Panniculitic primary cutaneous gamma delta T-cell lymphoma with concomitant features of autoimmune disease emphasizing a pathophysiologic continuum of lupus profundus with the panniculitic T cell lymphomas.

Certain T-cell lymphomas exhibit unique homing properties of the neoplastic lymphocytes for the subcutaneous fat. There are two primary forms of subcutaneous panniculitic lymphomas of T-cell origin. One falls under the designation of primary cutaneous gamma-delta T-cell lymphomas (PGD-TCL) whereby there is dominant involvement of the fat defininng a panniculitic form of PGD-TCL. The neoplastic cells are of the gamma-delta subset and are either double negative for CD4 and CD8 and/or can express CD8. They often have an aggressive clinical course. The other form of panniculitic T-cell lymphoma falls under the designation of subcutaneous panniculitis-like T-cell lymphoma (SPTCL). It represents a subcutaneous lymphoma derived from CD8+ T cells of the alpha-beta subset and typically has an indolent course. These two forms of panniculitic T-cell lymphoma exhibit overlapping histologic features with lupus profundus (LP), a putative form of panniculitic T-cell dyscrasia. We present three cases of PGD-TCL of the fat in the setting of lupus erythematosus (LE) (two cases) and dermatomyositis (DM) (one case), respectively. There were concurrent features of LE and DM in their lymphoma biopsies in two cases while a prior biopsy in one was interpreted as LP. In this latter case, the LP diagnosis presaged the diagnosis of panniculitic PGD-TCL by three years. One patient diagnosed with panniculitic PGD-TCL had hemophagocytic syndrome after developing a lupus-like complex including certain supportive serologies such as antibodies to double-stranded DNA following initiation of statin therapy. The second patient presented with PGD-TCL and concomitant features of anti-nuclear matrix 2 (NXP2) DM. The third patient presented in 2003 with LP and overlying skin features of acute LE, initially responding to Plaquenil, and then four years later was diagnosed with PGD-TCL heralded by Plaquenil treatment resistance. Two of the patients died of their lymphoma. All biopsies showed a characteristic histopathology of PGD-TCL. In two cases, the PGD-TCL was associated with overlying LE-cutaneous findings; another case had skin changes of lymphocyte-rich DM. In two cases, the MXA stain was strikingly positive, the surrogate type I interferon marker that is typically upregulated in biopsies of LE and DM. There are eight prior reported cases describing SPTCL with concomitant cutaneous changes of LE. In six cases there was an established history of LE, including LP responding initially to Plaquenil, similar to one of our cases. In the context of SPTCL or panniculitic PGD-TCL, panniculitic T-cell lymphomas can be associated with concomitant clinical and histologic features of LE or DM, including an upregulated type I interferon signature. Identifying histologic features associated with either of these prototypic autoimmune conditions should not be considered exclusionary to diagnosing any panniculitic T-cell lymphoma. A clinical, histomorphologic, and pathophysiologic continuum exists with LP, SPTCL and panniculitic PGD-TCL.

Non-scarring alopecia of lupus erythematosus: A comprehensive review.

BACKGROUND: Although non-scarring alopecia (NSA) is a frequent clinical finding in patients with systemic lupus erythematosus (SLE), it has been poorly described in the literature. It is considered a nonspecific sign in the current classification of skin lesions of LE. The aim of this study was to give an updated overview of the spectrum of NSA in LE patients, with emphasis on the clinical significance thereof. METHOD: We conducted a review of the English literature using the PubMed-Medline database using the keywords "Alopecia" + "Lupus erythematosus". Publications describing LE patients with NSA were included. RESULTS: Data for 237 patients from 27 publications were analyzed. Ninety-one patients had diffuse NSA, 43 had patchy NSA, 83 had lupus hair, 3 had alopecia of dermal cutaneous LE, and 17 had alopecia of linear and annular lupus panniculitis of the scalp. Patients with diffuse/patchy NSA and lupus hair shared the following features: strong association with systemic activity of LE, subtle clinical/trichoscopic signs of inflammation, histological aspect consistent with lesions specific to cutaneous LE, high likelihood of response to SLE therapy, and absence of progression to scarring alopecia. Association with SLE was rare in patients with dermal cutaneous LE or linear and annular lupus panniculitis of the scalp, and skin-directed therapies were most often effective. One patient of each subtype progressed to scarring alopecia. DISCUSSION: Diffuse/patchy NSA and lupus hair may represent a topographic variation of a single entity specific for LE. Prospective studies are warranted to further document the clinical significance of this manifestation.

Rare Initial Manifestation of Lupus as Lobular Panniculitis of the Breast-A Case Report and Review of the Literature.

ABSTRACT: Lupus mastitis is a rare complication of systemic or discoid lupus erythematosus with an uncommon initial presentation when limited to the breast. In this article, we report a 42-year-old woman who presented with constant pain and tenderness in her breasts. Ultrasound imaging of the left breast revealed a 14-mm oval mass, suspicious for malignancy; a needle core biopsy was performed. Sections showed necrosis of the fat lobules with associated mixed nodular lymphoplasmacytic aggregates. Karyorrhectic debris, fibrinoid necrosis of small vessels, and microcalcifications were all present while the background breast parenchyma was unremarkable. The diagnosis of lupus mastitis was rendered. Subsequent serology showed negative dsDNA but positive antinuclear antibodies, C4, and anti -Sjögren';s-syndrome-related antigen A antibodies. Clinical features of active systemic disease were not identified at the patient's follow-up dermatology appointment 1 month after the biopsy, and she elected management for her nodules with steroids. To the best of our knowledge, only 40 other cases of lupus mastitis have been reported in the English literature, of which 25 presented as a mass and only in 6 lupus mastitis of the breast was the initial presentation. In conclusion, we bring much needed awareness to lupus mastitis as the first presentation of disease.

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.

Distinctive lupus panniculitis of scalp with linear alopecia along Blaschko's lines: a review of the literature.

Lupus panniculitis of the scalp (LPS) is a rare and distinctive clinical feature of lupus erythematosus panniculitis (LEP) with linear alopecia along Blaschko's lines. In this study, we investigated clinical features and treatments of LPS by literature review of articles in the English language from PubMed and SCOPUS databases up to April 2018. The following key words, "lupus panniculitis, "lupus erythematosus panniculitis", "lupus profundus", "head", and "scalp", were used. Twenty cases of LPS were identified (mean age = 26.4 [10-53] years, female: male ratio = 1:1, mean disease duration = 86.89 [8-336] weeks). The most commonly affected areas of scalp included parietal (70%), frontal (45%), temporal (40%), occipital (30%), and vertex (10%), along 70% of Blaschko's lines with morphologic lesions linear, annular, arch-shaped, and ulcer. Besides, ANA (60%) was in particular noted. Hydroxychloroquine, oral prednisolone, intralesional corticosteroid, and methotrexate were the most common treatments, with complete response after an average period of 8.08 (2-12) weeks. Systemic lupus erythematosus (SLE) was developed in four cases (20%) during follow-up, with high recurrence of 35%. We reported distinctive and rare cases of LPS.The continuing follow-up was highly recommended to avoid probable recurrence and SLE development.

Evaluation of Repairing Facial Depression Deformities Secondary to Lupus Erythematosus Panniculitis With Autologous Fat Grafting.

BACKGROUND: A facial depression deformity secondary to lupus erythematosus panniculitis results from fat necrosis, which seriously alters the patients' appearance and thus affects their psychological health. Few studies have discussed the repair of depression deformities. The authors repaired depression deformities due to atrophic lesions by using autologous fat grafting to obtain volumetric restoration of the facial skin, and the authors report our outcomes. METHODS: In this series, 30 depression deformities in 18 patients were repaired. The authors suctioned fat grafts from the abdomen and centrifuged them at 500×g for 2 minutes. After discarding the upper oil and bottom liquid, the middle fat was injected into the depressed areas (ie, the cheek, temple, and zygoma) by using multiplane and multitunnel injections. The patients, plastic surgeons on staff, and laypersons evaluated the cosmetic outcome of each patient. RESULTS: No infection, subcutaneous nodules or cysts, cutaneous necrosis, blood vessel embolism, or other complications were found in any patient. Five patients had 1 injection, 5 had 2 injections, and 8 had 3 injections. All depression deformities improved. Regarding the cosmetic outcome, 33.3% of patients, 27.8% of laypersons, and 38.9% of doctors were satisfied with the results; 44.4% of patients, 55.6% of laypersons, and 50.0% of doctors were mostly satisfied. CONCLUSION: Autologous fat grafting can be a good choice for repairing facial depression deformities in patients with lupus erythematosus panniculitis, although 1 or more subsequent injections may be required to maintain the improved outcome.

The involvement of T regulatory lymphocytes in a cohort of lupus nephritis patients: a pilot study.

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.

Facial myiasis and canthariasis associated with systemic lupus panniculitis: a case report.

BACKGROUND: Infestation of insects can cause myiasis due to dipetran larvae and canthariasis due to coleopteran larval or adult stages. OBJECTIVE: To report a case of facial myiasis and canthariasis in a patient known to have panniculitis due to systemic lupus erythematosus and autoimmune hemolytic anemia. METHOD: Larval and pupal stages of insects were recovered from the face of a patient, mounted, and identified. RESULT: Initially, the pupal stage of the common green bottle fly Lucilia sericata was extracted from the patient's face, treated, and then came back with secondary infestation caused by two species of beetles, the drugstore beetle, Stegobium paniceum, and a beetle of the genus Trogoderma. CONCLUSION: This is the first report on facial canthariasis due to larval stages caused by two species of beetles and recovery of pupal stages of the common green bottle fly.