Cutaneous Crohn disease presenting as "knife-edged" ulcers: a case report.

BACKGROUND: CCD presents as non-caseating granulomas within the skin at a site distant from the GI tract. CCD is a debilitating extraintestinal sequela of CD that can sometimes precede its GI manifestations. In the absence of GI symptoms, the histopathologic and clinical features of CCD can present as a variety of inflammatory skin conditions that can range from ruptured follicle-associated granulomas to cutaneous ulcerations. While a variety of therapeutic options for patients with CCD and concurrent luminal CD have been described in the literature, there is no standard treatment algorithm for the management of refractory CCD with limited or covert GI involvement. CASE REPORT: The authors discuss the case of a 33-year-old female who presented to the wound care clinic with multiple "knife-edged" cutaneous ulcerations involving the intertriginous spaces, found to be consistent with CCD. Her original cutaneous symptoms and diagnosis manifested with minimal GI involvement and responded to IVIG treatment. CONCLUSIONS: This case supports the inclusion of CCD in the differential diagnosis in patients with knife-edged granulomatous skin lesions in intertriginous locations. This clinical condition may present in the setting of no or limited GI symptoms. The management of CCD and a proposed treatment algorithm are also presented.

Nonuremic Calciphylaxis: A Rare and Unexpected Diagnosis of Necrotic Ulcers.

Calciphylaxis is a rare and devastating condition found almost exclusively in patients with end-stage renal disease. Nonuremic calciphylaxis, an even more rare diagnosis, occurs in patients with preserved kidney function. We present a fatal case of nonuremic calciphylaxis with delayed and unexpected diagnosis despite early biopsy and testing. The patient presented with a 2-month history of painful ulceration to the left leg. Early biopsy was negative for calciphylaxis. Laboratory tests were negative for renal disease and autoimmune disorders. There was elevated parathyroid hormone (96 pg/mL) 3 months after initial presentation and documented cobalamin deficiency. The patient went on to develop wounds to both legs and her thighs. A second biopsy of a left thigh wound by means of the dermatology service revealed calciphylaxis. The purpose of this case report is to raise awareness of calciphylaxis as a differential diagnosis for chronic necrotic skin ulcers, especially in patients with preserved renal function and those on warfarin therapy.

Eligibility Criteria for Active Ulcerative Pyoderma Gangrenosum in Clinical Trials: A Delphi Consensus on Behalf of the UPGRADE (Understanding Pyoderma Gangrenosum: Review and Assessment of Disease Effects) Group.

At present, there are no standardized guidelines for determining patient eligibility for pyoderma gangrenosum (PG) clinical trials. Thus, we aim to determine which clinical features, histopathological features, or laboratory features should be included in active ulcerative PG clinical trial eligibility criteria for treatment-naïve patients and patients already treated with immunomodulating medications (treatment-exposed patients). This study employed 4 rounds of the Delphi technique. Electronic surveys were administered to 21 international board-certified dermatologists and plastic surgeon PG experts (June 2022-December 2022). Our results demonstrated that for a patient to be eligible for a PG trial, they must meet the following criteria: (i) presence of ulcer(s) with erythematous/violaceous undermining wound borders, (ii) presence of a painful or tender ulcer, (iii) history/presence of rapidly progressing disease, (iv) exclusion of infection and other causes of cutaneous ulceration, (v) biopsy for H&E staining, and (vi) a presence/history of pathergy. These criteria vary in importance for treatment-naïve versus treatment-exposed patients. Given the international cohort, we were unable to facilitate live discussions between rounds. This Delphi consensus study provides a set of specific, standardized eligibility criteria for PG clinical trials, thus addressing one of the main issues hampering progress toward Food and Drug Administration approval of medications for PG.

Trigeminal trophic syndrome, a rare and often overlooked cause of facial ulceration: a case report and literature review.

Trigeminal trophic syndrome (TTS) is an uncommon and relatively unknown cause of facial ulceration that occurs after damage to the trigeminal nerve. It characteristically involves non-healing facial ulceration(s) with accompanying anesthesia, paresthesia, and dysesthesia along the distribution of a trigeminal dermatome. The ulcerations are believed to be self-induced in response to paresthesia. The disease is most common in middle-aged women, manifesting as a unilateral crescent-shaped ulceration on the ala nasi, with sparing of the nasal tip. The diagnosis is clinical and mostly based on exclusion of other possible causes of facial ulcerations, with emphasis on neoplasms, infection-associated vasculitis, and factitial disorders. There are no specific histological or laboratory signs. There is no standard treatment protocol; however, a number of different successful strategies have been reported, including pharmaceutical and surgical interventions, transcutaneous nerve stimulation, and simple occlusion dressings. Due to the self-inflicted nature of this disorder, the cornerstone of management is patient education with behavioral modification. Here, we report a case of TTS following herpes zoster ophthalmicus and review the current literature on this subject.

Cutaneous Deep Ulcerations as Initial Presentations of Granulomatosis with Polyangiitis: Two Case Reports and Differential Diagnosis.

Background: Granulomatosis with polyangiitis (GPA) is an antineutrophil-cytoplasmic-antibody (ANCA)-associated small-vessel vasculitis characterized by necrotizing granulomatous inflammation. Symptoms of skin involvement can appear in 30-50% of patients with GPA, and may present as the initial presentation. Case Presentation: We describe two patients who presented with multiple deep, large, nonhealing skin ulcers postoperatively with purulent drainage and fever. Both patients were diagnosed with GPA after an extensive evaluation, including histopathology. Infectious, connective tissue disease and malignant etiologies were excluded. Their cANCA and PR3-ANCA levels were positive. Patient 2 was diagnosed early and recovered well after treatment with corticosteroids and rituximab; however, Patient 1 had a poor prognosis due to a long disease course. Conclusions: Diseases with multiple deep, large skin ulcers and fever can be infectious or noninfectious. Atypical manifestations may lead to missed diagnosis and misdiagnosis. GPA may initially present in a localized form before progressing to a generalized disease. The two cases we have highlighted will prompt clinicians to nevertheless call for a low threshold for diagnosis.

Association between oxygen delivery and digital ulcers in systemic sclerosis.

OBJECTIVE: Tissue hypoxia due to microvasculopathy is the main cause of digital ulcers (DUs) in systemic sclerosis (SSc). Reduced oxygen delivery (DO2) to the tissues may also contribute to the development of DU. This study was conducted to investigate the association between DO2 and DUs in patients with SSc. METHODS: In all, 111 patients and 30 healthy controls were enrolled. DO2 was calculated by using the formula; DO2 = Cardiac output × arterial oxygen saturation (SpO2) × serum haemoglobin level × 1.39 × 10. Both right index finger SpO2 measurements (index-SpO2) and highest value of SpO2 (maximum SpO2) obtained among the fingers of the subjects were used for the calculations and DO2 results were adjusted both for weight and body surface area (BSA). RESULTS: Mean DO2 was lower in SSc patients as compared to controls in all 4 different calculations but the difference was only statistically significant when using index-SpO2 and adjusting for BSA (498 mL/min/m2 vs 549 mL/min/m2, p = 0.03). There was a strong positive correlation between cardiac output and DO2 calculated by using the index-SpO2 (r = 0.903; p < 0.001). Of the SSc patients, 46 (41.4 %) had DUs within the last 12 months. Patients with DUs had higher mean mRSS, lover mean FVC and more frequently diffuse disease, interstitial lung disease, anti-SCL70 antibody positivity (p < 0.05 for all). No difference was observed in DO2 among DU positive or DU negative groups by any calculation (p > 0.05 for all). CONCLUSIONS: DO2 in SSc patients seems to be lower than healthy controls. However, DO2 is similar between the patients with and without DUs. Our results suggest that the contribution of DO2 is negligible to the development of DU and support the major role of microvasculopathy in SSc patients with DUs.

Choroid, a unique site for metastasis in Marjolin's ulcer.

Marjolin is an aggressive malignancy, which shows initial local aggression, followed by the rapid regional and distant dissemination during the latter part of its growth. Metastasis to the uveal tract, although infrequent, is usually secondary to the malignancies of the breast and lung. Marjolin's ulcer metastasizing to choroid is quite rare and unheard of. We intend to report one such case here. Here, we present a case report of a 55-year-old male, who developed Marjolin's ulcer over a previously injured left lower limb. He developed recurrence and re-recurrences requiring multiple surgeries, radiation, and chemotherapy cycles. He eventually developed disseminated systemic metastases. Over the course of his disease, he reported eye symptoms of pain, blurring of vision, and field defect in the left eye. On further evaluation with imaging and fundoscopy, a metastatic deposit was identified in the choroid of the left eye. Metastasis to the uveal tract is an unusual unreported event in the disease progression of Marjolin's ulcer. The treatment is usually palliative. It heralds the terminal stage of the disease, with inevitable death.

Get It Off Your Chest: A Narrative Review of Breast Ulcers.

GENERAL PURPOSE: To provide comprehensive information about breast ulcers to facilitate accurate diagnosis and treatment of these lesions. TARGET AUDIENCE: This continuing education activity is intended for physicians, physician assistants, nurse practitioners, and nurses with an interest in skin and wound care. LEARNING OBJECTIVES/OUTCOMES: After participating in this educational activity, the participant will:1. Differentiate common wound complications after mastectomy.2. Identify the clinical manifestations of various types of breast ulcers.3. Explain appropriate pharmacologic and nonpharmacologic treatment options for various types of breast ulcers.

Cutaneous breast ulcers are uncommon but important encounters in clinical practice. Myriad causes may introduce ulcers in the breast tissue. Women are more prone to breast ulcers than men because of having heavier breast fatty tissue. Thorough medical history may easily reveal the underlying etiology; however, a tissue biopsy is often required to rule out other potential causes. The clinical presentation varies based on the underlying etiology, but some clues include surgical scars for postoperative wound dehiscence or well-defined violaceous borders with undermined ulcer base in pyoderma gangrenosum. In this article, the authors divide breast cancers into two major groups: with and without underlying mass. Depending on the underlying etiology, treatment may involve topical medications; optimal wound care; systemic medications such as antibiotics, immunosuppressive medications, or biologics; surgery; or a combination of all of the above. This article aims to shed light on a less frequent anatomic location of ulcers and provide advice to clinicians to facilitate accurate diagnosis and treatment.

[Emphasis on multidisciplinary collaboration in clinical diagnosis and treatment of autoimmune disease-related ulcers].

The global prevalence and incidence of autoimmune diseases are increasing year by year, and the autoimmune diseases have become a major threat to public health. In the progression of the diseases, persistent and complex abnormal immune responses often lead to long-term unhealed skin ulcers, which not only affect the life quality of patients, but also lead to the aggravation of primary diseases. Therefore, doctors in burn surgery and other wound repair surgeries should pay attention to the understanding of autoimmune diseases. In the treatment of autoimmune disease-related ulcers, it is recommended to formulate a unified treatment plan according to the law of occurrence and development of the diseases, and multidisciplinary cooperation is needed to accelerate wound healing and improve the quality of wound healing.

Trigeminal trophic syndrome: An unusual nasal ulceration. A case report and review of the literature.

Trigeminal trophic syndrome is a rare condition characterized by self-inflicted persistent facial ulceration. It is believed to be consequent to central or peripheral insult to trigeminal nerve, which may have taken place even years before the ulcer development. The aggression to the nerve pathway causes dysesthesias in the trigeminal dermatomes that induce a self-mutilating behavior, with repetitive pinching or scratching in order to mitigate the altered sensation. Due to associated skin anesthesia, the patient does not interrupt manipulation of the affected area despite severe skin necrosis. Ulceration typically occurs in the ala nasi and may resemble other more common cutaneous diseases, such as tumors or infections. Given that this condition is not included in our daily clinical practice, the risk is that of a diagnostic delay with devastating functional and esthetic facial consequences. We present the case of a patient with a history of meningioma resection who developed this syndrome and we have reviewed the published literature to provide an update on the etiopathogenesis, diagnosis and treatment of this rare condition.

Serum resistin is predictive marker of development of new digital ulcers in systemic sclerosis.

Systemic sclerosis (SSc) is autoimmune disease characterized by endothelial dysfunction and microvascular damage. Resistin has been implied in microvascular dysfunction. Objective of this study is to evaluate the association between baseline resistin and development of new digital ulcers (DUs) in SSc patients. At baseline, serum resistin has been assessed in 70 female SSc patients and 26 healthy controls (HC). In SSc patients, clinical assessment was performed at baseline and after a 52-weeks follow-up. Serum resistin level was increased in SSc patients compared to HC [5.89 ng/ml (2.5 ng/ml-8.1 ng/ml) vs 2.3 ng/ml (0.4 ng/ml-2.4 ng/ml), p = 0.0004)]. Resistin was lower (p = 0.005) in SSc patients with early capillaroscopic pattern than patients with active or late capillaroscopic pattern [2.49 ng/ml (0.89 ng/ml-5.81 ng/ml) vs 7.11 ng/ml (3.48 ng/ml-11.35 ng/ml) and 6.49 ng/ml (3.35 ng/ml-8.87 ng/ml), respectively]. After a 52-weeks follow-up, 34 (48.6%) patients developed new DUs. Median serum resistin was significantly higher in patients with new DUs than in patients without new DUs [6.54 ng/ml (3.35 ng/ml-11.02 ng/ml) vs 4.78 ng/ml (1.06 ng/ml-7.6 ng/ml), p = 0.019]. Kaplan-Meier curves show a significantly reduced free survival from DUs in patients with increased resistin (p = 0.002). In multivariate analysis, resistin is associated with the development of new DUs. Increased serum resistin level is a predictive marker of new DUs in SSc.

Extensive Cutaneous Ulceration of the Scalp - Unknown Disease Entity or Dermatitis Artefacta?

Psychological disturbances and emotional stress events may elicit a wide spectrum of skin disturbances which are classified as dermatitis artefacta. This diagnosis should be taken into consideration when symptoms coexist in a bizarre pattern or indicate at least several distinct skin pathologies while laboratory tests remain inconclusive. We present a case of dermatitis artefacta which produced very extensive loss of the scalp. Our intention was to show difficulties in diagnostic management of this recurrent and complex psychiatric disorder which may inconvenience clinicians. Neither laboratory tests (including bacteriology) nor X-ray of the skull identified any significant pathology. Although histopathology excluded skin malignancy, it showed an unspecific pattern not attributable to the most probable skin conditions like pyoderma gangrenosum or infection. Psychiatric consultation was inconclusive. Despite undetermined diagnosis, the patient was eligible for reconstructive surgery, which restored his scalp coverage. Different skin conditions may share very similar spectra of clinical symptoms, and even deep medical investigation does not always enable us to define the observed condition. However, both laboratory and imaging tests are necessary to exclude infections or potential malignancies before the diagnosis of dermatitis artefacta is established, whereas psychiatric consultation may or may not identify mental issues.

Tofacitinib Treatment of Refractory Cutaneous Leukocytoclastic Vasculitis: A Case Report.

Introduction: To date, there is no treatment with proven efficacy for cutaneous leukocytoclastic vasculitis (CLV). Several reports have suggested that CLV responds favorably to corticosteroids, colchicine, nonsteroidal anti-inflammatory drugs (NSAIDs), azathioprine, and hydroxychloroquine (HCQ). To the best of our knowledge, the oral small molecule Janus kinase inhibitor, tofacitinib, plays an important role in the treatment of autoimmune and inflammatory diseases. Therefore, tofacitinib may be a prospective therapy in patients with CLV. Case Presentation: A 29-year-old woman presented to our hospital with a 5-year history of symmetric skin lesions mainly affecting both lower extremities. The results for anti-neutrophil cytoplasmic antibodies (ANCA), anti-extracted nuclear antigens (ENA) autoantibodies, anti-double-stranded deoxyribonucleic acid (dsDNA) antibodies, and antinuclear antibodies (ANA) were all negative. The definite diagnosis of CLV was determined by a skin biopsy. However, the patient exhibited a poor response to prednisone, HCQ, methotrexate, colchicine, azathioprine, and tripterygium wilfordii polyglycoside tablets (TGTs) treatments. She was then treated with oral tofacitinib (5 mg twice daily) and oral prednisone (25 mg daily). Outcomes: Her skin lesions gradually improved over a period of 4 weeks. Two months later, the skin ulcers completely resolved. No evidence of recurrence of skin ulcers was observed during a 6-month follow-up. Conclusion: We present the first case of a female patient receiving short-term tofacitinib therapy for refractory CLV. Tofacitinib may be a promising oral alternative for patients with CLV. However, its efficacy and safety require further appraisal through clinical trials.

An Eschar-like souvenir from a journey to Colombia: Ecthyma gangrenosum as a differential diagnosis of tropical diseases in immunocompromised patients - a case report.

BACKGROUND: Ecthyma gangrenosum (EG) is a cutaneous infectious disease characterized by eschar-like skin ulcers typically caused by Pseudomonas aeruginosa. Here, we report a case of relapsing EG in a patient who had returned from a trip to Colombia, thus establishing EG as an important differential diagnosis of tropical diseases, and demonstrating that even long-term antibiotic treatment can result in only partial remission of EG. CASE PRESENTATION: A 77-year-old man with underlying chronic lymphocytic leukemia (CLL) on ibrutinib treatment was admitted because of a superinfected mosquito bite on the left ear and multiple partially necrotic skin lesions disseminated all over the entire body five days after returning from a trip to Colombia. The initial clinical suspicion of a tropical disease (leishmaniosis, systemic mycosis, or others) could not be confirmed. During the diagnostic workup, microbiological cultures of the skin biopsies and bronchoalveolar lavage revealed Pseudomonas aeruginosa, leading to a diagnosis of EG. Initial antibiotic treatment resulted in partial remission. However, the patient had to be re-admitted due to a relapse 3-4 weeks after the first episode. Finally, the patient was successfully treated with a combined approach consisting of antibiotics, recurrent surgical incisions, and administration of immunoglobulins. CONCLUSIONS: In conclusion, EG should be considered as a differential diagnosis in immunosuppressed patients presenting with eschar-like skin ulcers. A combined treatment approach seems to be the best choice to achieve clinical cure and avoid relapse.