Disseminated nodular granulomatous perifolliculitis

Nodular granulomatous perifolliculitis is a well-recognized infection of the dermal and subcutaneous tissue caused by dermatophytes, which normally do not invade beyond the epidermis. We report here one such case that occurred in an immunosuppressed individual. The patient was a 35-year-old farmer who presented with small pruritic eruption that had initially appeared on the lower leg and then had gradually spread to hair-bearing areas of the body, finally producing nodular and pustular inflammatory lesions with exacerbations and remissions. Fungal examination by direct potassium hydroxide mount and culture revealed Trichophyton rubrum. Granulomatous changes were seen on histopathological examination. The patient completely responded to systemic antifungal therapy.     

Idiopathic granulomatous mastitis with erythema nodosum: Is it a variant of clinical presentation indicating treatment resistance? A retrospective cohort study

Idiopathic granulomatous mastitis (IGM) rarely occurs with erythema nodosum (EN) as a systemic finding. However, the impact of their coexistence on disease severity and response to steroids has not been investigated yet. Patients diagnosed with IGM between September 2014 and October 2018 were divided into two groups according to the presence or absence of EN during the first admission retrospectively. The IGM was more severe in patients with EN as it was presented more often as bilateral and diffuse involvement of the breast. Findings of mastitis did not resolve with steroids in 50% of this group. Repetitive excisions and mastectomy with reconstructions were required to control the disease. Coexistence of EN and IGM was found to be related to bilateral and aggressive involvement, which could be associated with insufficient response to steroids. Associated patients should be informed in terms of the aggressive course, and surgery can be highlighted as a first‐line treatment.     

Granulomatous mastitis — a diagnostic dilemma

Granulomatous lobular mastitis is a rare benign breast disease. It is characterized by chronic, non-caseating granulomatous lobulitis. It may be misdiagnosed as a carcinoma of the breast and may lead to mastectomy. Diagnostic criteria include-A) Granulomatous infl ammation with multinucleated giant cells, epithelioid histiocytes. B) It is centered on lobules with minor ductal and periductal infl ammation. C) It nearly always follows the pregnancy. A case of GLM, which was treated with local excision and postoperative steroid therapy is being reported to increase awareness amongst surgeons and pathologist.     

Molecular analysis of X-linked chronic granulomatous disease in five unrelated Korean patients

Chronic granulomatous disease (CGD) is a fatal genetic disorder in which phagocytes fail to produce antimicrobial superoxide because of NADPH oxidase deficiency. Molecular defects in CYBB gene causing X-linked CGD are responsible for about 70% of all cases. This study was done to confirm genetic defects of CYBB gene in five Korean patients who were highly suggestive of having CGD by clinical history. We performed initial screening for five unrelated Korean patients using single strand conformation polymorphism (SSCP) and then selective sequencing for the regions involving the abnormal bands. Activated NBT tests revealed that all patients were X-linked. SSCP analysis for CYBB gene showed abnormal bands in all patients. The molecular defects of five patients were as follows: c.1663insT, c.1111-1G>T, c.39_40insG, c.927delC and c.434T>C mutation. This result will help the families with prenatal diagnosis or genetic counseling.     

Role of interleukin-6 in a glucan-induced model of granulomatous vasculitis

The role of interleukin-6 (IL-6) in granulomatous vasculitis is not well understood. To investigate its involvement in this type of vasculitis a model of glucan-induced pulmonary vasculitis employed interleukin-6 deficient (IL-6-/-) mice. Briefly, IL-6-/- mice and C57B/J6 wild type (IL-6+/+) mice were injected intravenously with a suspension of glucan isolated from the cell wall of bakers yeast which results in a granulomatous vasculitis primarily in the pulmonary vasculature. Histological examination demonstrated no significant difference in the number of infiltrating leukocytes between the IL-6+/+ and IL-6-/- glucan-injured mice. Similar numbers of granulomas were noted in both the IL-6+/+ and IL-6-/- injured animals, while no granulomas were seen in saline injected control mice. Cells recovered from the bronchoalveolar lavage (BAL) fluid were differentially stained and counted. While there was a significant increase in infiltrating leukocytes recovered from the BAL following glucan-induced injury, there was no significant difference between the IL-6+/+ and IL-6-/- mice. In addition, no difference was demonstrated in total protein content in the BAL fluid between IL-6+/+ and IL-6-/- mice. However, myeloperoxidase (MPO) activity in the lungs of the IL-6-/- mice was less than in their IL-6+/+ counterparts suggesting that these animals have a partial defect in their ability to recruit neutrophils in this model. Studies done to look for levels of other cytokines/chemokines in these animals to compensate for the loss of IL-6 revealed that only IL-10 in the sera (p<0.016) and BAL fluid (p<0.05) of IL-6-/- mice was significantly higher then their IL-6+/+-injured counterparts. These studies suggest that IL-6, while possibly involved in early neutrophil accumulation in this model does not appear critical to the development of the TH-2 mediated granulomatous vasculitis.     

肉芽肿性小叶性乳腺炎伴发乳腺导管扩张症的临床病理学观察

目的 分析肉芽肿性小叶性乳腺炎伴发乳腺导管扩张症的临床病理特征.方法 收集2005年8月至2013年5月诊断的此类病例32例,对其临床和组织病理学资料进行回顾性分析.结果 患者年龄26～45岁,2例未生育,其余有生育史,其中14例患侧乳腺未哺乳或哺乳障碍.大体检查乳腺肿块直径3～12 cm,与周围组织界限不清,切面见小脓腔或小囊腔,直径0.1～0.5 cm,腔内有灰白或淡黄色分泌物.镜下观察32例均有肉芽肿性小叶性乳腺炎表现,在此基础上均可见乳腺导管扩张症.11例曾行抗生素治疗无效,12例因切开引流致皮肤窦道形成.术后随访5 ～ 90个月,3例患侧复发,1例对侧发生肉芽肿性小叶性乳腺炎伴发乳腺导管扩张症,2例对侧发生肉芽肿性小叶性乳腺炎,4例失访.结论 肉芽肿性小叶性乳腺炎可与乳腺导管扩张症伴发,准确诊断有助于针对性治疗和减少复发.     

T-cell signature cytokines distinguish pulmonary sarcoidosis from pulmonary tuberculosis

Sarcoidosis is a systemic inflammatory disorder characterized by tissue infiltration due to mononuclear phagocytes and lymphocytes and associated noncaseating granuloma formation. Pulmonary sarcoidosis (PS) shares a number of clinical, radiological, and histopathological characteristics with that of pulmonary tuberculosis (PTB). Due to this, clinicians face issues in differentiating between PS and PTB in a substantial number of cases. There is a lack of any specific biomarker that can diagnose PS distinctively from PTB. We compared T-cell-based signature cytokines in patients with PS and PTB. In this study, we proposed a serum biomarker panel consisting of cytokines from cells: T helper (Th) 1 [interferon-gamma (IFN-γ); tumor necrosis factor-alpha (TNF-α)], Th9 [interleukin (IL)-9], Th17 [IL-17], and T regulatory (Treg) [IL-10; transforming growth factor-beta (TGF-β)]. We performed the principal component analysis that demonstrated that our serum cytokine panel has a significant predictive ability to differentiate PS from PTB. Our results could aid clinicians to improve the diagnostic workflow for patients with PS in TB endemic settings where the diagnosis between PS and PTB is often ambiguous.     

Long-term natural history of Crohn＇s disease

Crohn＇s disease is a chronic inflammatory granulomatous process that usually involves different sites in the intestinal tract. Genetic and environmental factors are thought to play a role in its etiology and pathogenesis. The disorder has a heterogeneous clinical expression and data from tertiary care settings have documented its female predominance, occasional familial nature, and high rate of stricture formation and penetrating disease. It may appear from early childhood to late adulthood, although over 80% are currently diagnosed before age 40 years, usually with terminal ileal and colonic involvement. Several studies have now shown differences in phenotypic clinical expression depending on the initial age at diagnosis, with pediatric-onset disease being more severe and more extensive with more involvement of the upper gastrointestinal tract compared to adult-onset disease. In addition, longterm studies from these tertiary care settings have documented that the disorder may evolve with time into a more complex disease with stricture formation and penetrating disease complications （i.e. fistula and abscess）. Although prolonged remission with no evidence of inflammatory disease may occur, discrete periods of symptomatic and active granulomatous inflammatory disease may re-appear over many decades. Long-term studies on the natural history have also suggested that discrete events （or agents） may precipitate this granulomatous inflammatory process.     

Bacille Calmette-Guérin — associated neonatal hepatitis

We describe a full-term immunocompetent neonate who developed jaundice at 3 weeks of age. Physical examination disclosed no abnormalitics. Laboratory investigations showed direct reacting hyperbilirubinaemia and elevated liver enzymes. Liver biopsy revealed a noncaseating granulomatous hepatitis. The patient made an uneventful recovery within 4 weeks without therapy. Bacille Calmette-Guérin hypersensitivity reaction is suggested as the reason for this neonatal hepatitis.