Emi Ishibazawa

Pyoderma gangrenosum (PG) is a noninfectious neutrophilic dermatosis that can occur either idiopathically or secondary to various systemic conditions. PG is rare in childhood and even rarer in infancy. Due to its rarity, the pathophysiological mechanisms remain unclear. Although systemic glucocorticoids are considered the first‐line therapy, no standardized second‐line or subsequent therapeutic approach has been established, particularly for refractory cases. In this report, we present a case of infantile idiopathic PG showing sustained clinical improvement after the addition of the anti–tumor necrosis factor (TNF)‐alpha antibody infliximab (IFX). In addition, we conducted multiplex cytokine analysis to explore the patient’s inflammatory profile and provide preliminary observations regarding the inflammatory milieu associated with this condition. The patient, a 9‐month‐old infant, developed multiple pustules and ulcerative skin lesions on the face and extremities following Bacillus Calmette–Guérin (BCG) vaccination. Pathogen investigations were all negative, and the diagnosis of PG was made based on clinical findings, supported by histological features and exclusion of other conditions. After initial treatment with glucocorticoids and cyclosporine, remission was achieved; however, repeated relapses occurred. IFX was subsequently initiated, resulting in significant clinical improvement. Serum cytokine profiling at the onset revealed a broad elevation of proinflammatory cytokines, including soluble TNF receptors 1 and 2, with a pattern partially overlapping with that observed in Kawasaki disease. The clinical course and cytokine analysis results suggest that IFX may be a viable treatment option for refractory PG, even in infants.

Digital clubbing is typically linked to pulmonary or cardiovascular diseases. There have been no reported cases of systemic lupus erythematosus (SLE) where the initial complaint was solely foca digital clubbing and a chilblain-like rash on the ngers. A 13-year-old girl experienced digital clubbing for 3 months. Physical examination revealed focal digital clubbing of the thumb, index nger, and middle nger on her right hand, along with slight blotchy erythema assessed as a chilblain-like rash. Laboratory data showed hypocomplementemia and a high anti-double-stranded DNA antibody titer. She was also positive for antiphospholipid antibodies, had prolonged prothrombin time and activated partial thromboplastin time, and elevated anti-prothrombin antibody levels. She was diagnosed with SLE complicated by hypoprothrombinemia associated with lupus anticoagulant. Prednisolone treat ment resolved the digital clubbing and laboratory abnormalities. Digital clubbing may be an initial symptom of SLE. Screening for autoimmune diseases should be considered when a patient presents with digital clubbing not associated with pulmonary or cardiovascular diseases or malignant tumors. Cite this article as: Sato M, Nagamori T, Kurisawa MJ, Tsuda J, Ishibazawa E, Takahashi S. Focal digital clubbing as an initial symptom of adolescent-onset systemic lupus erythematosus: a case report. Eur J Rheumatol. 2025, 12(4), 0075, doi:10.5152/eurjrheum.2025.24075.

Background: Shiga toxin‐producing Escherichia coli ‐associated hemolytic uremic syndrome (STEC‐HUS) is a life‐threatening condition complicated by acute kidney injury, acute respiratory distress syndrome, and central nervous system disorders. The early identification of high‐risk patients is required to facilitate timely and appropriate treatment. Methods: The medical records of patients with STEC‐HUS treated at 11 hospitals in Hokkaido, Japan, were reviewed retrospectively. A multi‐institutional retrospective analysis was performed in which patients were divided into two groups according to the presence or absence of severe complications requiring blood purification therapy or encephalopathy. We compared the laboratory values at diagnosis between the severe and mild groups. To identify patients at high risk of developing severe complications, a scoring system, referred to as the “STEC‐HUS severity (STEC‐HUSS) score,” was constructed based on the parameters showing significant differences. Results: Of the 41 patients with STEC‐HUS, 11 were classified into the severe group and 30 into the mild group. Significant differences were observed between the groups in terms of white blood cell count, activated partial thromboplastin time, fibrinogen, D ‐dimer, total protein, aspartate transaminase, alanine transaminase, lactate dehydrogenase, creatinine, and C‐reactive protein levels. The STEC‐HUSS score was calculated on a scale of 0–10 by summing the number of test items that demonstrated abnormal values. The STEC‐HUSS score, when the cut‐off value was 4, showed a sensitivity of 100% and a specificity of 91% in the severe group. Conclusion: We developed a novel scoring system to identify patients at high risk of severe STEC‐HUS.