fibrosis in all cases associated with morphea and necrobiosis lipoidica and in 35% and 25% of venous insufficiency- and erythema nodosum-related cases, respectively. In lupus panniculitis, lipomembranes were most prominent in areas of hyaline necrosis. We conclude that lipomembranous fat necrosis is most likely a nonspecific form of ischemic fat degeneration that may be induced by various clinical entities. This change is most often seen in venous insufficiency-associated chronic sclerotic plaques typically observed in middle-aged obese women, and we propose the term stasis-associated lipomembranous panniculitis (SALP) to describe this most common form of lipomembranous fat necrosis.