Neurofibromatosis-1 (NF-1) is an autosomal dominant neurocutaneous syndrome with incomplete penetrance and variable phenotypic expressivity. Although it may affect any system of the body, the disease remains generally innocuous. NF-1 has been commonly linked to neurological symptoms, including headaches, seizures, cognitive deficits, and learning disabilities, and others. While optic glioma is the most common CNS complication, vasculopathy and cerebrovascular anomalies have also been rarely reported. Specifically, intracerebral hemorrhage with vascular stenosis in NF-1 is an extremely rare manifestation. Herein, we report a patient with NF-1 presenting with stroke secondary to intracranial hemorrhage and underlying quasi-Moyamoya disease. Since the cutaneous features of NF-1 appear early in life and are striking, dermatologists might be the first contact for such patients with the medical system, and consequently play an important role in the diagnosis, evaluation, and prevention of complications.
BACKGROUND Background: Premature greying of hair (PGH) is a clinical entity which has multiple ramifications such as low self- esteem, inferiority complex and may interfere with an individual’s social interaction capabilities and hence adversely affect the individual’s psychosocial health. As there is paucity of epidemiological, investigative and psychoanalytical studies on PGH from the Indian subcontinent, the present study has been undertaken to ascertain role of biochemical factors and assess knowledge, attitude, practice in patients of premature greying of hair, so as to explore the possible association of this entity with common systemic disorders and deficiencies which may possibly be related to its etiology and study comprehensively in detail the impact of quality of life in these patients of PGH by using a detailed KAP questionnaire. OBJECTIVE Objective: To study the biochemical factors and knowledge, attitude, practice in patients of premature greying of hair. METHODS Materials and methods: A case control study including 75 cases and equal number of age and sex matched controls was conducted in a tertiary care hospital. Various epidemiological variables and biochemical parameters (Vit B12, Vit D, Calcium, Iron profile, lipid profile, thyroid hormones) were ascertained and these were compared between the cases and controls. Severity of greying was assessed using Greying severity score (GSS) and a knowledge, attitude, practice study was conducted by administering a pre validated questionnaire to assess impact on quality of life of these affected individuals. RESULTS Results : Significant differences between cases and controls in the serum levels of biochemical parameters namely Vit B12 (P =.001), Vit D (P = .004), serum iron (P <.001), ferritin (P <.001) & TIBC(P = .037), and thyroid hormones T4 (P =.002) & TSH (P= .041) was found. No correlation between PGH and serum calcium & lipid profile was derived. Along with this, statistically significant difference was noted in the appetite pattern of cases and controls (P =.003). Stress, smoking, altered sleep pattern and bowel habits were also more prevalent in cases as compared to controls. It was also noted that majority study cases belonged to lower middle class of the modified Kuppuswamy scale. Through KAP study, which was extremely comprehensive and detailed in nature, it was observed that PGH has a significant affect on quality of life of these patients. CONCLUSIONS Conclusion: The study reveals a strong association between PGH and deficiency of Vit D, abnormal levels of Vit B12, hypothyroidism and deranged iron profile; and altered appetite patterns. Along with this prevalence of stress, smoking, altered sleep pattern and bowel habits was also higher in PGH cases. Hence, all these parameters should be analysed in such patients. KAP analysis was a unique approach to study common conceptions and practices in PGH cases and it’s psychological impact.
Cutaneous Pili Migrans (CPM) is a rare creeping eruption with only 40 cases reported so far. CPM is defined as a condition where a hair shaft or fragments gets embedded in superficial skin. It is known to affect both adults and children. We report a 23-year-old male patient who presented with sudden sharp pain in the foot on walking. Cutaneous examination revealed a black linear thread-like lesion on the medial plantar aspect of the right great toe associated with mild to moderate tenderness. The particle was removed by superficial paring along with gentle forceps extraction. Microscopic examination confirmed it to be a hair shaft fragment. While the exact etiology of CPM is unclear, it is proposed to be an acquired condition. Since it can mimic other creeping eruptions like Larva migrans, it is important to be aware of this condition.
Necrobiosis lipoidica diabeticorum (NLD) is a chronic non-infectious granulomatous disease characterized by sclerodermiform plaques, papules, or inflammatory nodules. NLD may be associated with diabetes mellitus (DM), sarcoidosis, rheumatoid arthritis, and inflammatory bowel disease. While reports of patients having NLD, DM, and vitiligo at the same time exist, there are, to the best of our knowledge, no reports of the cooccurrence of NLD and vitiligo in the absence of evidence of DM. Herein, we report a 57-year-old, non-diabetic vitiligo patient, who had developed yellowish-brown plaques over bilateral shins with central scarring and atrophy. Histopathology was compatible with NLD. While there is a case report of a patient having psoriasis, NLD, DM, and vitiligo concurrently, this case, having NLD and vitiligo without clinically evident DM, is prominent. Since around 20–40% of cases of vitiligo may have metabolic syndrome, this case draws attention to the possible overlap in pathogeneses and the need for the intensive monitoring of such patients.
Submission of an original paper with copyright agreement and authorship responsibility.I (corresponding author) certify that I have participated sufficiently in the conception and design of this work and the analysis of the data (wherever applicable), as well as the writing of the manuscript, to take public responsibility for it. I believe the manuscript represents valid work. I have reviewed the final version of the manuscript and approve it for publication. Neither has the manuscript nor one with substantially similar content under my authorship been published nor is being considered for publication elsewhere, except as described in an attachment. Furthermore I attest that I shall produce the data upon which the manuscript is based for examination by the editors or their assignees, if requested.Thanking you.
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