Sarcoidosis is an idiopathic inflammatory disorder that is commonly treated with glucocorticoids. An imprecise understanding of the immunologic changes underlying sarcoidosis has limited therapeutic progress. Here in this open-label trial (NCT03910543), 10 patients with cutaneous sarcoidosis are treated with tofacitinib, a Janus kinase inhibitor. The primary outcome is the change in the cutaneous sarcoidosis activity and morphology instrument (CSAMI) activity score after 6 months of treatment. Secondary outcomes included change in internal organ involvement, molecular parameters, and safety. All patients experience improvement in their skin with 6 patients showing a complete response. Improvement in internal organ involvement is also observed. CD4+ T cell-derived IFN-γ is identified as a central cytokine mediator of macrophage activation in sarcoidosis. Additional type 1 cytokines produced by distinct cell types, including IL-6, IL-12, IL-15 and GM-CSF, also associate with pathogenesis. Suppression of the activity of these cytokines, especially IFN-γ, correlates with clinical improvement. Our results thus show that tofacitinib treatment is associated with improved sarcoidosis symptoms, and predominantly acts by inhibiting type 1 immunity.
Pyoderma gangrenosum (PG) is a rare inflammatory skin disease of unknown origin. As with other vaccines, COVID-19 vaccines have been associated with many cutaneous reactions. Although COVID-19 vaccination is crucial, it is important for dermatologists and other physicians to be aware of the possible cutaneous reactions that can occur following COVID-19 vaccination. In this report, we describe a 73-year-old woman with a personal history of PG who experienced a recurrence after receiving her second dose of the tozinameran vaccine. Although extremely rare, flares of other inflammatory dermatoses, including lichen planus, have been reported following COVID-19 vaccination. Here we discuss the overlap in pathogenesis of PG and COVID-19, proposing possible mechanisms behind this rare phenomenon.
Drug-induced Eosinophilia and Systemic Symptoms (DRESS), Stevens-Johnson Syndrome (SJS), and Toxic Epidermal Necrolysis (TEN) are rare but life-threatening immune-mediated drug reactions known as Severe Cutaneous Adverse Reactions (SCARs). These severe drug reactions have been associated with many commonly prescribed medications, including sulfonamides, allopurinol, carbamazepine, and several antiepileptic drugs including lamotrigine.1 Although the risk of these adverse events is recognized by many medical providers, the risk may be overlooked when prescribing lamotrigine for mood disorders. Review of the literature and the experience of these cases suggest that the risk of lamotrigine-associated SCARs is increased when starting lamotrigine at high initial doses. Here we present and discuss two cases of SCARs attributed to high-dose lamotrigine prescribed for mood disorders. A third patient also presented with a SCAR related to high-dose lamotrigine prescribed for a mood disorder during this time but was lost to follow-up and was not reachable. All three patients presented to our hospital system from 2019-2020. Due to this clinical experience, we recommend that pharmacists and prescribers alike be alerted of the risk of severe cutaneous drug reactions when lamotrigine is prescribed, particularly at initial doses greater than 25 mg.
Granuloma annulare is a relatively common inflammatory skin disease, yet patient-centered impacts of GA have not been thoroughly investigated, including the potential psychosocial and symptomatic burden, patients’ motivation for treatment, and patients’ perception of usefulness of commonly used medications. We conducted a survey of greater than 900 patients with GA and found an unexpectedly significant impact of GA on the quality of life, a generally poor reported response to commonly used therapies, and that symptoms such as itch and discomfort are not uncommon and may be the primary motivator for seeking treatment. More effective therapies and an understanding of their effect on quality of life is needed.
Background: Dermatofibrosarcoma protuberans (DFSP) is an uncommon, locally aggressive malignancy with wide local excision (WLE) or Mohs micrographic surgery (MMS) representing the treatment of choice. This article illustrates the experience of a single academic institution in treating DFSP with MMS and adds two particularly large, difficult closures of the glabella/central forehead and sternum to the body of literature.Objective: To report the results of 15 patients with DFSP treated with MMS over a five-year period by a single Mohs surgeon at the University of Arkansas for Medical Sciences (UAMS).Methods: A total of 15 patients between the ages of 16 and 80 years were diagnosed with DFSP and treated with MMS and were contacted in October 2021 to assess for recurrence.Results: None of the 15 patients had a recurrence of DFSP following MMS, with a mean follow-up interval of 22.4 months and an average of 1.93 Mohs layers required for tumor clearance.Conclusion: This experience reaffirms that MMS is an effective treatment for DFSP and adds additional examples of closure techniques of large, ovoid surgical defects on the glabella/central forehead and sternum to the literature.
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