Successful treatment of bullous lupus with corticosteroids and belimumab: A case report.

Cutaneous lupus erythematosus (CLE) is one of the most common manifestations of systemic lupus erythematosus (SLE), although it can manifest as an independent entity as well. Bullous systemic lupus erythematosus (BSLE) is a rare cutaneous manifestation of SLE presenting as tense vesiculobullous eruptions in a photosensitive distribution. Pathophysiology is secondary to autoantibodies against noncollagenous domain 1 and 2 (NC1 and NC2) type VII collagen, and histopathology reveals dense neutrophilic infiltration of the dermis with direct immunofluorescence showing IgG deposition at dermoepidermal junction. There is lack of data on available therapeutic options to treat BSLE, and varying responses to dapsone, methotrexate, azathioprine and corticosteroids have been reported. Belimumab, a fully humanised Change to Immunoglobulin G1λ (IgG1λ) monoclonal antibody targeting soluble B lymphocyte stimulator protein, was the first Food and Drug Administration-approved drug for SLE and has been reported to be effective for CLE. We present the case of a 41-year-old black female with SLE presenting with BSLE, who was successfully treated with corticosteroids and belimumab and did not experience disease relapse even after discontinuation of corticosteroids. To our knowledge, this is the first reported case of successful treatment of BSLE with belimumab, and further research can help determine the role of belimumab in the treatment of BSLE.

Vasculitis in Patients With Sarcoidosis: A Single-Institution Case Series of 17 Patients.

OBJECTIVES: Vasculitis in patients with sarcoidosis is rare and can affect any sized blood vessel. Limited information describing this association is available. METHODS: A single-institution medical records review study was performed reviewing all patients with a diagnosis code for sarcoidosis and vasculitis between January 1, 1998, and December 31, 2019. Data were abstracted regarding diagnosis, treatment, and outcomes from medical records. Patients were diagnosed with vasculitis based on biopsy and/or arterial imaging. Comparison between patients presenting with large and/or medium vessel vasculitis (L/MVV) versus patients with only small vessel vasculitis (SVV) was performed. RESULTS: Seventeen patients were identified during the study period. Nine patients (56% female) had L/MVV, and 8 (50% female) had SVV. Sarcoidosis preceded vasculitis in 4 (44%) L/MVV and 3 (38%) SVV. The mean ± SD age at sarcoidosis diagnosis was 53.2 ± 17.8 and 51.9 ± 11.4 years, and the mean ± SD age at vasculitis diagnosis was 57.4 ± 19.6 and 59.0 ± 13.4 years in L/MVV and SVV, respectively. Number of organ systems involved by sarcoidosis was similar (median [interquartile range], 3 [1-4] L/MVV vs 2.5 [1.75-3.25] SVV). The mean length of follow-up was 11.5 ± 12.8 in L/MVV and 13.1 ± 14.3 years in SVV. Complete response to therapy for vasculitis was observed in 8 of 9 with L/MVV and 7 of 8 with SVV. Four patients with SVV were able to stop all immunosuppression as compared with only 1 patient with L/MVV at the last follow-up. CONCLUSIONS: This series observed a comparable number of patients with L/MVV and SVV. Although a variety of treatments were used, most patients achieved remission regardless of vessel size affected. Clinicians should be aware of the overlap between sarcoidosis and vasculitis.

Symmetrical drug-related intertriginous and flexural exanthema (Baboon syndrome) associated with Infliximab: a case-based review.

BACKGROUND: SDRIFE is a rare cutaneous eruption characterized by symmetrical intertriginous dermatitis, caused by delayed Type-IV immune reaction, with several reported drug-triggers. OBJECTIVE: We present a case of SDRIFE associated with infliximab in a 70-year-old female with rheumatoid arthritis, and review cases of SDRIFE associated with TNF-inhibitors. METHODS: A literature review about SDRIFE cases associated with TNF-inhibitors was performed. Articles published in English from inception to January 6th, 2022, restricted to humans, and directly related to this review were included. RESULTS: Ours is the third reported case of SDRIFE associated with TNF-inhibitors, and second with infliximab. SDRIFE can occur anytime during treatment with TNF-inhibitors, and presents with similar clinical and histopathological features as SDRIFE secondary to other drugs. No systemic manifestations have been reported, and the rash resolves after discontinuation of the TNF-inhibitor without any long-term sequelae. CONCLUSION: SDRIFE is benign, and an accurate diagnosis and discontinuation of the responsible drug remain the cornerstone of management.

The Many Faces of Purpura: Vancomycin-Induced Leukocytoclastic Vasculitis.

Leukocytoclastic vasculitis is a rare form of immune-mediated vasculitis that might be caused by infections or autoimmune diseases or might be precipitated by specific medications. We describe a 65-year-old patient, who was receiving vancomycin for a methicillin-sensitive Staphylococcus aureus permacath infection. Vancomycin was chosen due to medication non-adherence and the patient's desire to receive antimicrobial therapy in conjunction with his scheduled dialysis sessions. The patient's medical history was notable for untreated hepatitis C infection and end-stage renal disease, requiring hemodialysis three times a week. Vancomycin was administered during dialysis sessions. After one week of therapy, the patient developed bilateral lower extremity purpura. Skin biopsy was suggestive of leukocytoclastic vasculitis with an absence of intravascular thrombi. Serum cryoglobulins were negative, making cryoglobulinemia due to HCV infection unlikely. Following cessation of vancomycin therapy, the rash gradually disappeared with scarring in the form of post-purpuric hyperpigmentation. Despite its widespread use, vancomycin is a rare cause of leukocytoclastic vasculitis. Clinicians should keep in mind a wide range of differential diagnosis of bilateral lower extremity purpura as treatment differs depending on its underlying etiology.

Metastatic Carcinoma of Prostate as a Mimicker of SAPHO Syndrome.

Paraneoplastic arthritides are a group of immune-mediated inflammatory arthropathies associated with occult or manifest malignancy. Musculoskeletal spread of an underlying malignancy may also mimic many rheumatologic conditions. Distinguishing primary rheumatologic condition from paraneoplastic arthritides versus direct musculoskeletal spread of malignancy can be challenging especially in individuals with prior history of cancer and new musculoskeletal complaints. SAPHO (synovitis, acne, pustulosis, hyperostosis and osteitis) syndrome is an uncommon, although under recognized autoimmune disorder. Two musculoskeletal manifestations, namely inflammatory osteitis and hyperostosis of anterior chest wall with or without dermatologic manifestations, constitute a unifying feature of SAPHO syndrome. However, diagnosis of SAPHO syndrome is one of exclusion, and a wide variety of disorders including infections, malignancy (chondrosarcoma/osteosarcoma/metastasis), metabolic bone disorders (Paget's disease), osteoarthritis, seronegative spondyloarthropathy (spA) and osteonecrosis form part of a broad differential diagnosis. We present the case of a man, aged 72 years, with signs and symptoms of SAPHO syndrome and skin findings. Detailed history, radiological imaging, dermatology appearance, and role of immunohistochemical markers, especially staining for NKX3.1 protein with a novel antibody, led to a diagnosis of metastatic prostate adenocarcinoma. To our knowledge, this is the first case of metastatic adenocarcinoma of the prostate manifesting as SAPHO syndrome and cutaneous metastasis.

Disseminated Blastomycosis Presenting with Spontaneous Coronary Artery Dissection.

Spontaneous coronary artery dissection (SCAD) is increasingly recognized as an important cause of acute coronary syndrome (ACS) and myocardial infarction (MI) in individuals with few or no known atherosclerotic risk factors. While systemic autoimmune inflammatory disorders are associated with precipitating SCAD, the role of infection-induced systemic inflammation in SCAD is not well defined. We present the case of a 49-year-old Caucasian woman with ST-elevation myocardial infarction (STEMI) diagnosed as SCAD from a severe systemic inflammatory response related to disseminated blastomycosis. Punch biopsy of a skin lesion and synovial fluid culture confirmed Blastomyces dermatitidis. This case suggests the possibility of systemic infection-induced inflammation as a precipitating factor in SCAD pathogenesis similar to autoimmune inflammatory disorders. LEARNING POINTS: Recognize the role of systemic inflammation from severe infection as a possible cause of spontaneous coronary artery dissection (SCAD).Recognize that cardiac involvement is rare in blastomycosis.Coronary revascularization may be required in SCAD for haemodynamic instability, ischaemic chest pain progression, and myocardium at risk.

Leukocytoclastic vasculitis as a rare dermatologic manifestation of Crohn's disease mimicking cellulitis: a case report.

BACKGROUND: Leukocytoclastic vasculitis (LCV) is an immune-complex mediated vasculitis characterized by neutrophilic inflammation and nuclear debris in post capillary venules. LCV is a rare dermatologic manifestation of Crohn's disease (CD) and may occur with the onset of the disease or any time after the diagnosis including the period of exacerbation. CASE PRESENTATION: We present a 70 year old woman with history of psoriasis and treatment refractory CD requiring monoclonal antibody therapy with ustekinumab. One month prior to the current admission, she developed abdominal pain, worsening diarrhea and was diagnosed with CD exacerbation for which she was given ustekinumab. While her abdominal symptoms mildly improved with ustekinumab, she developed new bilateral lower extremity rash initially treated with levofloxacin for presumed cellulitis. The rash consisted of mild erythematous, non-scaling patches with scattered non-palpable petechiae on the lower extremities with subsequent involvement of abdomen, lower back and buttocks. Abdominal exam showed diffuse tenderness without mass, guarding or rebound while reminder of physical exam was unremarkable. Following the failure of antimicrobial therapy, she was diagnosed with LCV by skin biopsy. Complete work up was negative for infectious, malignant and inflammatory etiologies of LCV. Patient improved with increased dose of budesonide and subsequently continued to tolerate ustekinumab without recurrence of LCV. DISCUSSION AND CONCLUSION: LCV is a rare form of vasculitis and one of the rarest dermatologic manifestations of CD, appearing at any stage of the disease. LCV has been associated with autoimmune diseases, infections, specific drugs (levofloxacin, ustekinumab), and malignancy. Clinical presentation of LCV is variable and frequently mistaken for cellulitis. LCV should be considered in differential diagnosis of bilateral lower extremity rash in patients with CD after infectious, malignant and auto-immune/inflammatory etiologies are excluded. Unlike erythema nodosum (EN) and pyoderma gangrenosum (PG), LCV requires biopsy for diagnosis. Most patients respond well to steroids without scarring.

A Case of Linear Cutaneous Lupus Erythematosus in a 55-Year-Old Woman.

BACKGROUND Linear cutaneous lupus erythematosus (LCLE) is uncommon and occurs mainly in children and young adults. To our knowledge, only ten cases of LCLE in adults have been previously reported. A case is presented of LCLE of the left arm in a 55-year-old woman. CASE REPORT A 55-year-old Caucasian woman from the Midwestern United States presented with a three-month history of a pruritic linear eruption on the left arm. She had a previous history of methicillin-resistant Staphylococcus aureus (MRSA) infection of the left forearm. She had previously been treated with topical triamcinolone, hydrocortisone cream, hydroxyzine, and two courses of prednisone. Physical examination showed a unilateral and linear erythematous skin lesion of the left arm that contained papules and followed the embryonal developmental epidermal lines of Blaschko. Histopathology of a 4 mm skin punch biopsy showed an interface dermatitis with keratinocyte necrosis and increased dermal mucin. Immunofluorescence of the skin biopsy, including for antinuclear antigen (ANA), was negative. Prednisone treatment reduced the symptoms of pruritis but did not resolve the rash. However, following topical treatment with betamethasone dipropionate cream for between two and three weeks, and the use of sunblock, the skin lesions resolved. CONCLUSIONS This rare case of LCLE in an older adult showed a similar response to treatment as other forms of cutaneous lupus erythematosus, with treatment that included topical steroids and sun protection. Also, this case supports that environmental trigger factors, such as prior infections, might provide insights into the etiology of LCLE.

Mesenteric panniculitis in a patient with new onset dermatomyositis.

Mesenteric panniculitis (MP), part of the spectrum of sclerosing mesenteritis, is an often asymptomatic disorder that is characterised by chronic inflammation of abdominal mesentery. We present a case of an 83-year-old woman who presented with proximal muscle weakness and erythematous, photosensitive rash of the face and upper torso and was subsequently diagnosed with dermatomyositis based on skin biopsy, electromyography and muscle biopsy. She had radiographic evidence of panniculitis on CT scan of the abdomen and pelvis for malignancy surveillance, which improved on serial CT scan 3 months after beginning treatment for her underlying dermatomyositis with prednisone and mycophenolate mofetil. Our case highlights that MP can be associated with underlying autoimmune disease. Connective tissue disease could be considered in the differential of MP when other etiologies such as surgery, trauma and malignancy are ruled out.

Drug Reaction with Eosinophilia and Systemic Symptoms (DRESS) Syndrome Secondary to Furosemide: Case Report and Review of Literature.

BACKGROUND DRESS is a rare, life threatening syndrome that occurs following exposure to certain medications, most commonly antibiotics and antiepileptics. While sulfonamide antibiotics are frequently implicated as causative agents for DRESS syndrome, furosemide, a nonantibiotic sulfonamide, has not been routinely reported as the causative agent despite its widespread use. CASE REPORT A 63 year old male who started furosemide for lower extremity edema 10 weeks prior presented with diarrhea, fever of 39.4°C, dry cough and maculopapular rash involving >50% of his body. He self-discontinued furosemide due to concern for dehydration. The diarrhea spontaneously resolved, but he developed hypoxia requiring hospitalization. CT scan demonstrated mediastinal lymphadenopathy and interstitial infiltrates. Laboratory evaluation revealed leukocytosis, eosinophilia and thrombocytopenia. He was treated empirically for atypical pneumonia, and after resuming furosemide for fluid excess, he developed AKI, worsening rash, fever and eosinophilia of 2,394 cell/µL. Extensive infectious and inflammatory work up was negative. Skin biopsy was consistent with a severe drug reaction. Latency from introduction and clinical worsening following re-exposure indicated furosemide was the likely inciter of DRESS. The RegiSCAR scoring system categorized this case as "definite" with a score of 8. CONCLUSIONS We report a case of severe DRESS syndrome secondary to furosemide, only the second case report in medical literature implicating furosemide. Given its widespread use, the potentially life-threatening nature of DRESS syndrome and the commonly delayed time course in establishing the diagnosis, it is important to remember that, albeit rare, furosemide can be a cause of DRESS syndrome.

Melanotic schwannoma arising in association with nevus of Ota: 2 cases suggesting a shared mechanism.

Melanotic schwannoma is a rare markedly pigmented peripheral nerve sheath tumor comprising cells with prominent melanization and schwannian features. The psammomatous variety is associated with Carney complex, a multiple neoplasia syndrome with spotty skin pigmentation. We present the first 2 reported cases of melanotic schwannoma arising in patients with a history of nevus of Ota, a rare dermal melanosis believed to represent a failure of melanocyte migration to the epidermis during embryogenesis. Case 1 involves a 40-year-old woman with a 1.8-cm, deeply pigmented, trigeminal nerve mass and pigmentation of the maxillary sinus mucosa and bone. Case 2 involves a 53-year-old woman with a 1.5-cm mass adjacent to the clavicle. Microscopically, both masses consist of partially encapsulated epithelioid and spindle cells with abundant melanin pigment, arising in association with peripheral nerves. Morphological, immunohistochemical, and ultrastructural features support a diagnosis of melanotic schwannoma. No psammoma bodies are noted, and neither patient exhibits any additional features of Carney complex. Melanotic schwannoma is most often benign but has been associated with malignant behavior in some cases. Distinguishing this nerve sheath tumor from malignant melanoma can be difficult but is of great clinical importance due to differences in prognosis and treatment.