BACKGROUND: Tuberculous mastitis (TBM), is an uncommon form of extra-pulmonary tuberculosis. Clinical and radiological overlap of tuberculous mastitis with malignancy and other granulomatous conditions, along with its paucibacillary nature, make it a diagnostic challenge. In our study, we aim to assess the radiological response of microbiologically negative granulomatous mastitis cases to anti-tuberculous treatment (ATT) in an endemic country.
METHODS: Eighty-seven cases demonstrating granulomatous lesions on breast biopsy were identified. Of these, 49 patients who were treated with ATT and had at least two serial ultrasound follow-ups were included in our study. Mammogram and ultrasound were used for initial imaging. Subsequently, ultrasound was used for serial follow-up. Mantoux skin test, acid fast staining and histological examination of tissue sample were the other investigations used.
RESULTS: Radiologically, on ultrasound, well-circumscribed hypoechoic masses were noted in 18 patients, followed by ill-defined collections with tubular extensions in 15 cases, abscesses in 8, and a focal heterogeneity in 8 patients. Following ATT, 17 patients showed radiological resolution in 4 weeks, 18 of them at 3 months, and nine of them in 6 months.
CONCLUSIONS: Excellent and prompt radiological response to ATT, indicates the need for a high degree of suspicion for tuberculous mastitis (TBM), in endemic countries, even though microbiological tests may turn out negative.

BACKGROUND: Non-infectious (inflammatory) cutaneous granulomatous disorders include cutaneous sarcoidosis (CS), granuloma annulare (GA), necrobiosis lipoidica (NL), and necrobiotic xanthogranuloma (NXG). These disorders share macrophage predominant inflammation histologically, but the inflammatory architecture and the pattern of extracellular matrix alteration varies. The underlying molecular explanations for these differences remain unclear.
OBJECTIVE: To understand spatial gene expression characteristics in these disorders.
METHODS: We performed spatial transcriptomics in cases of CS, GA, NL, and NXG to compare patterns of immune activation and other molecular features in a spatially resolved fashion.
RESULTS: CS is characterized by a polarized, spatially organized T helper (Th) 1 predominant response with classical macrophage activation. GA is characterized by a mixed, but spatially organized pattern of Th1 and Th2 polarization with both classical and alternative macrophage activation. NL showed concomitant activation of Th1, Th2, and Th17 immunity with a mixed pattern of macrophage activation. Activation of type 1 immunity was shared among, CS, GA, and NL and included upregulation of IL-32. NXG showed upregulation of CXCR4-CXCL12/14 chemokine signaling and exaggerated alternative macrophage polarization. Histologic alteration of extracellular matrix correlated with hypoxia and glycolysis programs and type 2 immune activation.
CONCLUSIONS: Inflammatory cutaneous granulomatous disorders show distinct and spatially organized immune activation that correlate with hallmark histologic changes.

A 35-year-old gentleman came to the ophthalmology outpatient department with complaints of bilateral ocular pain, redness and photophobia since three weeks with similar prior history. The patient was a diagnosed case of systemic sarcoidosis since two years with pulmonary, dermatological and neurological involvement for which he was already on treatment which included oral immunosuppressants, steroids, anticonvulsants and multivitamins. On examination, the best corrected visual acuity was 6/18 in the right eye and 6/12 in the left eye. On slit lamp and fundus examination, the patient showed signs of anterior and posterior uveitis in both eyes, the right eye more than the left eye. Treatment was initiated with topical corticosteroids and beta blockers and the patient improved following medical management.

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BACKGROUND: Paediatric granulomatous uveitis (PGU) is rare. In addition, lack of awareness often leads to delayed diagnosis and poor visual outcome. Identifying the underlying cause and deciding how best to treat each patient is challenging.
OBJECTIVE: To evaluate the demographics, aetiologies, complications, treatments, and visual prognosis of paediatric non-infectious granulomatous uveitis.
METHODS: Retrospective chart review of non-infectious PGU occurring in children before the age of 16 years recruited from the Paediatric Rheumatology Unit, Bicêtre Hospital, France, from 2001 to 2023.
RESULTS: We included 50 patients with 90 affected eyes: 29 with idiopathic uveitis, 15 with sarcoidosis, 5 with juvenile idiopathic arthritis, and one with Vogt-Koyanagi-Harada disease. Median age at diagnosis was 9.8 years (range 7.2-12.5). The sex-ratio M/F was 0.52. The most common features of PGU were: panuveitis (56%), bilateral (84%), and chronic (84%). Sarcoidosis was the most frequent diagnosis after idiopathic disease, particularly in the presence of lymphopenia and hypergammaglobulinemia. Uveomeningitis was present in 12% of cases. Upon diagnosis, ocular complications were present in 68 of 90 eyes (76%) particularly in cases of panuveitis. The most commonly used treatments were systemic corticosteroids (72%) and methotrexate (80%). Twenty-three percent of eyes were in remission at last follow-up, 68% were inactive and 4% remained active. The median duration of follow-up was 5.8 years.
CONCLUSIONS: We report the largest cohort of PGU. PGU were mostly idiopathic and had a high rate of complications. Sarcoid and idiopathic panuveitis are serious illnesses in which disease-modifying therapy should be initiated at diagnosis to improve management.

BACKGROUND: Interstitial mycosis fungoides (IMF) is a rare subtype of mycosis fungoides (MF) characterized by atypical lymphocytes infiltrating the reticular dermis between collagen bundles with limited epidermotropism and variable granulomatous features.
METHODS: Retrospective single institution review of 31 cases of IMF including clinical characteristics, disease course and pathological features.
RESULTS: Our cohort was predominately male (19; 61%, M:F 1.6:1) with a mean age at diagnosis of 43 years (range 11-85), mean signs/symptoms duration of 7 years prior to diagnosis, and 6 years mean follow-up duration. Clinically, patients often exhibited symmetric ill-defined patches/plaques involving intertriginous regions with tan-yellow hyperpigmentation and follicular-based papules, wrinkling, and alopecia. Lymphadenopathy was noted in seven patients. Fifteen (52%) patients were in near or complete clinical remission at the latest follow-up. T-cell receptor gene rearrangement was positive in 23/24 (96%) cases. Histopathologically, atypical cells were small-medium, CD4+ (29; 94%) or rarely CD4+/CD8+ (1; 3%) lymphocytes infiltrating the reticular dermis with thickened collagen bundles (27; 87%), multinucleated giant cells (12; 39%), and often tracing along adnexa with subtle folliculotropism (12/20; 60%).
CONCLUSIONS: Our study demonstrates IMF is an indolent subtype of MF with distinct features, including frequent granulomatous and subtle follicular involvement resulting in alopecia.

OBJECTIVE: To describe the clinical and histopathological features of a sterile granulomatous panuveitis syndrome in 33 dogs that underwent enucleation and ocular histopathology.
METHODS: Retrospective review of the medical records and ocular histopathology reports of 33 cases. Inclusion criteria were enucleation in conjunction with characteristic clinical and histopathological features.
RESULTS: Thirteen breeds were represented (including crossbreeds). Panuveitis was acute and fulminating, and secondary glaucoma was common (n = 27). Interval from initial presentation to enucleation was 99 days (median 33 days, range 5-605 days). The mean age at enucleation was 6.7 years. Ocular signs were initially unilateral (n = 18) or bilateral (n = 15). The disease became bilateral in 18/25 cases that initially underwent unilateral enucleation, resulting in enucleation or euthanasia in 9/18 (mean interval of 168 days). Seven out of 59 eyes had a good outcome following topical anti-inflammatory and systemic immunosuppressive therapy. None of the dogs had travel history nor relevant systemic signs from presentation to follow-up (mean 619 days, range 16-3012 days). Histopathology revealed histiocytic and lymphoplasmacytic panuveitis with pigment dispersion, and no infectious agents were identified on light microscopy.
CONCLUSIONS: To the authors\' knowledge, this is the first report of a sterile granulomatous panuveitis syndrome in dogs in the UK. The clinical signs are severe, with rapid progression, and can result in bilateral enucleation or euthanasia in affected dogs. There does not appear to be an age or breed predisposition, however further research is necessary in this regard. Early and aggressive intervention, with both topical and systemic immunosuppressive therapy, is recommended to reduce the risk of blindness, enucleation, and euthanasia.

The co-existence of granulomatous mastitis and collagenous spherulosis in a breast lump is an uncommon finding. The awareness of cytomorphological features can help corroborate a cytological diagnosis. A palpable breast lump in an elderly female warrants urgent attention and fine needle aspiration is a rapid, reliable method of evaluation. An elderly female with a firm breast lump mimicking malignancy was subjected to fine needle aspiration cytology (FNAC). Smears showed ill-formed granulomas, inflammatory cells and homogeneous hyaline stromal globular elements intermingled with the benign ductal epithelial and myoepithelial cells.

Sarcoidosis is a sterile non-necrotizing granulomatous disease without known causes that can involve multiple organs with a predilection for the lung and thoracic lymph nodes. Worldwide it is estimated to affect 2-160/100,000 people and has a mortality rate over 5 years of approximately 7%. For sarcoidosis patients, the cause of death is due to sarcoid in 60% of the cases, of which up to 80% are from advanced cardiopulmonary failure (pulmonary hypertension and respiratory microbial infections) in all races except in Japan were greater than 70% of the sarcoidosis deaths are due to cardiac sarcoidosis. Scadding stages for pulmonary sarcoidosis associates with clinical outcomes. Stages I and II have radiographic remission in approximately 30%-80% of cases. Stage III only has a 10%-40% chance of resolution, while stage IV has no change of resolution. Up to 40% of pulmonary sarcoidosis patients progress to stage IV disease with lung parenchyma fibroplasia, bronchiectasis with hilar retraction and fibrocystic disease. These patients are at highest risk for the development of precapillary pulmonary hypertension, which may occur in up to 70% of these patients. Sarcoid patients with pre-capillary pulmonary hypertension can respond to targeted pulmonary arterial hypertension medications. Stage IV fibrocytic sarcoidosis with significant pulmonary physiologic impairment, >20% fibrosis on HRCT or pre-capillary pulmonary hypertension have the highest risk of mortality, which can be >40% at 5-years. First line treatment for patients who are symptomatic (cough and dyspnea) with parenchymal infiltrates and abnormal pulmonary function testing (PFT) is oral glucocorticoids, such as prednisone with a typical starting dose of 20-40 mg daily for 2 weeks to 2 months. Prednisone can be tapered over 6-18 months if symptoms, spirometry, PFTs, and radiographs improve. Prolonged prednisone may be required to stabilize disease. Patients requiring prolonged prednisone ≥10 mg/day or those with adverse effects due to glucocorticoids may be prescribed second and third line treatements. Second and third line treatments include immunosuppressive agents (e.g., methotrexate and azathioprine) and anti-tumor necrosis factor (TNF) medication; respectively. Effective treatments for advanced fibrocystic pulmonary disease are being explored. Despite different treatments, relapse rates range from 13% to 75% depending on the stage of sarcoid, number of organs involved, socioeconomic status, and geography. CONCLUSION: The mortality rate for sarcoidosis over a 5 year follow up is approximately 7%. Unfortunately, 10%-40% of patients with sarcoidosis develop progressive pulmonary disease, and >60% of deaths resulting from sarcoidosis are due to advance cardiopulmonary disease. Oral glucocorticoids are the first line treatment, while methotrexate and azathioprine are considered second and anti-TNF agents are third line treatments that are used solely or as glucocorticoid sparing agents for symptomatic extrapulmonary or pulmonary sarcoidosis with infiltrates on chest radiographs and abnormal PFT. Relapse rates have ranged from 13% to 75% depending on the population studied.

Die Necrobiosis lipoidica (NL) ist eine seltene granulomatöse Erkrankung mit scharf begrenzten, teleangiektatischen, braun-roten Plaques mit atroph-gelblichen Zentren, die zu Ulzerationen neigen und hauptsächlich an den Schienbeinen auftreten. Bei Kindern ist NL sehr selten, jedoch sind die Therapieresistenz, das problematische kosmetische Erscheinungsbild, die schmerzhaften Ulzerationen und die mögliche Entwicklung von Plattenepithelkarzinomen besonders herausfordernd für diese Altersgruppe. Unsere Literaturrecherche inkludiert 29 Berichte über NL bei Patienten unter 18 Jahren, die seit 1990 auf PubMed, EMBASE und Medline publiziert wurden. Das mittlere Alter war 14,3 Jahre mit weiblicher Prädominanz von 2 : 1 und hoher Prävalenz von Diabetes mellitus (80%). Aus den Daten geht hervor, dass hochpotente topische Steroide bis zu zweimal täglich Behandlung erster Wahl sind. Therapierefraktäre Fälle können auf Tacrolimus umgestellt werden. Ulzerationen profitieren von phasenadaptierter Wundversorgung und antientzündlichen medizinischen Verbänden wie mit medizinischem Honig. Das Hinzufügen einer hyperbaren Sauerstoffbehandlung zur lokalen oder systemischen Therapie kann bei schwer behandelbaren ulzerierten Läsionen in Betracht gezogen werden. Therapieresistente Fälle können umgestellt werden auf topische Photochemotherapie oder systemische Behandlung mit TNF-alpha-Inhibitoren, systemischen Steroiden (bevorzugt bei Patienten ohne Diabetes), Pentoxifyllin oder Hydroxychloroquin. Necrobiosis lipoidica in der Kindheit ist mit über 40% fehlgeschlagenen Therapieversuchen schwer zu behandeln, weshalb weitere Forschung über Patientenregister empfohlen wird.