Eosinophilic granuloma of the sternum in a child treated with closed biopsy

Langerhans cell histiocytosis is a rare neoplastic proliferative disorder of the Langerhans cells. The clinical course is variable, ranging from a low symptomatic single bone lesion to fatal multiple organ involvement. Rarely, the sternum can be the first and single location of the disease. We report on a 12‐year‐old boy who presented with an aggressive lytic lesion of the proximal sternum associated with local pain and afternoon fever. Histopathological analysis of the closed biopsy specimen indicated eosinophilic granuloma of bone/Langerhans cell histiocytosis. Soon after the biopsy procedure the pain and fever subsided. Computed tomography at 2 months showed healing of the lytic lesion. The patient received no other type of treatment. At 2 year follow up he was symptom and disease free.     

An update on pityrosporum folliculitis in Singapore from a single tertiary care dermatological centre

INTRODUCTIONPityrosporum folliculitis (PF) is a common skin condition that can be easily misdiagnosed, especially by non-dermatologists. While the initial diagnosis is often made clinically, skin microscopy may help to confirm the same. However, there is scant literature regarding the clinical epidemiology of PF. To our knowledge, in Singapore, only one prior epidemiological study was performed in 1987. Through the present study, we aimed to provide an update regarding the epidemiology, diagnosis and treatment of patients with PF in Singapore.METHODSWe performed a retrospective review of patients with clinical presentations compatible with PF who presented to the dermatology clinic at the National University Hospital, Singapore, between 1 January 2011 and 31 December 2015. The medical records of patients identified as having clinical presentations that resembled PF were reviewed via written and electronic databases. Information regarding the demographics and clinical presentation of the patients was collected.RESULTSOf the 375 patients identified, 214 (57.1%) were confirmed as having PF based on Gram-stain microscopy. Most (35.0%) of these 214 patients were aged between 21 and 30 years, with a male-to-female ratio of 3:1. The lesions predominantly occurred on the trunk and the back. The majority of patients presented with symptoms that lasted more than one month. 128 (59.8%) patients received oral antifungal treatment, whereas 82 (38.3%) patients were treated with topical antifungal treatment alone.CONCLUSIONA typical Singapore patient with PF is a young man aged 21–30 years, with erythematous follicular papules or pustules over the trunk and the back.     

Reliability and Validity Study of Turkish Version of Pediatric Eosinophilic Esophagitis Symptom Scores® (Tr-PEESS v2.0) Led to Development of a New Pediatric Eosinophilic Esophagitis Scale: GaziESAS

BackgroundThere has been no valid and reliable Turkish scale that measures symptoms in children with eosinophilic esophagitis (EoE). The aim of the study is to test the validity and reliability of the Turkish version of Pediatric Eosinophilic Esophagitis Symptom Scores® (Tr-PEESS v2.0).Methods Relevant forms of Tr-PEESS v2.0 were applied to 2-18 years old children with EoE and to their parents. KINDL QoL patient and parent questionnaires and the GaziESAS scale developed in this study were used to test the convergent validity of Tr-PEESS v2.0. Discriminant validity was evaluated among 3 EoE treatment groups: under treatment, off treatment due to remission, and uncompliant with treatment. Reliability was evaluated by internal consistency, test–retest reliability, and item analysis.Results Fifty-two children/teens (mean age 130.2 ± 60.3 months) and 84 parents were interviewed twice one week apart. The mean duration of EoE was 47.2 ± 35.6 months. Tr-PEESS v2.0 reports correlated with GaziESAS (range 0.361-0.855) and KINDL QoL questionnaires (range −0.316 to 0.413). Parent report of Tr-PEESS v2.0 discriminated children uncompliant with treatment from the ones off treatment and undertreatment. Cronbach’s α values and intraclass correlation coefficients (ICC) values of Tr-PEESS v2.0 ranged from 0.614-0. 895 and 0.646-0.910, respectively.Conclusion Tr-PEESS v2.0 is a valid and reliable tool to use in Turkish children. GaziESAS is a new parent-proxy pediatric EoE scale with an additional adaptive behavior domain that passed scale developmental stages successfully for Turkish children with EoE.     

Folliculitis decalvans-like pustular plaques on the limbs sparing the scalp

Folliculitis decalvans is a neutrophilic cicatricial alopecia characterised by progressive pustular folliculitis. Folliculitis decalvans is seen as a condition usually limited exclusively to the scalp and rarely affects the limbs. We present a case of a 63-year-old man with a 3-year history of progressive pustular folliculitis with inflammatory patches and central scarring alopecia on both forearms and a circumscribed patch on his right lower leg. His presentation, clinical course and isolation of Staphylococcus aureus together with the histopathological findings all supported a folliculitis decalvans-like pustular folliculitis limited to the limbs. Biopsies revealed follicular pustules, gross interfollicular fibrosis with plasma cells and concentric perifollicular fibrosis with lymphocytes, all features seen with folliculitis decalvans. The positive response to antibiotics combined with topical corticosteroids mirrored the response seen with scalp folliculitis decalvans. In contrast to the previously reported cases, the patient had no evidence of folliculitis decalvans on the scalp.     

Eosinophilic fasciitis associated with generalized morphea and IgA nephropathy

Eosinophilic fasciitis (EF) is a rare connective tissue disease characterized by increased peripheral blood eosinophils and diffuse fasciitis, generalized morphea (GM) is a subtype of localized scleroderma, and IgA nephropathy is a chronic glomerulonephritis caused by abnormal deposition of IgA in the mesangial area of the glomeruli. We describe a 49‐year‐old male patient with hard skin, cutaneous hyperpigmentation, and proteinuria. The patient had suffered from a long disease course of hard skin, while urine protein was newly detected. Finally, the clinical presentation and physical examination, limb MRI, skin biopsy, and renal biopsy confirmed the diagnosis of eosinophilic fasciitis associated with generalized morphea and IgA nephropathy. This case is the first report of EF associated with GM and IgA nephropathy.     

Two region‐dependent pathways of eosinophilic neuronal death after transient cerebral ischemia

Various types of eosinophilic neurons (ENs) are found in the post‐ischemic brain. We examined the temporal profile of ENs in the core and peripheral regions of the ischemic cortex, and analyzed the relationship to the expression of various cell death‐related factors. Unilateral forebrain ischemia was induced in Mongolian gerbils by transient common carotid artery occlusions, and the brains from 3 h to 2 weeks post‐ischemia were prepared for morphometric and immunohistochemical analysis of ENs. ENs with minimally abnormal nuclei and swollen cell bodies appeared at 3 h in the ischemic core and at 12 h in the periphery. In both locations multiple cell death‐related factors including calcium, µ‐calpain, cathepsin D, 78 kDa glucose‐regulated protein (GRP78) and ubiquitin were activated. In the ischemic core, pyknosis and irregularly atrophic cytoplasm peaked at 12 h, which was associated with significant increases in staining for calcium and µ‐calpain. ENs with pyknosis and scant cytoplasm peaked at 4 days and were positive for TUNEL and calcium staining. In the ischemic periphery, ENs had slightly atrophic cytoplasm and sequentially developed pyknosis, karyorrhexis and karyolysis over 1 week. These cells were positive for TUNEL and calcium staining. All types of EN were negative for caspase 3. There may be two region‐dependent pathways of EN changes in the post‐ischemic brain: pyknosis with cytoplasmic shrinkage in the core, and nuclear disintegration with slightly atrophic cytoplasm in the periphery. This difference coordinates different activation patterns of cell death‐related factors in ENs.