The hyper-IgE syndrome (HIES) is a rare group of primary immunodeficiency characterised by recurrent infections, eczema, and elevated serum levels of IgE. Autosomal dominant HIES is caused by mutations in transcription factor – signal transducer and activator of transcription-3. Autosomal-recessive (AR) HIES was described in 2004 due to mutation of tyrosine kinase 2 gene, and subsequently, another mutation in dedicator of cytokinesis 8 gene was discovered in 2009. Although both the forms have many common clinical features, few characteristic findings help in differentiating them. AR-HIES is characterized by recurrent bacterial and viral infections, atopic eczema, and raised serum IgE levels. We report a case of a 4-year-old girl presenting with the features of AR-HIES to highlight the presentation of this rare disease.
We report two cases of epitheloid hemangioma presented with multiple nodular lesions over head and neck region. One of them gave history of bleeding on minor trauma. Pyogenic granuloma was considered as a differential diagnosis from the morphological appearance and history of bleeding. Nodular leprosy, sarcoidosis, and secondary syphilis were also considered. Histopathological examination of both was typical of epitheloid hemangioma, an entity commonly overlooked clinically due to its rarity.
Multiple myeloma (MM) is a proliferative disorder of plasma cells which produce abnormal immunoglobulin proteins. Skin involvement is rarely found in this disorder. They are either specific or nonspecific lesions. We report four such interesting patients who presented to us initially with common dermatoses such as leukocytoclastic vasculitis, pyoderma gangrenosum, and vesiculobullous disorders and were subsequently diagnosed to have MM. There were no skeletal involvements or renal function abnormality at the time of presentation. Unusual presentation, nonresponsiveness to conventional therapy, and abnormal blood parameters prompted us to suspect some underlying systemic conditions which were later confirmed to be MM after serum immunoelectrophoresis for M-band and bone marrow biopsy.
Background: As face is the index of the mind, so is the nail the index to health, as the nail is capable of mounting only a limited number of reaction patterns to the large number of disorders affecting it. Dermoscopy is thus a valuable aid not only in enhancing visible nail features but also in revealing cryptic features of diagnostic value. Aims: To study the clinical and dermoscopic features in nails of papulosquamous disorders and correlate it with disease severity. Methods and Material: This was a cross-sectional study with convenient sampling. After obtaining ethical clearance, according to inclusion and exclusion criteria, papulosquamous disorders were enrolled in the study. Finger nails and toe nails were numbered 1–10. Detailed clinical examination was done. Wet and dry dermoscopic examination was made in both polarised and non-polarised mode using ultrasound (USG) gel. Psoriasis area and severity index (PASI) and body surface area (BSA) were compared with nail changes. Statistical analysis of data was performed using the Statistical Package for the Social Sciences (SPSS) version 26. Results: Out of 203 patients, 117 were male. Psoriasis was the most common disease (55.6%). A total of 65.51% of patients had nail changes. Pitting was the most common finding in psoriasis, both dermoscopically and clinically. Splinter haemorrhage, oil drop, dilated capillaries, and pseudofibre sign were detected better on dermoscopy (P < 0.05). Positive correlation was found between PASI and nail psoriasis severity index (NAPSI). A strong correlation was also found between clinical (cNAPSI) and dermoscopic (dNAPSI). Thinning was the most common in lichen planus. No relation between BSA and nail changes was observed. Conclusions: Dermoscopy is thus a valuable aid not only in enhancing visible nail features but also in revealing cryptic features of diagnostic value and reducing the requirement for invasive procedures like nail biopsies, early diagnosis, directing management.
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