CRP and sCD25 help distinguish between adult-onset Still's disease and HLH.

OBJECTIVE: Adult-onset Still's disease (AOSD) and secondary hemophagocytic lymphohistiocytosis (sHLH) are both hyperferritinemic cytokine storm syndromes that can be difficult to distinguish from each other in hospitalized patients. The objective of this study was to compare the inflammatory markers ferritin, D-dimer, C-reactive protein (CRP), and soluble CD25 (sCD25) in patients with AOSD and sHLH. These four markers were chosen as they are widely available and represent different aspects of inflammatory diseases: macrophage activation (ferritin); endothelialopathy (D-dimer); interleukin-1/interleukin-6/tumour necrosis factor elevation (CRP) and T cell activation (sCD25). METHODS: This was a single-center retrospective study. Patients diagnosed by the Hematology service at Vancouver General Hospital for AOSD or sHLH from 2009 to 2023 were included. RESULTS: There were 16 AOSD and 44 sHLH patients identified. Ferritin was lower in AOSD than HLH (median 11 360 µg/L vs. 29 020 µg/L, p = .01) while D-dimer was not significantly different (median 5310 mg/L FEU vs. 7000 mg/L FEU, p = .3). CRP was higher (median 168 mg/L vs. 71 mg/L, p <.01) and sCD25 was lower (median 2220 vs. 7280 U/mL, p = .004) in AOSD compared to HLH. The combined ROC curve using CRP >130 mg/L and sCD25< 3900 U/mL to distinguish AOSD from HLH had an area under the curve (AUC) of 0.94 (95% confidence interval 0.93-0.97) with sensitivity 91% and specificity 93%. CONCLUSIONS: These findings suggest that simple, widely available laboratory tests such as CRP and sCD25 can help clinicians distinguish AOSD from HLH in acutely ill adults with extreme hyperferritinemia. Larger studies examining a wider range of clinically available inflammatory biomarkers in a more diverse set of cytokine storm syndromes are warranted.

The Canadian Dermatology Association's Top Five Choosing Wisely Canada Recommendations.

INTRODUCTION: In this article, we present the Canadian Dermatology Association's (CDA) Choosing Wisely Canada (CWC) list of top "Five Things Physicians and Patients Should Question in Dermatology" and the evidence in support of each recommendation. METHODS: Using a nominal technique, the CDA Working Group and Task Force generated an initial list based on literature review and expert consultation. After several rounds of list refinement via a modified Delphi process, a final list of recommendations was generated. These were approved by the CDA Board of Directors, presented at the CDA 93rd Annual Conference in 2018, and published by CWC in 2019. RESULTS: The top five recommendations are as follows: (1) Don't routinely prescribe antibiotics for bilateral lower leg redness and swelling; (2) Don't routinely prescribe topical combination corticosteroid/antifungal products; (3) Don't routinely use topical antibiotics on a surgical wound; (4) Don't prescribe systemic antifungals without mycological confirmation of dermatophyte infection; and (5) Don't use oral antibiotics for acne vulgaris for more than 3 months without assessing efficacy. DISCUSSION: This list of recommendations aims to encourage both physicians and patients to reevaluate ineffective, yet common, practices in treating dermatologic conditions. These recommendations represent actionable changes in practice, and therefore have considerable potential to enhance value-based care in dermatology. CONCLUSIONS: This list was developed to identify tangible changes in practice within dermatology that may reduce inefficiencies, prevent potential patient harm, and improve care. Future advocacy work may include updates, feedback obtainment, and patient care handouts, to continue to promote value-based healthcare and best practices.

One-Year Review of the SCREEN (Skin Cancer Post-Transplant) Clinic.

The Skin Cancer Post-Transplant (SCREEN) Clinic is a skin-cancer screening clinic that is fully integrated into the renal transplantation clinic at St Paul's Hospital in Vancouver, British Columbia. The purpose of this review was to determine characteristics of patients most at risk for skin cancer, to specify types and locations of skin cancers diagnosed, and to identify areas for patient and physician education. Transplant patients (91% renal; 5% heart) screened by a dermatologist during a 12-month period were stratified into low-, medium-, and high-risk groups based on detailed history and skin examination. In total, 118 skin cancers were diagnosed. White males were found to be most at risk. Squamous cell carcinoma accounted for the majority of tumours, over 25% of which demonstrated invasion. Forty-two percent of patients used only 1 to 2 bottles of sunscreen per year, and sun-protective practices were limited. With this information, we have identified potential target areas for patient and physician education.

Experiences From a Combined Dermatology and Rheumatology Clinic: A Retrospective Review.

BACKGROUND: The Dermatology and Rheumatology Treatment Clinic is a novel multidisciplinary clinic where patients are concomitantly assessed by a rheumatologist and dermatologist. OBJECTIVES: To determine the number of patients seen in clinic, patient demographics, and most common diagnoses. METHOD: A retrospective review was performed over a 2-year period. Data collected included patient age, sex, dermatologic diagnosis, rheumatologic diagnosis, biopsies performed, and number of follow-up visits. RESULTS: A total of 320 patients were seen (78% female, 22% male). The most common rheumatologic diagnoses were systemic lupus erythematosus (18%), rheumatoid arthritis (15%), psoriatic arthritis (13%), and undifferentiated connective tissue disease (8%). The most common dermatologic diagnoses were dermatitis (17%), psoriasis (11%), cutaneous lupus (7%), various types of alopecia (6%), and infections (5%). CONCLUSIONS: Skin diagnoses were often unrelated to the underlying rheumatologic diagnosis. Rheumatologists and dermatologists can both benefit from being aware of the dermatologic conditions that rheumatologic patients are experiencing.

Cytomegalovirus Scrotal Ulcer in a Renal Transplant Patient.

BACKGROUND: Cytomegalovirus (CMV) is a highly prevalent herpesvirus that can present with cutaneous disease in immunocompromised individuals. This may reflect systemic involvement, which is associated with significant morbidity and mortality. OBJECTIVE: To report a case of cutaneous CMV in an immunocompromised patient and to discuss the differential diagnosis of genital ulcers. METHODS: A medical chart review was conducted on a patient who presented with a scrotal ulcer after renal transplantation. A review of the literature on cutaneous CMV disease was also completed. RESULTS: Biopsy of the scrotal ulcer revealed classic findings of CMV disease. The patient also developed CMV viremia. Treatment with valganciclovir resolved his scrotal ulcer and viremia. CONCLUSION: The differential diagnosis for genital ulcers is broad, especially in the immunocompromised patient. Cutaneous CMV disease should be ruled out with biopsy and immunohistochemical examination in immunocompromised patients, as it may reflect systemic involvement and significantly affect patient care.

Practical Strategies to Improve the Clinical Utility of the Dermatopathology Report.

CONTEXT: -Dermatologists and subspecialty dermatopathologists, working together over many years, develop a common understanding of clinical information provided on the requisition and of terminology used in the pathology report. Challenges arise for pathologists without additional subspecialty training in dermatology/dermatopathology, and for any pathologist reporting skin biopsies for nondermatologists such as general practitioners or surgeons. OBJECTIVE: -To provide practical strategies to improve efficiency of dermatopathology sign-out, at the same time providing the clinician with clear diagnostic and prognostic information to guide patient management. DATA SOURCES: -The information outlined in this review is based on our own experiences with routine dermatopathology and dermatology practice, and review of English-language articles related to the selected topics discussed. CONCLUSIONS: -Using generic diagnoses for some benign lesions, listing pertinent negatives in the pathology report, and using logical risk management strategies when reporting on basal cell carcinoma, partial biopsies, or specimens with incomplete clinical information allow the pathologist to convey relevant and useful diagnostic information to the treating clinician.

Multiple Cutaneous Creamy Papules and Nodules: A Case of Miliarial Gout.

BACKGROUND: Tophaceous gout is the nonarticular deposition of monosodium urate resulting from a disorder in purine metabolism that causes an elevation of serum uric acid. Cutaneous variants of tophaceous gout include papular, nodular, ulcerative, and pustular forms. OBJECTIVE: We present a case of a 67-year-old man who presented with multiple cutaneous creamy white papules and nodules. A biopsy was taken, and a diagnosis of cutaneous tophaceous gout was made. The treatment and pathophysiology are discussed. CONCLUSION: Miliarial gout is a rare form of cutaneous tophaceous gout that is treated using xanthine oxidase inhibitors such as allopurinol and febuxostat or uricosurics such as probenecid.

Bullous pemphigoid associated with acquired hemophilia a: a rare association of autoimmune disease.

BACKGROUND: Acquired hemophilia (AH) is a rare autoimmune disease with an annual incidence of one per million and has a mortality rate of up to 22%. It is caused by the development of autoantibodies against factor VIII. Approximately half of the reported cases are associated with autoimmune disorders, pregnancy, malignancies, and adverse drug reactions. Autoimmune diseases are the most frequently associated disorders and include rheumatoid arthritis, systemic lupus erythematosus, cryoglobulinemia, pemphigus vulgaris, and bullous pemphigoid. There are a few reports of acquired hemophilia and bullous pemphigoid in the literature. METHOD: We report a 73-year-old male who presented with cutaneous blistering, upper gastrointestinal bleeding, and hemoptysis. He later developed right flank pain secondary to a retroperitoneal hematoma. He had a prolonged partial thromboplastin time, a low factor VIII level, and a high factor VIII inhibitor level, all consistent with acquired hemophilia. Skin biopsies were diagnostic for bullous pemphigoid. RESULTS: He was treated successfully with prednisone, cyclophosphamide, rituximab, and intravenous immunoglobulin.

Trichodysplasia spinulosa: rare presentation of polyomavirus infection in immunocompromised patients.

BACKGROUND: Trichodysplasia spinulosa (TS) is a rare, striking, folliculocentric papular eruption seen exclusively in immunosuppressed patients. The eruption can be disfiguring, associated with leonine faces and alopecia. TS is caused by a polyomavirus, identified as trichodysplasia spinulosa polyomavirus (TSPyV). Few reports exist in the literature, and support for treatment options is sparse. METHOD AND RESULTS: We report a patient with TS with underlying lupus nephropathy and renal transplant-associated immunosuppression. Diagnosis was confirmed by biopsy and pathognomonic histologic findings in the context of her extensive, spiculated monomorphous papules. With a biopsy-confirmed diagnosis, oral valganciclovir was prescribed, and the patient showed marked skin texture improvement and hair regrowth. CONCLUSION: The continued reporting of cases of TS will improve clinical identification of this condition and provide better information regarding treatment and long-term consequences.

Sarcoidosis can present with necrotizing granulomas histologically: two cases of ulcerated sarcoidosis and review of the literature.

BACKGROUND: Sarcoidosis is a systemic inflammatory disorder with cutaneous involvement present in 25% of cases. The presence of naked granulomas histologically is the hallmark of sarcoidosis. The presence of necrotizing granulomas is highly suggestive of other granulomatous conditions and leads the clinician to pursue other diagnoses, such as infectious causes. OBJECTIVES: We describe two cases of sarcoidosis in which necrotizing granulomas were present on biopsy. Both patients had ulcerated cutaneous lesions of sarcoidosis. In one case, the presence of these atypical histologic features led to a delay in diagnosis of almost 10 years. We review the various histopathologic findings associated with cutaneous sarcoidosis and discuss a potential connection between ulcerated sarcoidosis and atypical histologic findings. CONCLUSION: When atypical histopathologic features are present, the differential diagnosis of sarcoidosis should not be excluded.

Three-dimensional analysis of a calciphylaxis plaque: clues to pathogenesis.

BACKGROUND: Calciphylaxis is a rare, life-threatening disorder associated with chronic renal failure, presenting with ulcerating plaques leading to death by sepsis in 60% of patients. Calcification of subcutaneous arterioles, thromboses, and extravascular calcification have been demonstrated in incisional biopsy specimens. However, the sequence of these pathologic events is unknown. OBJECTIVE: We examined a calciphylaxis plaque to document the wave of pathologic change from its center to its periphery. METHODS: A calciphylaxis plaque was excised postmortem from a female patient. It was examined histologically along 12 radii from the center of the lesion to its periphery. RESULTS: Calcification of small subcutaneous vessels was present in all histologically abnormal sections and extended further peripherally than extravascular calcification by up to 3.0 cm and further than subcutaneous thrombosis by up to 1.5 cm. CONCLUSION: Vascular mural calcification is an early and essential process in the development of a calciphylaxis plaque.

Central nervous system involvement in neonatal lupus erythematosus.

Computerized tomography (CT) of the brain was performed in 10 of 11 consecutive infants with neonatal lupus erythematosus (NLE) (five boys and six girls). Ten of the 11 infants had brain neurosonography. Nine of 10 infants had abnormal CT scans. There was diffuse, markedly reduced attenuation of the cerebral white matter in four infants studied in the first week of life, and also in an infant 5 weeks of age. Patchy reduced subcortical white matter attenuation was observed in another 5-week-old infant. Basal ganglia calcifications were present in two infants at 2 months of age, one of whom also had mild ventriculomegaly. A patient with macrocephaly studied at 4 months of age had enlarged ventricles and subarachnoid spaces consistent with benign macrocephaly of infancy. Cerebral ultrasound examination was abnormal in all five infants studied in the first week of life and in one infant at 2 months of age. Findings included subependymal cysts (4), echogenic white matter (3), and echogenic lenticulostriate vessels (3). Apart from one case of macrocephaly, there was no clinical evidence of neurologic disease and the subsequent development of these infants has been normal. Subclinical central nervous system (CNS) disease in NLE is likely to be a transient phenomenon that resolves as maternal antibodies are cleared from the infant's circulation. It is important to be aware of these neuroimaging abnormalities to avoid misdiagnosis of congenital viral infection in a newborn with multisystem NLE. The potential for neurologic sequelae is uncertain.