Crohn's Disease Presenting as Metabolic Myopathy: A Case Report.

Myopathies associated with systemic diseases results from several different disease processes. Myopathy as the initial presenting symptom in Crohn's disease is a rare presentation. We report a 20-yearr-old lady who presented with a painful proximal myopathy. On examination, she was malnourished with pallor, angular cheilitis, Bitots spots, and bilateral pitting pedal edema. Laboratory evaluation showed iron deficiency anemia, hypoalbuminemia, and very low vitamin D levels with elevated creatine phosphokinase levels. A possibility of osteomalacic metabolic myopathy due to vitamin D deficiency was considered. The malabsorption workup was negative. A colonoscopic biopsy showed noncaseating granulomatous inflammation suggestive of Crohn's disease. With supplementary therapy and specific treatment, she was asymptomatic at 6-months follow-up with no residual neurological deficits. A detailed history and an algorithmic approach will be very useful in making the differential diagnosis in any patient presenting with muscle weakness in myopathy associated with systemic illness.

Majocchi's granuloma on the forearm caused by Trichophyton tonsurans in an immunocompetent patient.

Majocchi's granuloma is an uncommon fungal infection of the dermis and subcutaneous tissue. The most frequently identified cause of Majocchi's granuloma is anthropophilic Trichophyton rubrum, and it is most commonly located on the anterior aspect of the lower limbs in women. Here, we report a case of Majocchi's granuloma on the forearm, a site that is rarely involved, in a 62-year-old woman who had been bitten by a dog. Histological examination revealed a dense dermal infiltrate composed of lymphoplasmacytic cells and neutrophils, with hyphae in the dermis. The presence of the fungus, Trichophyton tonsurans, was confirmed by mycological examination and molecular methods. Therefore, histological and mycological examination confirmed the diagnosis of Majocchi's granuloma. The patient was treated with local moxibustion and itraconazole, 200 mg/day, for 60 days, which facilitated a complete resolution of the lesions.

Wound healing of metastatic perineal Crohn's disease using hyperbaric oxygen therapy: A case series.

BACKGROUND: Metastatic Crohn's disease (CD) is a rare manifestation of CD. It involves inflammatory skin lesions with histopathological findings (granulomas) similar to CD, without connection to the gastrointestinal tract. Hyperbaric oxygen therapy (HBO) has been suggested as a possible treatment option. OBJECTIVE: This study aimed to identify and treat a consecutive series of patients with biopsy-proven metastatic CD and monitor wound healing using prospectively acquired outcomes. METHODS: Pathology results of all patients with ongoing perineal wound-healing problems after proctectomy between 2005 and 2018 at the Amsterdam University Medical Centre were assessed for metastatic CD. Patients with a biopsy-proven diagnosis of perineal metastatic CD were offered HBO (40 daily sessions of 100% oxygen at 2.4 atmosphere absolute). Wound healing was monitored using photographs and standardised questionnaires (the Inflammatory Bowel Disease Questionnaire, EuroQol Visual Analogue Scale and the Female Sexual Function Index) at baseline and 1 and 3 months after HBO. RESULTS: Out of 13 patients in the cohort with persisting perineal wounds after proctectomy, six (46%) had biopsy results consistent with metastatic CD. Of these, three accepted treatment with HBO. All three patients were female. One patient had complete healing of her perineal wound; another patient showed initial improvement but had a flare of luminal and perineal disease at the 3-month follow-up. The third patient showed improvement solely in the questionnaires, with higher scores on all three questionnaires. CONCLUSION: A high rate of metastatic CD was found in patients with ongoing wound-healing problems after proctectomy, implying that the disease might not be as rare in these selected patients as previously thought. HBO might be beneficial in the treatment of metastatic CD.

Necrotizing Granulomatous Dermatitis and Panniculitis Masquerading as T Cell Lymphoma.

A 51-year-old white woman with a past medical history significant for steroid-dependent ulcerative colitis, rheumatoid arthritis, and diabetes mellitus presented to the hospital with fever and painful, erythematous subcutaneous nodules on the medical aspects of both thighs. Histopathologic examination showed features suggestive of an abscess, but her condition failed to improve with intravenous broad-spectrum antibiotics. Molecular studies detected T cell receptor-ß gene rearrangements. The lesions later exhibited signs of necrosis, requiring multiple debridements as well as therapy with hyperbaric oxygen. She was later referred to the MD Anderson Cancer Center for evaluation for possible subcutaneous panniculitis-like T cell lymphoma.

Linfadenitis por Mycobacterium bovis, una entidad a tener en cuenta / Mycobacterium bovis lymphadenitis, an entity to consider

Las medidas de control del ganado y la pasteurización de los productos lácteos han hecho que la enfermedad tuberculosa por Mycobacterium bovis sea infrecuente en nuestro medio. El diagnóstico de esta entidad requiere un alto índice de sospecha, fundado en una historia clínica detallada y pruebas complementarias oportunas. La presentación más frecuente es extrapulmonar, principalmente como linfadenitis, por lo que ante una adenopatía de mala evolución se recomienda remitir al paciente para estudio y valorar la realización de punción aspiración con aguja fina para examen citológico y cultivo. Presentamos un caso de linfadenitis por M. bovis en una niña de siete años que respondió adecuadamente al tratamiento médico (AU9

Cattle control measures and milk products pasteurization have made Mycobacterium bovis disease uncommon in our environment. The diagnosis of this disease requires a high index of suspicion based on a detailed medical history and appropriate laboratory tests. Extrapulmonary cases are the main presentation, especially lymphadenitis, for this reason, is recommended to refer to study all the adenopathies with poor evolution, for evaluating de need of a fine-needle aspiration for citologý examination and culture. We report the case of M. bovis lymphadenitis in a 7-year-old girl with adequate response to medical treatment (AU)

In situ photoimmunotherapy for cutaneous granuloma caused by itraconazole-resistant Candida guilliermondii.

Cutaneous granulomas caused by Candida guilliermondii are difficult to cure. In situ photoimmunotherapy (ISPI) is a novel method composed of local photothermal therapy and immunoadjuvant. In this study, ISPI was used the first time clinically for cutaneous granuloma caused by itraconazole-resistant C.guilliermondii. A 10-week cycle of ISPI was composed of (1) 5% imiquimod applied topically every other day and (2) irradiation of lesions with an 808-nm diode laser at Days 14, 28, 42, and 56. Here we report our first case. A patient was treated with ISPI for four cycles. After the treatment, the lesions were eliminated without recurrence during a 12-month follow-up. Our results demonstrate that ISPI can be used as an effective treatment modality for cutaneous fungal granuloma.

Two cases of sporotrichosis of the right upper extremity in right-handed patients with diabetes mellitus / Dos casos de esporotricosis de la extremidad superior derecha en pacientes diestros con diabetes mellitus

Background. Sporothrix species have proved to show high degrees of endemicity. Sporothrix globosa is the only pathogenic Sporothrix species that has till date been reported from China, where it is endemic in the northeastern provinces. Aims. We report two cases of lymphocutaneous sporotrichosis with diabetes mellitus as underlying disease in patients from the non-endemic area of China. Methods. A 59-year-old farmer and a 60-year-old gardener were admitted in February and June 2014, respectively. Both patients were right-handed men and presented with progressive plaques and nodules, which they had for several years, involving the right upper extremity. Skin biopsy from the granuloma was taken and cultured on Sabouraud medium, and molecular identification based on the calmodulin region was performed. Antifungal susceptibility testing was also performed with the microdilution method. Results. Biopsy of the lesions showed the presence of infectious granuloma. The fungal cultures were identified as Sporothrix globosa by conventional methods, and confirmed by molecular identification. A subsequent course of oral antifungal therapy with low dosage of itraconazole was well tolerated and resolved the infection. Conclusions. Identification of fungal species and antifungal susceptibility testing are mandatory for epidemiological and therapeutic reasons. Early diagnosis of sporotrichosis is essential to prevent those sequelae when the disease progresses and provides highly effective methods for treating this emerging disease. Avoiding the exposure to plant material potentially contaminated with fungal spores should be recommended, especially in immunocompromised patients (AU)

Antecedentes. Las especies de Sporothrix han demostrado un alto nivel de endemicidad. Sporothrix globosa es la única especie patógena descrita hasta la fecha en China, donde es endémica en las provincias del nordeste. Objetivos. Se describen dos casos de esporotricosis linfocutánea con diabetes mellitus como enfermedad de base, en pacientes procedentes de un área no endémica de China. Métodos. Un campesino de 59 años y un jardinero de 60 años de edad fueron atendidos en febrero y junio de 2014, respectivamente. Ambos eran varones, diestros y se presentaron con placas y nódulos de varios años de evolución, que afectaban a la zona superior del brazo derecho. Se tomaron biopsias de los granulomas de la piel, que fueron cultivados en medio de Sabouraud, y se realizó una identificación molecular basada en la región de la calmodulina. Se evaluó la sensibilidad a los antifúngicos mediante el método de microdilución. Resultados. La biopsia de las lesiones mostró la presencia de un granuloma infeccioso. Los hongos aislados en los cultivos fueron identificados como Sporothrix globosa por métodos convencionales, y confirmados mediante identificación molecular. La subsecuente administración de terapia antifúngica oral con bajas dosis de itraconazol fue bien tolerada y resolvió la infección. Conclusiones. La identificación de las especies fúngicas y el análisis de la sensibilidad a los antifúngicos son necesarios por razones epidemiológicas y terapéuticas. El diagnóstico temprano de la esporotricosis es esencial para prevenir las secuelas que genera el progreso de la enfermedad y para ofrecer métodos altamente efectivos para el tratamiento de esta enfermedad emergente. Debe recomendarse evitar la exposición a material vegetal potencialmente contaminado con esporas fúngicas, especialmente en pacientes inmunocomprometidos (AU)

Scarring of lupus miliaris disseminatus faciei: treatment with a combination of trichloroacetic acid and carbon dioxide laser.

We present a case of a 35-year-old man having a 12-month history of multiple reddish-brown papules on the chin, forehead, cheeks, and eyelids. Histopathologic findings revealed epithelioid cell granulomas with central necrosis consistent with a diagnosis of lupus miliaris disseminatus faciei. After 9 months of combined treatment with ethambutol, rifampin, and pyrazinamide, most lesions gradually resolved but remained as severe disfiguring scars. After 10 sessions of treatments with 100% trichloroacetic acid and CO2 laser, the lupus miliaris disseminatus faciei scars have been much improved and the patient has never experienced a recurrence of disease during subsequent years of follow-up.

Successful simple limbal epithelial transplantation (SLET) in lime injury-induced limbal stem cell deficiency with ocular surface granuloma.

A 9-year-old boy presented with unilateral, total limbal stem cell deficiency (LSCD) complicated by the presence of a large fibro-vascular ocular surface mass lesion secondary to accidental lime injury. The pathological tissue covering the cornea was excised and simple limbal epithelial transplantation (SLET) was performed using autologous limbal tissue from the fellow eye. Histopathology of the excised ocular surface tissue revealed exuberant granulation tissue interspersed with retained calcium particles. At 6 weeks postoperatively, a focal recurrence of LSCD with symblepharon and forniceal shortening was noted superiorly. This was successfully managed by performing conjunctival autografting along with supplemental SLET. The unaided vision had improved from light perception at presentation to 20/40 at 6 months postoperatively. The fornices were deep and the corneal surface was avascular, epithelised and stable. This case demonstrates the efficacy of SLET in a child with severe ocular burns, highlighting the role of supplementary procedures customised to treat focal recurrences of LSCD.

Cytological specimens obtained by endobronchial ultrasound-guided transbronchial needle aspiration: sample handling and role of rapid on-site evaluation.

Recently developed, endobronchial ultrasound-guided transbronchial needle aspiration (EBUS-TBNA) is a minimally invasive modality for mediastinal lymph node staging in lung cancer patients as well as for the diagnosis of mediastinal and hilar lymphadenopathy. It has been shown in systematic reviews and meta-analysis that a high diagnostic yield can be achieved with EBUS-TBNA for staging lung cancer. Though still not a standard of practice, this novel technology has attracted physicians and surgeons as an alternative modality to surgical biopsy for the assessment of the mediastinum. Standard cytology, thin layer preparations in liquid medium or cell blocks of cells obtained by EBUS-TBNA can be applicable not only for pathological diagnosis but also for further investigations such as immunohistochemistry and fluorescence in situ hybridization. In addition, samples obtained by EBUS-TBNA can also be used for molecular analysis. The key to a successful EBUS-TBNA is to understand the anatomy of the mediastinum as well as the basic steps of the procedure. Moreover, handling of the sample obtained by EBUS-TBNA is crucial for diagnosis since no amount of skill or interest of the interpreter can make up for a badly prepared sample. The goals of rapid on-site evaluation during EBUS-TBNA include determination of whether sampling of the target has been achieved and more importantly triage of samples to secondary investigations. This manuscript explains the detailed techniques of EBUS-TBNA to master this innovative procedure.

An in vivo canine study to assess granulomatous responses in the MedStream Programmable Infusion System (TM) and the SynchroMed II Infusion System®.

OBJECTIVE: A rare, but consistently reported complication of intrathecal (IT) delivery of opioids via programmable implantable infusion pumps is aseptic granuloma formation around the tip of the IT catheter. In the current study, the incidence and severity of IT granuloma formation was assessed for the MedStream Programmable Infusion and SynchroMed II Infusion pumps when delivering saline, baclofen, or morphine at varying concentrations and daily doses. DESIGN: Randomized study with six groups: 1) MedStream with 0.9% saline; 2) SynchroMed II with 0.9% saline; 3) MedStream with morphine sulfate; 4) SynchroMed II with morphine sulfate; 5) MedStream with baclofen; or 6) SynchroMed II with baclofen. The groups receiving morphine received either low or high concentrations (6 or 12.5 mg/mL) delivered in rates resulting in low to high (1.25-12 mg/day) daily doses. Animals receiving baclofen began at low dose/low concentration and underwent stepwise increases in concentration and dose to a maximum of 2 mg/mL/day. Animals receiving saline received a constant flow rate through either a silicone or a polyurethane IT catheter. SETTING: The study was set as in vivo laboratory experiment. SUBJECTS: 52 (21-32 kg) canines. INTERVENTIONS: Delivering IT saline, baclofen, or morphine through SynchroMed II and MedStream Programmable Infusion pumps. OUTCOME MEASURES: The incidence and severity of IT catheter tip granuloma formation was assessed with paraffin-embedded spinal cord sections by a pathologist without knowledge of treatment and compared between drug type, drug concentration, daily dose, and pump type. RESULTS: Granuloma formation occurred only in animals receiving morphine, with zero incidence in baclofen or saline infusion, regardless of catheter type. Granuloma incidence increased with increasing morphine concentration or daily dose (one [12.5%] low dose/low concentration, 3 [37.5%] low dose/high concentrations, 4 [50%] high dose/low concentration, and 7 [100%] high dose/high concentration). A granulomatous response to morphine was observed in 10 (63%) animals with SynchroMed II pumps and 5 (33%) animals with MedStream pumps, P = 0.156. CONCLUSIONS: In this in vivo canine model of IT drug delivery, catheter tip granuloma formation was associated with higher concentrations and daily doses of morphine infusion while none were seen with baclofen or saline, and was not associated with catheter type. For both pumps, granulomas were only produced in the presence of morphine infusion. These results suggest that the MedStream Programmable Infusion System has a granuloma safety profile at least equivalent to that of the SynchroMed II pump.

[Intracerebral xanthogranuloma with specific MRI features: a case report].

We report a case of a 47-year-old male who had a left frontal lobe mass with specific magnetic resonance imaging (MRI). Four years previous to his admission to our hospital, he developed acute myelocytic leukemia and he was implanted with peripheral blood stem cell transplantation. One month before his admission, he was unable to walk due to right leg motor weakness. MRI revealed a round mass lesion at the left frontal lobe. Initial diagnosis was a brain abscess, so he was administered some antibacterial drug. However, his neurological symptoms kept getting worse with increase of mass size on MRI. T1-weighted MR images showed the mass as a low intensity lesion with incomplete ring-like Gd-enhancement. T2-weighted images showed the mass as a high intensity lesion with many linear shadows inside the mass. Diffusion weighted images showed the mass as a low intensity lesion. MR spectroscopy revealed an elevation of choline and lactate peak. Differential diagnosis was glioma or demyelinating disease. At the operation, the mass was light yellow and seen to be rich with vessels. The pathological diagnosis was xanthogranuloma in the brain. Postoperative course was uneventful. The frontal residual mass lesion decreased in size and Gd-enhancement.

Granuloma faciale with disseminated extra facial lesions.

Granuloma faciale (GF) is a rare cutaneous disorder categorized as a localized form of small vessel vasculitis. Clinically, it manifests as single or multiple, well-demarcated, red-brown plaques, papules and nodules, nearly always confined to the face. Herein, we report a 39-year-old man with multiple red-brown, infiltrated plaques on his face and extrafacial lesions on the back, shoulders, and both arms. Skin biopsy revealed typical histopathological findings of GF. The patient failed to respond to pulsed dye laser, but intralesional triamcinolone combined with cryotherapy led to an acceptable response.

Granuloma annulare.

We present a case of a 77-year-old, diabetic male with a 20-year history of a migratory erythematous, asymptomatic, generalized, nonscaly, and nonitchy rash that started over the dorsum of his left hand. On examination, there were multiple annular erythematous plaques, distributed symmetrically and diffusely over his torso and arms, with central clearing and no scales. A punch biopsy of the skin helped us to arrive at the diagnosis of a generalized granuloma annulare (GA). GA is a benign, self-limiting skin condition of unknown etiology that is often asymptomatic. The cause of this condition is unknown, but it has been associated with diabetes mellitus, infections such as HIV, and malignancies such as lymphoma. These lesions typically start as a ring of flesh-colored papules that slowly progress with central clearing. Lack of symptoms, scaling, or associated vesicles helps to differentiate GA from other skin conditions such as tinea corporis, pityriasis rosea, psoriasis, or erythema annulare centrifugum. Treatment is often not needed as the majority of these lesions are self-resolving within 2 years. Treatment may be pursued for cosmetic reasons. Available options include high-dose steroid creams, PUVA, cryotherapy, or drugs such as niacinamide, infliximab, Dapsone, and topical calcineurin inhibitors.

Non-sarcoidal, non-tuberculoid granuloma in common variable immunodeficiency.

Sarcoidal (non-caseating) or tuberculoid granulomas are cutaneous manifestations of common variable immunodeficiency (CVID). In this case report, we describe a patient with CVID but with non-sarcoidal, non-tuberculoid granuloma. The 29-year-old Egyptian male patient presented with a vitiliginous patch on the chin of 1 year duration and multiple recurrent warts on the hands and feet of 8 years duration. He is a known case of CVID with chronic diarrhea, recurrent otitis media, pneumonia, purulent conjunctivitis, septic arthritis, hepato-splenomegaly, and generalized lymphadenopathy. In addition, he had evidence of multiple non-tender subcutaneous nodules predominantly juxta-articular and recurrent rheumatoid-like arthritis. The skin overlying the nodules was either normal or slightly erythematous. Laboratory findings revealed markedly reduced serum immunoglobulins (IgG 3.4, n = 7.2-16.9 g/l; IgA 0.1, n = 0.69-3.82 g/l and IgM 0.1, n = 0.63-2.77 g/l) and deficient T cell function. Histopathologic examination of a skin nodule showed well demarcated areas of fibrinoid degeneration of collagen that stain homogeneously and are surrounded by histiocytes in a palisading arrangement, suggestive of granuloma annulare. No microorganisms could be detected. Serology for rheumatoid factor and HIV infection has been persistently negative. Although most infections, including common warts responded well to intravenous immunoglobulin replacement therapy (12 g/i.v., every 2 weeks) and oral broad spectrum antibiotic therapy, the subcutaneous nodules persisted. The vitiliginous patch responded favorably and disappeared within 24 local PUVA sessions. Since skin nodules are asymptomatic, no further treatment was given.

Mycobacterium intermedium granulomatous dermatitis from hot tub exposure.

Nontuberculous mycobacteria, which are widespread in the environment, frequently cause opportunistic infections in immunocompromised patients. We report the first case of a patient with chronic granulomatous dermatitis caused by a rarely described organism, Mycobacterium intermedium. The infection was associated with exposure in a home hot tub.