We report a case of metastatic amelanotic melanoma

We report a case of metastatic amelanotic melanoma with BRAFi V600E mutation and unknown primary cancer as the initial presentation. The patient presented with neutrophilic panniculitis 1 week after treatment with vemurafenib. These lesions subsided after reduction of dosage with low-dose steroid and nonsteroid anti-inflammatory drug use. To the best of our knowledge, this is the first reported case of neutrophilic panniculitis associated with vemurafenib for melanoma treatment in Taiwan.
Introduction Tripterygium wilfordii polyglycosidium (T2) is a traditional Chinese medicine widely used in China in organ transplantation and for the treatment of inflammatory and autoimmune diseases, neurodegenerative diseases, and some tumors. Bullous systemic lupus erythematosus (BSLE) is a rare and severe category of systemic lupus erythematosus (SLE) associated with relative treatment difficulty. Here we report two female BSLE patients treated effectively with T2 and a low dose of corticosteroids.
Case reports
Discussion BSLE is a form of subepidermal autoimmune bullous dermatosis, a rare entity that accounts for less than 5% of SLE. Females in their 20s to 40s have high chances of being diagnosed with BSLE. Etiology is unclear. Genetics and autoimmunity for VII collagen in the basement membrane zone may take part in the separation of the nrf2 keap1 and dermis. The most common presentation of BSLE is asymptomatic tense blisters confined to sun-exposed areas or sometimes present in a more widespread distribution. Multisystem damage, especially lupus nephritis, is also common. The histopathological manifestation of vesicles is similar to that of dermatitis herpetiformis, characterized by a subepidermal split and neutrophilic infiltration of the papillary dermis. DIF is positive for deposition of immunoglobulin G, M, or A along the basement membrane zone. Both our patients fit the criteria for SLE of the Systemic Lupus International Collaborating Clinics, presenting with bullous cutaneous lupus and synovitis, and testing positive for ANA, anti-Sm, anti-dsDNA, and low complement levels. Case 2 also suffered oral ulcers. Common treatment options include dapsone, high-dose corticosteroids, antimalarial drugs, immunosuppressants, and biologic drugs. BSLE responds well to dapsone, but purchasing dapsone in China is difficult because of a halt in production, so corticoid hormones at a high dose or in combination with immunosuppressants are the primary option. However, BSLE patients often resist these treatments. T. wilfordii Hook F. (TwHF), also known as thunder god vine or lei gong teng, is a traditional Chinese herb that has attracted great interest around the world. The extract from its woody root, T. wilfordii multiglycoside, also known as T2 or TII, is one of the most widely used TwHF preparations in China. T2 contains different concentrations of therapeutically active compounds, including diterpenoids, alkaloids, triterpenoids, and glycosides. Diterpenoids, especially triptolide and tripdiolide, play the major anti-inflammatory and immunosuppressive roles by inhibiting the proliferation and inducing the apoptosis of T and B cells; reducing the production of cytokines, transcription factors, and adhesion molecules; and suppressing pro-inflammatory enzymes. T2 has been widely used in China as a cheap and effective drug to treat a broad spectrum of dermatoses, such as generalized eczema, atopic dermatitis, vasculitis, psoriasis, SLE, and pemphigus. The main side effects include digestive tract reactions, leukocytopenia, hepatic and renal dysfunction, antifertility, and paramenia, which are mostly reversible if T2 is discontinued in time. Therefore, regular laboratory examination of the blood cell count and the liver and renal function indices is recommended. Both patients responded distinctly to T2 combined with low-dose prednisone, which showed efficacy in 1–2 weeks and exhibited good control in half a year. Case 2 was complicated at the first visit by urine protein, which tested negative after 1 week of treatment and has remained negative since, so it is unclear whether kidney damage really existed or the treatment just dramatically worked. In clinical trials, T2 is frequently combined with steroids for treating systemic diseases. The combined treatments usually produce better symptomatic reduction with fewer adverse reactions than any single high-dose treatment. Triptolide binds to the glucocorticoid receptor. The glucocorticoid receptor–triptolide complex cannot activate glucocorticoid-responsive genes and may suppress the transcriptional activity of nuclear factor kappa B (NF-κB) and activator protein 1 (AP-1), producing a combination of anti-inflammatory and steroid-sparing effects. A hypothesis has been proposed that a combined remedy of TwHF and corticosteroids enhances the therapeutic efficacy and reduces the side effects of the corticosteroids.