mycophenolic-acid and Skin-Ulcer

The management of systemic sclerosis (SSc) is complex, evolving, and requires a multidisciplinary approach. At diagnosis and throughout the disease course, clinical assessment and monitoring of skin involvement is vital using the modified Rodnan Skin Score, patient-reported outcomes, and new global composite scores (such as the Combined Response Index for Systemic Sclerosis, which also considers lung function). Immunomodulation is the mainstay of skin fibrosis treatment, with mycophenolate mofetil considered first line. Meanwhile vasculopathy-related manifestations (Raynaud's phenomenon, digital ulcers) and calcinosis, require general measures combined with specific pharmacologic (calcium-channel blockers, phosphodiesterase type 5 inhibitors, and prostanoids), nonpharmacologic (digital sympathectomy and botulinum toxin injections), and often multifaceted, management approaches. Patients should be screened at the time of diagnosis specifically for systemic manifestations and then regularly thereafter, with appropriate treatment. Numerous targeted therapeutic options for SSc, including skin fibrosis, are emerging and include B-cell depletion, anti-interleukin 6, Janus kinase, and transforming growth factor β inhibition. This second article in the continuing medical education series discusses these key aspects of SSc assessment and treatment, with particular focus on skin involvement. It is vital that dermatologists play a key role in the multidisciplinary approach to SSc management.

Topics: Adult; Botulinum Toxins; Calcium; Fibrosis; Humans; Janus Kinases; Mycophenolic Acid; Phosphodiesterase 5 Inhibitors; Prostaglandins; Raynaud Disease; Scleroderma, Systemic; Skin Ulcer; Transforming Growth Factor beta

Graft-versus-host disease (GVHD) is a common complication of allogeneic hematopoietic cell transplantation (HCT). In the treatment of chronic GVHD, skin directed therapy, systemic corticosteroids, calcineurin inhibitors (such as cyclosporine (CsA) and tacrolimus), rituximab, mycophenolate mofetil (MMF), extracorporeal photopheresis (ECP) and ruxolitinib are used.. We present an 18 year old male with Philadelphia chromosome positive acute B lymphoblastic leukemia, treated with allogeneic HCT from a full matched sibling donor. The patient had grade 2 chronic cutaneous GVHD resistant to corticosteroids, CsA, MMF, and ECP treatment. Three months after initiation of ruxolitinib therapy, the patient developed skin ulcers on his lower extremities.. The biopsy revealed that the changes were caused by the drug reactions. We suspected ruxolitinib as the likely cause of these ulcerative lesions after evaluating the adverse drug reaction probability scale. The adverse drug score was 4, therefore, ruxolitinib treatment was discontinued. Ulcerative lesions fully recovered after 4 weeks of follow-up.. Ruxolitinib is used in the treatment of chronic GVHD that has been resistant to steroids and other salvage therapies. In our case, ruxolitinib was used as a salvage therapy in a patient who had refractory chronic skin GVHD. Ruxolitinib-related skin lesions with ulcers of lower extremities and whole body erythematous skin lesions were reported previously in patients with myelofibrosis. The pathophysiology of ruxolitinib related skin ulcers is unknown. Skin changes of patients using ruxolitinib should be closely monitored, and newly developing lesions should be suspected of being drug-related and biopsied.

Topics: Adolescent; Adrenal Cortex Hormones; Graft vs Host Disease; Hematopoietic Stem Cell Transplantation; Humans; Male; Mycophenolic Acid; Nitriles; Pyrazoles; Pyrimidines; Skin Ulcer

Topics: Administration, Intravenous; Administration, Oral; Adolescent; Antibiotics, Antineoplastic; Drug Therapy, Combination; Female; Glucocorticoids; Humans; Immunologic Factors; Induction Chemotherapy; Lupus Nephritis; Male; Methylprednisolone; Mycophenolic Acid; Prednisolone; Prolidase Deficiency; Rituximab; Skin Ulcer; Treatment Outcome

Pulmonary arterial hypertension (PAH) and interstitial pneumonia (IP) are relatively rare complications of systemic lupus erythematosus (SLE) and are associated with a poor prognosis. Overcoming these complications is a challenge for improving the prognosis.. In SLE complicated by PAH/IP, reports on the efficacy of MMF are scarce, and our findings suggested that MMF may be a treatment option in such cases.

Topics: Adult; Female; Humans; Hypertension, Pulmonary; Immunosuppressive Agents; Lung Diseases, Interstitial; Lupus Erythematosus, Systemic; Mycophenolic Acid; Pulmonary Arterial Hypertension; Skin Ulcer; Tomography, X-Ray Computed

Antimelanoma differentiation-associated gene 5 (MDA-5) dermatomyositis is a subtype of dermatomyositis that is associated with rapidly progressive interstitial lung disease (RP-ILD), as well as with a variety of cutaneous manifestations. Patients with MDA-5 dermatomyositis tend to have a poor prognosis that is often attributed to the high rates of concurrent RP-ILD. Given the severity of disease, early diagnosis and aggressive management is pivotal. We present a case of a 40-year-old woman diagnosed with MDA-5 dermatomyositis who presented with weakness, painful cutaneous ulcerations and interstitial lung disease. She was treated with monthly intravenous Ig (IVIg), weight-based prednisone and mycophenolate mofetil (MMF). After approximately 2 years of treatment, her interstitial lung disease remains stable and she has had significant improvement in weakness and cutaneous ulcerations. Our case provides evidence for early and aggressive treatment of MDA-5 dermatomyositis with a combination of weight-based prednisone, MMF and IVIg.

Topics: Adrenal Cortex Hormones; Adult; Dermatomyositis; Female; Glucocorticoids; Humans; Immunoglobulins, Intravenous; Interferon-Induced Helicase, IFIH1; Lung Diseases, Interstitial; Mycophenolic Acid; Prednisone; Skin Ulcer

Topics: Adrenal Cortex Hormones; Aged; Anti-Bacterial Agents; Colitis, Ulcerative; Humans; Male; Mycophenolic Acid; Pyoderma Gangrenosum; Skin Ulcer; Surgical Stomas; Treatment Outcome

Topics: Aged; Animals; Anti-Bacterial Agents; Duodenal Diseases; Endoscopy, Digestive System; Fishes; Graft Rejection; Humans; Immunosuppressive Agents; Kidney Transplantation; Male; Multiple Pulmonary Nodules; Mycobacterium Infections, Nontuberculous; Mycophenolic Acid; Pets; Prednisone; Severity of Illness Index; Skin Ulcer; Tacrolimus; Tomography, X-Ray Computed; Zoonoses

Topics: Clone Cells; Diagnosis, Differential; Epstein-Barr Virus Infections; Herpesvirus 4, Human; Humans; Immune Tolerance; Immunocompromised Host; Immunosuppression Therapy; Immunosuppressive Agents; Lymphoma, B-Cell; Male; Middle Aged; Mycophenolic Acid; Skin Ulcer

Topics: Chronic Disease; Diagnosis, Differential; Female; Granuloma, Lethal Midline; Humans; Immunocompromised Host; Leishmania; Leishmaniasis, Cutaneous; Lupus Erythematosus, Systemic; Lymphoma, T-Cell, Cutaneous; Middle Aged; Mycophenolic Acid; Nose Diseases; Opportunistic Infections; Prednisone; Sjogren's Syndrome; Skin Ulcer