Intravenous immunoglobulin in the management of refractory lupus profundus.

Lupus profundus, often known as lupus panniculitis, is a rare form of persistent cutaneous lupus erythematosus. It usually manifests as painful plaques or nodules that can ulcerate and cause atrophy and scarring. It may respond to topical treatments and antimalarials, although treatment might be difficult at times, necessitating immunosuppressive medications. A 36-year-old woman from the United Arab Emirates presented with multiple painful disfiguring nodules involving the face and shoulders. The disfiguring skin nodules were resistant to systemic glucocorticoids, hydroxychloroquine, azathioprine, mycophenolate mofetil, and cyclosporine, but they significantly improved with monthly intravenous immunoglobulin over a 6-month period.

Centrifugal lipodystrophy on a spectrum with lupus erythematosus panniculitis in children and efficacy and safety of hydroxychloroquine: A clinicopathological study.

This study aimed to investigate the relationship between centrifugal lipodystrophy (CLD) and lupus erythematosus panniculitis (LEP), and the efficacy and safety of hydroxychloroquine (HCQ) for treating CLD in children. A total of 29 cases clinically diagnosed as CLD (n = 24) and CLD/LEP overlap (n = 5) were enrolled and all were confirmed by skin biopsies of CLD and LEP. The clinicopathological findings, clinical outcomes, and prognosis with the treatment of HCQ between CLD and LEP were compared. All 29 cases (male: female = 1:1.6; median age at onset: 3 years) had cutaneous lesions of centrifugally expanding lipoatrophy, of which five cases overlapped with LEP lesions presented as erythematous indurated plaque (n = 2), subcutaneous nodules (n = 2) and alopecia along Blaschko's lines (n = 1). Antinuclear antibodies were found in six (25.0%) CLD and two (40.0%) overlapped patients (p = 0.597). Histopathologically, of the 24 cases of CLD, 14 (58.5%) exhibited subcutis loss or mild lobular inflammation. Ten (41.7%) cases displayed lobular panniculitis with moderate to dense lymphohistiocytic infiltrate and plasma cells, similar to the five cases of overlap. Small clusters of CD123 positive plasmacytoid dendritic cells were found in 62.5% (5/8) of CLD and 66.7% (2/3) of overlap cases (p > 0.99). HCQ (5 mg/kg/d) treatment showed improvement in 91.3% (21/23) of CLD and all overlap cases, including four cases unresponsive to previous oral glucocorticosteroid treatment. Our findings suggested that CLD and LEP represent a spectrum within the same disease. HCQ (5 mg/kg/d) was effective and safe for treating CLD (age >1.5 years), and early treatment and a regular long-term follow-up are essential.

Successful treatment of refractory lupus erythematosus profundus with the interleukin-6 antagonist tocilizumab: a case report.

Lupus erythematosus profundus (LEP) is an unusual form of cutaneous lupus erythematosus (CLE) characterized by multiple subcutaneous induration and associated with considerable physical and psychological morbidity. The heterogeneity of CLE makes it difficult to understand its underlying pathogenesis and represents a therapeutic challenge. Recently, new insight into the pathogenesis of CLE has implicated various cytokines, opening doors to targeted biologic agents. We report a case of a 23-year-old female who presented with refractory LEP ulcers as an initial presentation of systemic lupus erythematosus. The lesions were resistant to multiple conventional therapies and remarkably responded to tocilizumab.

Kikuchi-Fujimoto disease preceded by lupus erythematosus panniculitis: do these findings together herald the onset of systemic lupus erythematosus?

Kikuchi-Fujimoto disease (KFD), also known as histiocytic necrotizing lymphadenitis, is a rare disorder that must be distinguished from systemic lupus erythematosus (SLE). Although a minority of patients with KFD develop SLE, most patients have a self-limited disease. Importantly, KFD can have skin manifestations resembling cutaneous lupus. Therefore, the diagnosis of SLE should be predicated on a complete rheumatologic workup and not on the constellation of skin disease and lymphadenitis. Nonetheless, as our exceedingly rare case illustrates, patients who do not initially meet diagnostic criteria for SLE require dermatologic follow-up. We present a young adult woman who had a remote history of KFD and later presented with combined features of discoid lupus and lupus erythematosus panniculitis (LEP). On subsequent rheumatologic workup, she fulfilled criteria for SLE. We discuss the differential diagnosis of both KFD and LEP and emphasize how strong communication among dermatologists and other healthcare providers is essential in the management of patients with KFD.

Orbital T-cell lymphoma versus lupus panniculitits: a T-cell-mediated spectrum of orbital lymphoproliferative disease.

This is a case description of a single male patient found to have T-cell-mediated inflammation and lymphoproliferation of the orbit. Chronic T-cell-mediated inflammatory disease can pose a diagnostic challenge particularly in its differentiation from a neoplastic process. The histopathology in this case demonstrated features of both lupus erythematosus panniculitis and features of orbital T-cell lymphoma. While both are rare, lupus erythematosus panniculitis of the orbit is even more exceptional; this patient's indolent, chronic relapsing course distinguished itself from the typical aggression of orbital T-cell lymphoma. We believe this rare case may actually represent an example of a newly described disease spectrum that incorporates lupus erythematosus panniculitis as well as subcutaneous panniculitis-like T-cell lymphoma.

Tumid lupus: An unexpected diagnosis for the otolaryngologist.

Tumid lupus is an uncommon variant of lupus erythematosus. Patients present with subcutaneous lesions. Ophthalmic literature reports disease manifestation as orbital inflammation. Autoimmune serology is often negative. Without a high index of suspicion, the diagnosis is easily overlooked delaying treatment. Tumid lupus is not significantly discussed in the Otolaryngologic literature. Here we present a Case Report of a male who initially presented to Ophthalmology with unilateral orbital complaint of eyelid puffiness. Orbital biopsy and subsequent biopsy of his submental skin lesions ultimately led to this unexpected diagnosis. We discuss the method to diagnose tumid lupus including representative histopathologic findings.

Linear Sclerodermoid Lupus Erythematosus Profundus in a Child.

Lupus erythematosus panniculitis, also known as lupus profundus, is a variant in the clinicopathological spectrum of lupus erythematosus (LE) affecting about 2%-3% of LE patients. A linear configuration of LE panniculitis has been reported rarely with rare reports describing the coexistence of different forms of cutaneous LE and localized morphea. In this study, the authors present a 9-year-old girl with linear arrangement of subcutaneous nodules on her left forearm. Microscopic findings from 2 biopsies included lymphocytes at the dermoepidermal junction with mild interface dermatitis, a dense lymphocytic infiltrate that was concentrated around adnexae and subcutaneous fat in concert with thickened collagen bundles and mild widening of fibrous septae surrounding fat lobules. Although the clinical differential diagnosis included panniculitis or a sporotrichoid infection, 1 biopsy showed a dense lymphocytic infiltrate histologically bordered on that of cutaneous lymphoid hyperplasia or a late stage of Lyme disease, and a second also demonstrated more prominent sclerodermoid collagen bundles rendering the diagnosis of linear sclerodermoid LE profundus.

Magnetic resonance imaging findings are useful for evaluating the three-dimensional development and follow-up of linear lupus erythematosus profundus.

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.

[Juvenile systemic lupus erythematosus with unusual manifestation of lupus-associated panniculitis]. / Juveniler systemischer Lupus erythematodes mit der ungewöhnlichen Manifestation einer Lupus-assoziierten Pannikulitis.

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.

The proportion of lymphocytic inflammation with CD123-positive cells in lupus erythematous profundus predict a clinical response to treatment.

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. On the other hand, the patients with minor lymphocytic inflammation with low percentages of CD123+ cells showed poor response to treatments. The mean percentage of CD123+ cells in patients who showed good response to therapy was significantly higher than those that showed poor response (p = 0.027). These results suggest that the clinical response to treatment of lupus erythematosus profundus could be predicted from the histological features.

Subcutaneous panniculitis-like T-cell lymphoma and lupus erythematosus profundus: a diagnostic dilemma.

A white Caucasian woman in her 30s presented with an indurated lesion on her right upper arm. Panniculitis was clinically suspected. Antinuclear antibody testing was positive but incisional biopsy showed subcutaneous panniculitis-like T-cell lymphoma (SPTCL), although with some unusual features more in keeping with lupus. Initial treatment was with oral prednisolone and radiotherapy but with only partial response. A second biopsy was taken from an area of presumed residual disease. This displayed histological features that were much more typical of lupus erythematosus profundus (LEP) but with tiny foci suggesting concomitant microscopic areas of SPTCL. Immunofluorescence for IgM was positive. This case highlights the rare occurrence of a patient with overlapping clinical and pathological features of SCPTL and LEP. It emphasises the need for close clinicopathological correlation in the workup of patients with suspected panniculitis and the importance of careful pathological examination for features of both diseases.

Rituximab for the treatment of lupus erythematosus panniculitis.

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.

Orbital Mass as the Only Presenting Sign with Overlapping Features of Lupus Erythematosus Panniculitis and Subcutaneous Panniculitis-Like T-Cell Lymphoma.

PURPOSE: Even though subcutaneous panniculitis-like T-cell lymphoma (SPTCL) and lupus erythematosus panniculitis (LEP) are two separate entities, recently they were claimed to represent two ends of a spectrum of T-cell-mediated orbital lymphoproliferative diseases. METHODS: A 78-year-old woman presented with a 1-month history of right-sided periorbital swelling and redness. There was a palpable mass in the medial right lower eyelid with restriction of upward and lateral gaze. MRI revealed a 14 × 7 mm hypointense lesion in the anteromedial orbit. RESULTS: The systemic and ocular findings, orbital biopsy with histopathology and immunochemistry showed overlapping features of LEP and SPTCL. The patient was consulted with rheumatology and hematology, and the physicians arrived at a consensus that the patient existed in the above-mentioned disease spectrum. She was started on systemic immunosuppressive treatment and her clinical findings improved substantially. CONCLUSIONS: This is the first report of a patient, who presented with orbital mass causing vision loss and gaze restriction that had overlapping clinical and histopathologic features of LEP and SPTCL consistent with this novel disease spectrum, in the literature.

A case of lupus erythematosus profundus with multiple arc-shaped erythematous plaques on the scalp and a review of the literature.

A 26-year-old Japanese female presented to us with a 2-year history of multiple arc-shaped erythematous lesions on her scalp and the right side of her forehead. Histopathological examination of two of the lesions showed lobular and septal panniculitis with deposits of IgG in the basement membrane zone. We diagnosed the case as lupus erythematosus profundus, and successfully treated her with 20 mg/day prednisolone. To our knowledge, there has been only one previously reported case of lupus erythematosus profundus with annular-shaped erythematous lesions and six cases with linear configuration.