Urticarial Vasculitis.

Urticarial vasculitis is a rare autoimmune disorder characterized by persistent edematous papules and plaques on the skin that last longer than 24 hours, often accompanied by systemic symptoms such as joint pain and fever. Unlike common urticaria, this condition involves inflammation of small blood vessels, leading to more severe and long-lasting skin lesions with a tendency to leave a bruiselike appearance. Diagnosis is challenging and may require a skin biopsy. Associated with underlying autoimmune diseases, treatment involves managing symptoms with medications such as antihistamines and corticosteroids, addressing the immune system's dysfunction, and treating any concurrent autoimmune conditions.

Cyclosporine in the Treatment of Drug Reaction With Eosinophilia and Systemic Symptoms Syndrome: Retrospective Cohort Study.

BACKGROUND: Drug reaction with eosinophilia and systemic symptoms (DRESS) syndrome is a severe, life-threatening reaction to a culprit drug that frequently involves end-organ damage. Corticosteroids are the first-line treatment for DRESS syndrome; however, corticosteroids may be contraindicated in certain patient populations. There are currently only 54 cases detailing the use of cyclosporine for the treatment of DRESS syndrome reported in the literature. OBJECTIVE: The aim of this case series was to examine the treatment of DRESS syndrome with cyclosporine in a large patient cohort by aggregating time to symptom resolution, recurrence rate, and treatment dose and duration. METHODS: This study was a retrospective cohort study. Patients diagnosed with DRESS syndrome by a board-certified dermatologist and treated at the University of Colorado Hospital from 2015 to 2019 were included. RESULTS: Our inclusion criterion was met by 19 occurrences of DRESS syndrome. With a short course of cyclosporine, 17 of 19 patients in our cohort (89%) had resolution of symptoms (mean treatment length of 5.26 days). DRESS syndrome's relapse after treatment with cyclosporine occurred in 3 of 19 (16%) occurrences of the cohort. CONCLUSIONS: Our study supports the use of cyclosporine in the treatment of DRESS syndrome, particularly in patients who are unable to sustain prolonged immunosuppression. Further research is necessary to compare the efficacy of cyclosporine to the current standard of care in a larger study population and investigate long-term outcomes.

A Review of Leishmaniasis: Current Knowledge and Future Directions.

PURPOSE OF REVIEW: The goal of this review is to summarize the current knowledge of the epidemiology, clinical manifestations, diagnosis, and treatment of cutaneous, mucosal, and visceral leishmaniasis. We will describe the most recent findings and suggest areas of further research in the leishmaniasis field. RECENT FINDINGS: This article reviews newer leishmaniasis tests (including rapid diagnostic tests using rK39 antibodies), vaccine candidates, and updated treatment recommendations. SUMMARY: While leishmaniasis is a complex disease, learning the prominent clinical manifestations and major parasite species can guide the recommendations for diagnosis and treatment.

Dermatitis Artefacta, a Form of Factitial Disorder Imposed on Self, Misdiagnosed as Pyoderma Gangrenosum for Eight Years.

Dermatitis artefacta is a rare psychological disorder in which patients self-inflict cutaneous lesions to satisfy an emotional need. Due to the nature of this disease, patients can present with a wide array of sometimes very severe skin lesions. Here, we describe a case of dermatitis artefacta initially misdiagnosed as pyoderma gangrenosum and treated as such for eight years. The patient reported a long history of cutaneous ulcers on her extremities and trunk, with resultant extensive scarring. Upon presentation, she displayed rapidly progressing necrotizing skin lesions on her bilateral distal lower extremities. Both the skin manifestations and histologic sections were extremely atypical for pyoderma gangrenosum leading to extensive medical records review and subsequent diagnosis of dermatitis artefacta. This case represents the importance of the timely recognition and treatment of dermatitis artifacta to prevent its progression to severe harm and even death.

Addisonian-Like Hyperpigmentation as an Indicator of Uncontrolled Congenital Adrenal Hyperplasia.

A 20-year-old man of Indo-Malaysian ancestry presented with a complaint of increased facial pigmentation that he first noticed at age 13. He had congenital adrenal hyperplasia (21-hydroxylase deficiency, salt-wasting variant; OMIM 201910), diagnosed during infancy. Glucocorticoid and mineralocorticoid therapy was started at that time, but he had several episodes of salt craving during adolescence. During the past 7 years, the degree of facial pigmentation waxed and waned but never returned to baseline of early adolescence. Progressive skin darkening was also observed in annual family photos, which also showed a vast difference in skin tones between the patient and other members of his immediate family.

Limb-Threatening Arterial Thrombosis in a Patient with Eosinophilic Granulomatosis with Polyangiitis.

Eosinophilic granulomatosis (EGPA), or Churg-Strauss syndrome, is a rare and necrotizing systemic vasculitis, which affects small-to-medium-sized vessels and often manifests with severe asthma and eosinophilia. We report a case of a 72 year-old male with a two-year lung-biopsy proven history of EGPA who presented with retiform purpura and patchy necrosis on his bilateral shins, which progressed to sharply demarcated, stellate ulcerations with surrounding erythema within two weeks. Laboratory work up revealed elevated anti-Cardiolipin IgM, rheumatoid factor, erythrocyte sedimentation rate, and C-reactive protein, although P-neutrophil cytoplasmic antibody (P-ANCA) and C-neutrophil cytoplasmic antibody (C-ANCA) were negative. Vascular studies revealed long anterior tibial and dorsalis pedis artery occlusion and severe small vessel disease in plantar digital arteries. Despite treatment with intravenous cyclophosphamide, pulse-dose methylprednisolone, and pentoxifylline, the patient experienced disease progression and limb threatening arterial thrombosis. This case highlights the importance of vascular and neuropathic sequelae that may result from untreated or undertreated EGPA in P-ANCA-negative patients without active pulmonary symptoms.

An unusual presentation of perforating metastatic calcinosis cutis.

A 37-year-old white man diagnosed with multiple myeloma was admitted for respiratory distress. He was found to have hypercalcemia (13.1 mg/dL), hyperphosphatemia (7.2 mg/dL), hyponatremia (121 mEq/L), and acute renal failure (serum urea nitrogen, 116 mg/dL; creatinine, 4.9 mg/dL). On hospital day 7, skin lesions over his elbows, wrists, and fingers were noticed and the dermatology department was consulted. Physical examination revealed 0.5- to 3.0-cm erythematous denuded patches with yellow crusts at the periphery that were localized to the bilateral interdigital web spaces, medial elbows, and volar wrists (Figure 1 and Figure 2). No oral mucosal or conjunctival findings were present. Results from skin biopsies of the lesions revealed focal epidermal necrosis with zones of amorphous basophilic material that focally appeared to perforate the epidermis (Figure 3). Results from Congo red and Verhoeff-van Gieson stains were negative, and results from Von Kossa stain were positive, confirming the perforating material as calcium. Ten days after initial dermatology examination, the patient's respiratory distress worsened and he subsequently died. Autopsy revealed diffuse pulmonary calcinosis and renal tubular calcification. As a result of the autopsy findings, the patient was diagnosed with perforating metastatic calcinosis cutis.