Acrodermatitis enteropathica: the need for sustained high dose zinc supplementation

Acrodermatitis enteropathica (AE) is a rare congenital disorder owing to an abnormality with intestinal absorption and/or transportation of zinc. We describe two male siblings, who presented with evidence of both acute and chronic zinc deficiency, despite being diagnosed with AE very early in life. We wish to highlight the importance of sustained high-dose zinc supplementation and regular monitoring in AE cases. Proper counselling of parents about the need for life long supplementation and increasing requirements with age is essential

Acrodermatitis enteropathica: the need for sustained high dose zinc supplementation

Acrodermatitis enteropathica (AE) is a rare congenital disorder owing to an abnormality with intestinal absorption and/or transportation of zinc. We describe two male siblings, who presented with evidence of both acute and chronic zinc deficiency, despite being diagnosed with AE very early in life. We wish to highlight the importance of sustained high-dose zinc supplementation and regular monitoring in AE cases. Proper counselling of parents about the need for life long supplementation and increasing requirements with age is essential

Is it lucio phenomenon or necrotic erythema nodosum leprosum?

Lucio phenomenon (LP) or erythema necroticans is a relatively rare, peculiar reaction pattern occurring in untreated lepromatous (LL) or borderline lepromatous (BL) leprosy cases. A 38-year-old male, a cook by occupation, was referred to the dermatology clinic from otolaryngology department with blistering over both the hands and feet of 2 days duration. He had been admitted 1 week back with epistaxis and nasopharyngeal myiasis in otolaryngology department. He was started on systemic antibiotics gentamycin, crystalline penicillin, and metronidazole with nasal instillation of turpentine oil 2 drops 6 times a day. Two days later, he had developed edema with painless hemorrhagic blistering over the dorsum of left hand followed by involvement of the right hand, dorsa of both feet, and both the earlobes within a day. Histopathology of the blister showed sub-epidermal blister, with necrotizing leukocytoclastic vasculitis of papillary dermal vessels with thrombosis, numerous acid-fast bacilli in macrophages, and macrophage granulomas extending up to subcutis. In view of the absent fever or constitutional symptoms, and the classical angular infarcts and hemorrhagic blisters evolving into ulcers with angulated margins, we considered LP as the most likely diagnosis. The patient was started on a combination of WHO recommended multibacillary anti-leprosy therapy and prednisolone (40 mg/day)

Melanogenesis Markers Expression in Premature Graying of Hair: A Cross-Sectional Study

&lt;b&gt;&lt;i&gt;Background:&lt;/i&gt;&lt;/b&gt; Studies on mice and aging human hair follicles provide compelling evidence that graying of hair results from premature differentiation of melanocyte stem cells in the niche/bulge. &lt;b&gt;&lt;i&gt;Objective:&lt;/i&gt;&lt;/b&gt; The aim of this study was to analyze whether differentiation of melanocyte stem cells is responsible for premature graying of hair (PGH). &lt;b&gt;&lt;i&gt;Methods:&lt;/i&gt;&lt;/b&gt; Twenty-five patients with PGH (&lt;i&gt;n&lt;/i&gt; = 25) attending the dermatology department were recruited. Five unpigmented and 5 pigmented hairs were obtained per patient by separating individual follicles after 1 mm punch biopsies. The hairs were dissected at a distance of 2 mm from the bulb to separate the stem cells (upper segment – US) from the melanocytes (lower segment – LS). RNA was extracted from hair follicle US and LS, and expression of GP100, tyrosinase (TYR), and tyrosinase-related protein-1 (TYRP1) genes was quantified using Qiagen one-step RT-PCR kit. &lt;b&gt;&lt;i&gt;Results:&lt;/i&gt;&lt;/b&gt; We found melanogenesis gene expression in both temporary (US) and permanent (LS) segments of unpigmented and pigmented hair follicles. When compared between the US and LS of white hair, the expression of TYR and GP100 was much higher in US than LS, suggestive of melanogenesis in the bulge. Similarly, when compared between white and black US, the expression of all 3 genes was higher in white US than black US, although not statistically significant. &lt;b&gt;&lt;i&gt;Limitations:&lt;/i&gt;&lt;/b&gt; Low samples size and lack of data pertaining to the expression of genes at protein level are the limitations of current study. &lt;b&gt;&lt;i&gt;Conclusion:&lt;/i&gt;&lt;/b&gt; Even though this pilot study data yielded key information about the expression of GP100, TYR, and TYRP-1 at the mRNA level, further studies quantifying the expression of these genes at protein level are needed to provide additional clues to further address the results in detail. </jats:p