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Oral manifestations may be the first sign of a systemic disease, or represent lesions associated with an established or recurrent disease. Oral health care providers are often the first to recognize these signs. Some lesions have characteristic features that allow for early detection and intervention. On the contrary, clinical manifestations may be diverse and require a comprehensive evaluation to establish a definitive diagnosis. This article reviews the oral manifestations of select systemic diseases to help clinicians develop a differential diagnosis that leads to early diagnosis and timely intervention.

UNASSIGNED: Confluent reticulated papillomatosis (CRP), terra-firme-forme-dermatitis (TFFD), and dermatitis neglecta (DN) are the benign, acquired conditions that present with dirty-looking hyperpigmented and hyperkeratosis lesions on neck, back, and truncal areas. They pose difficulty in diagnosis because of their clinical similarities and thereby in treatment approaches. Bedside test and histopathology is helpful in the diagnosis. Dermoscopy is utilized as an evidence-based diagnostic method.
UNASSIGNED: To evaluate and to compare the dermoscopic patterns among CRP, TFFD, and DN and to correlate them in terms of histopathological features.
UNASSIGNED: It was a cross-sectional observational study with a total of 62 patients, among whom 30 were CRP, 20 had TFFD, and 12 were diagnosed as DN. Clinical and dermoscopic evaluation was done in all patients, and histopathology was carried out in selective cases to confirm the diagnosis.
UNASSIGNED: Global view of dermoscopy in CRP revealed a cerebriform pattern. The arrangement of pigment globules was characteristic in CRP, TFFD, and DN, giving a \"cobblestone,\" \"mosaic,\" and \"cornflake-like\" appearance, respectively. The color of the pigment globules was strikingly significant. Yellow globules were predominant in CRP and TFFD, having p values of 0.001 and 0.004, respectively. Scales were conspicuously present in CRP, with a statistically significant p value of 0.003. Focal white areas and hair changes were observed in CRP alone, whereas black dots were found only in TFFD.
UNASSIGNED: Dermoscopy acts as an in vivo and a noninvasive, rapid technique in the diagnosis of clinically look-alike conditions. It demonstrates characteristic features in CRP, TFFD, and DN. Thus, it is an evidence-based diagnostic method that assists the treating physician in daily clinical practice.

GPR56 gene is best known for its pivotal role in cerebral cortical development. Mutations inGPR56give rise to cobblestone-like brain malformation, white matter changes and cerebellar dysplasia. This study aimed to identify causative variant in a consanguineous family having five individuals affected with developmental delay, mild to severe intellectual disability, speech impairment, strabismus and seizures. Whole exome sequencing was performed to identify mutation in affected individuals. Variants were filtered and further validated by Sanger sequencing and segregation analysis. A novel frameshift variant c.1601dupT leading to p.Ala535GlyfsTer17) was identified in GPR56 gene by whole exome sequencing and subsequent filtering. All five affected individuals were homozygous for the mutant allele while four asymptomatic individuals carried the variant in heterozygous state. Radiological findings of a representative patient presented features of GPR56-associated cobblestone like brain malformation. MRI findings suggested paucity of sulci, dilated ventricular system and brainstem atrophy. The microgyria were observed in a simplified gyral pattern (cobblestone). This single bp insertion, and the consequent frameshift, results in the truncation of GPR56 protein. This could result in a malformed cortex giving the brain a cobblestone like shape. Our study identified a 7th novel frameshift variant from Pakistani population in GPR56 gene, thus broadening mutation spectrum.

Pseudoxanthoma elasticum (PXE)-like papillary dermal elastolysis and focal dermal elastosis are acquired noninflammatory elastic tissue disorders. The skin lesions clinically resemble PXE without systemic involvement. We report the clinicopathological features of five cases and discuss the pathogenesis and differential diagnosis. Increased awareness of this newly described entity will broaden the differential diagnosis for dermatologists when encountering a similar case scenario.

BACKGROUND: Nevus sebaceous (NS) presents as alopecia and yellowish discoloration during infantile stage. In adult stage, lesions become verrucous. Importantly, various appendageal tumors such as trichoblastoma, syringocystadenoma papilliferum and basal cell carcinoma develop during this stage. Hence it is very important to follow the course of NS for early detection of neoplasms. Trichoscopy, being in vivo diagnostic technique, can be utilized in this condition. as it demonstrates specific trichoscopic patterns. Patterns of NS on trichoscopy are evaluated.
METHODS: Study was conducted in SN Medical College, Bagalkot. It is a case series study. Ten patients with NS were studied using polarised dermoscopy. All NS lesions were subjected to histopathology for confirmation of diagnosis. Correlation of histopathological changes and trichoscopic patterns was done. Data were tabulated in Microsoft excel sheet. The results are presented in proportions and percentages.
RESULTS: Total of 10 patients including 6males and 4 females were present in the study. The mean age was 20years. Two patients were with childhood stage and 8 patients were with adulthood stage of NS. Trichoscopy showed \'cobble stone pattern\' in 20% and \'cerebriform pattern\' in 80% of patients. Histopathological features showed numerous and hyperplastic sebaceous glands with primordial hair follicles which were consistent with NS in all the patients.
CONCLUSIONS: Trichoscopy helps in the diagnosis of NS with specific patterns. Yellowish globules in the cobblestone pattern and cerebriform patterns are specific to childhood and adult stages of NS respectively. Hence, trichoscopy is good, non-invasive diagnostic technique in NS. Authors strongly recommend regular trichoscopic examination of NS.

\'Crohn\'s disease\' is an inflammatory granulomatous disease of the gastrointestinal tract with extra-intestinal manifestations. Oral lesions may precede the intestinal disease and serve as a source for histological diagnosis. We present a case of orofacial Crohn\'s disease where orofacial symptoms were present for about 13 years and occasional constipation was present, since 6 months. Oral examination plays an important role in early diagnosis of Crohn\'s disease.

Vernal keratoconjunctivitis (VKC) is an unusually severe sight-threatening allergic eye disease, occurring mainly in children. Conventional therapy for allergic conjunctivitis is generally not adequate for VKC. Pediatricians and allergists are often not familiar with the severe clinical symptoms and signs of VKC. As untreated VKC can lead to permanent visual loss, pediatric allergists should be aware of the management and therapeutic options for this disease to allow patients to enter clinical remission with the least side effects and sequelae. Children with VKC present with severe ocular symptoms, that is, severe eye itching and irritation, constant tearing, red eye, eye discharge, and photophobia. On examination, giant papillae are frequently observed on the upper tarsal conjunctiva (cobblestoning appearance), with some developing gelatinous infiltrations around the limbus surrounding the cornea (Horner-Trantas dot). Conjunctival injections are mostly severe with thick mucus ropy discharge. Eosinophils are the predominant cells found in the tears and eye discharge. Common therapies include topical antihistamines and dual-acting agents, such as lodoxamide and olopatadine. These are infrequently sufficient and topical corticosteroids are often required for the treatment of flare ups. Ocular surface remodeling leads to severe suffering and complications, such as corneal ulcers/scars. Other complications include side effects from chronic topical steroids use, such as increased intraocular pressure, glaucoma, cataract and infections. Alternative therapies for VKC include immunomodulators, such as cyclosporine A and tacrolimus. Surgery is reserved for those with complications and should be handled by ophthalmologists with special expertise. Newer research on the pathogenesis of VKC is reviewed in this article. Vernal keratoconjunctivitis is a very important allergic eye disease in children. Complications and remodeling changes are unique and can lead to blindness. Understanding of pathogenesis of VKC may lead to better therapy for these unfortunate patients.