A Clinical Example of SLE presenting with Lupus Panniculitis of the Lower Extremities - Research Paper Example

A Clinical Example of SLE presenting with Lupus Panniculitis of the Lower Extremities: A Rare Case Report This is a case of a 36 year-old female diagnosed with Systemic Lupus Erythematosus who presented with nodules on her lower extremities seen in lupus panniculitis and is rarely seen presenting in patients with SLE. The blood works done on the patient reveal positive ANA and anti-dsDNA as well as normal C3 and C4. Skin biopsy was suggested for the rashes but was refused by the patient. Treatment done was with low dose steroids and Plaquenil which offered resolution of symptoms. Diagnosis of SLE is difficult and it takes a combination of study of the clinical symptoms as well as the laboratory results that include the blood tests. This patient presented with lupus panniculitis which is a type of rash rarely seen in presenting in patients with SLE and it requires clinical and pathologic knowledge to diagnose. The treatment for this condition can be done with a combination regimen using steroids and another drug, which in this case is Plaquenil. But sometimes this type of treatment would leave hyper pigmentations and scars on the affected skin area after resolution of symptoms. Introduction Lupus panniculitis which is a variant of erythema nodosum or lupus erythematosus has close resemblance clinically and can only be differentiated histopathologically. This is a rare disease which occurs as a separate entity or rarely along with systemic lupus erythematosus. In most cases, it is a disease of women between ages thirty to sixty (Jajic et. al. 29). This presentation is rare and there are no reports that talk about its incidence or prevalence (Jacyk 258). LEP is an uncommon variant in the clinico-pathological spectrum of lupus erythematosus (erythema nodosum in SLE) characterized by chronic inflammation and hyaline necrosis of subcutaneous tissue (Koley et. al 118). Lupus panniculitis occurs in two to five per cent of SLE patients. Conversely, 10 to 15% of the patients with this disease as well can have or develop SLE. Lupus panniculitis was seen in six out of 228 DLE (Discoid Lupus Erythematosus) patients (Patel and Marfatia 99). Lupus panniculitis or Lupus erythematosus profundus (LEP) affects the deep corium of the skin and the subcutaneous tissues are mainly involved. This type of panniculitis differs from other panniculitis by its distribution and clinical changes. Usually these lesions develop mostly on proximal parts of the upper extremities, trunks, face and head. They rarely appear on the lower extremities (Jajic et. al. 31) which were seen in the following case report. Case Report A 36 year old female from Saudi presented with tender subcutaneous nodules on both lower extremities primarily on the thighs and legs, which is seen in rare cases of lupus panniculitis. These nodules were followed by manifestations of inflammatory pains in the PIP, wrist and knee joints with history of oral ulcerations and no other associated SLE features. The blood examinations done showed the patient to have positive ANA by ELISA and positive antids-DNA. The patient was advised to undergo skin biopsy for the rashes but the patient refused. The patient had normal C3 and C4 as well on additional blood works that were done. She was treated initially with low dose tapering steroids and Plaquenil. The nodules seen on her lower extremities were recurrent before treatment was done and after treatment was completed, resolution of her symptoms was seen but left hyperpigmented skin changes after resolution. Discussion Systemic Lupus Erythematosus or SLE is a chronic inflammatory disease that can affect various organs of the body. Usually the patients present with skin rashes and arthritis, as well as fatigue and fever. Lupus attacks can vary from mild to severe, and usually alternate between periods of activity and periods of resolution (Ginzler and Tayar 1). The pathogenesis of SLE remains unclear, but the concept of apoptosis explains how the immune system recognize mostly intracellular antigens, thus autoantigens are then released by necrotic and apoptotic cells. The defects in the clearance of apoptotic cells have been seen in SLE which may lead to aberrant uptake by macrophages which function mainly to present the previously intracellular antigens to T and B cells, thus driving the autoimmune process (Cervera et al 2). SLE is hard to diagnose especially since it mimics many diseases. One way of diagnosing this disease is looking at its clinical symptoms and in this case we have rashes which are a type of panniculitis that is acute in onset and is characterized by the sudden eruption of erythematous, tender nodules located over the extensor aspects of the lower extremities. The lesions can spontaneously regress, without ulceration, scarring, and recurrent episodes are common. Moreover, it is a cutaneous process that may be triggered by a wide variety of possible stimuli such as infections, sarcoidosis, rheumatologic diseases, medications, autoimmune disorders such as SLE in this case (Requena and Yus 114). This panniculitis affects subcutaneous fat in the skin, usually first evident as an outcropping of erythematous nodules that are highly sensitive to touch (Schwartz and Nervi 695). In addition to these rashes, the patient also presented with oral ulcers and arthritis, and in the patients case is on the knees and wrist joints. The clinical symptoms seen in a patient will not be enough to diagnose SLE since a lot of autoimmune diseases have the same symptoms. Suspected cases must be confirmed by a series of blood tests and the most important test for Systemic Lupus Erythematosus measures ANA, the antinuclear antibody. Additionally specific tests such as the anti‐double strand DNA (dsDNA) and anti‐smith antibodies (Sm) also confirm the diagnosis of lupus. Levels of certain complement in the blood are also measured to help diagnose and track the disease. The presence of other types of antibodies (anti‐phospholipid antibodies) can also aid in helping doctors diagnose lupus (Ginzler and Tayar 2). In this case, the patient is positive for the anti-dsDNA antibodies as well as the ANA. The ANA is usually positive in all lupus patients making the patient likely to have SLE. Table 1. Diagnostic Criteria of SLE (Manson and Rahman 2) Criterion Definition/Examples 1. Malar Rash Fixed erythema over malar eminences extending to the nasolabial folds 2. Discoid Rash Eythematous raised patches, may scar 3. Photosensitivity Skin rash as a result of unusual reaction to light 4. Oral ulcers Usually painless 5. Arthritis Non-erosive: Jaccoud’s Arthropathy 6. Serositis a. Pleuritis b. Pericarditis 7. Renal Manifestations a. Proteinuria b. Cellucar casts in the urine 8. Neurologic Manifestations a. Seizures b. Psychosis 9. Hematologic Manifestations a. Haemolytic Anemia b. Leukopenia c. Lymphopenia d. thronmocytopenia 10. Immunologic Manifestations a. Anti-DNA antibodies b. Anti-Sm antibodies c. Anti-phopholipid antibodies 11. Antinuclear antibodies Exclude drug causes According to the table above, a patient is said to have SLE when the patients meets 4 out of 11 of these criteria whether the symptoms and signs appeared simultaneously or in succession. This shows that the patient in this case is in fact a case of SLE. Since the patient met 4 of the criteria above which include presence of the, oral ulcers, arthritis or inflammation and pain on her wrist and knee joints, as well as positive anti-dsDNA antibodies and positive ANA, it is most likely that this is a case of SLE. In addition to the symptoms showed above, the patient also presented with rashes on her lower extremities (thighs and legs) known as lupus panniculitis. Clinically, the lesions of lupus panniculitis can resemble other forms of panniculitis, but the persistence of the lesions and the rarity of involvement of the distal extremities as seen in this patient helped to distinguish it from conditions such as erythema nodosum (Koley et. al. 120). Since the patient refused skin biopsy to differentiate this panniculitis from others would depend on the clinical description of the rashes from its appearance until it regresses since biopsies are usually used in determining the different types of panniculitis. In atypical cases, the differential diagnosis between two entities may be solved by histopathological examination, because lupus erythematosus is a mostly septal panniculitis (Requena and Requena 4). On the other hand, lupus panniculitis or LEP has a characteristic histopathologic finding in the subcutis such as having mainly lobular panniculitis, lymphoid nodules and lymphocytic vasculitis. This requires differentiation of lupus panniculitis from panniculitis in other connective tissue diseases (Jacik 257). Antimalarials like chloroquine and hydroxychloroquine are the mainstay of treatment for LEP. The chronic and often relapsing course of LEP requires prolonged treatment and monitoring of laboratory parameters is necessary. Some patients require systemic corticosteroids, usually combined with antimalarials as seen in this case (Jacyk 2661). Furthermore, systemic steroids are a relatively safe therapeutic option if underlying infection and risk of sepsis, have been excluded upon evaluation. oral prednisone at a dosage of 60 mg every morning is typical. Treatment may also be disease-specific: steroids used in combination with hydroxychloroquine (Plaquenil), cyclosporin A (Sandimmune), or thalidomide (Thalomid) have been used to treat erythema nodosum (Schwartz and Nervi 696). In this case, steroids in combination with hydroxychloroquinone (Plaquenil) which is an antimalarial drug was used to treat the patient. Normally this drug is used when the patient presents with the milder form of disease such as presence of skin disease and joint pain as seen in the patient. Works Cited: Cervera, Ricard et al. Systemic lupus erythematosus: pathogenesis, clinical manifestations and diagnosis. January 2012. http: //www.eular.org/myuploaddata /files/Compendium_ sample_ chapter.pdf Ginzler, Ellen and Jean Tayar. Systemic Lupus Erythematosus (Lupus). American College of Rheumatology, 2008. Jacyk, Witold. Lupus Erythematosus Profundus (lupus panniculitis). Dermatologia Kliniczna 2011, 13.4 (2011): 257-261. Jajik,I. et. al. Lupus Profundus or Lupus Erythematosus Panniculitis. Department of Rheumatology, Medicine and Rehabilitation and Clinical Institute of Pathology. 15.1 (2001): 29-31. Koley, Sankha et. al. Lupus erythematosus panniculitis: a case report. Journal of Pakistan Association of Dermatologists 21 (2011): 118-121. Manson, Jessica and Rahman Anisur. Systemic Lupus Erythematosus. Orphanet Encyclopedia. November 2005. Patel RM and Marfatia YS. Lupus panniculitis as an initial manifestation of systemic lupus erythematosus. Indian J Dermatol 55 (2010): 99-101. Requena, Luis and Celia Requena. Erythema Nodosum. Dermatology Online Journal 8.1 (2002): 4. Requena, Luis and Evaristo Yus. Erythema Nodosum. Seminars in Cutaneous Medicine and Surgery 26 (2007): 114-125. Schwartz, Robert and Stephen Nervi. Erythema Nodosum: A Sign of Systemic Disease. American Family Physician 75.5 (2007): 695-700. Read More