A Rare Case of Acrodermatitis Enteropathica in a One Year Old Child.

Acrodermatitis enteropathica (AE) is a rare autosomal recessive disorder caused by zinc deficiency.[1] It can be classified as primary zinc deficiency, genetically based zinc deficiency (classical, AE, acquired zinc deficiency of lactogenic origin), and acquired secondary zinc deficiency.[2] Genetic zinc deficiency is associated with the defects in two zinc transporters, of which one is involved in intestinal zinc uptake ZRT- and IRT-like Protein-4 (ZIP), causing classical and AE. The other is responsible for zinc secretion in breast milk zinc transporter-2 (ZnT) resulting in zinc deficiency of lactogenic origin.[3] Here, we have discussed and reviewed the clinical aspects and probable role of zinc transporters in the manifestation of AE.Acrodermatitis enteropathica (AE), manifests as acral and periorificial dermatitis, alopecia, intractable diarrhoea, and failure to thrive. It is classified as primary zinc deficiency, genetically based deficiency, and acquired secondary deficiency. We hereby report a case of genetically based AE in a one year old child. After reviewing the literature, we have also emphasized the possible role of genetics in the manifestation of AE

A Rare Case of 10-Year-Old Glomus Tumour with Excellent Response to Surgical Excision

Glomus body is an arteriovenous anastomosis located mostly in the finger nailbed and has a role in thermoregulation. Glomus tumour is a rare benign hamartoma, comprising of vascular spaces (the Sucquet-Hoyer canal) internally lined by endothelium and externally surrounded by glomus cells, arising from the glomus body at arterial end. 1,2 Wood first reported it in 1812.1 Most of the glomus tumours are small, benign neoplasia located in the dermis and subcutaneous tissue of the extremities.3 They can vary in number from being solitary most often or multiple in few cases. They can develop in any part of the body. They account for 1-5% of all hand tumours. They are most commonly seen in middle age women. One such rare case of glomus tumour of 10 years duration is reported here.

A Rare Case of Herpes Zoster Ophthalmicus Secondary to Methotrexate Induced Immunosuppression.

Herpes zoster ophthalmicus (HZO) is defined as herpes zoster (HZ) due to involvement of the ophthalmic division of the fifth cranial nerve.1 It is the second most common type of herpes zoster, after thoracic zoster. Herpes zoster affects about 20% of the world's population at least once in their lifetime, with nearly 20% of these showing an ophthalmic involvement.2 It is estimated that 1 million adults in the USA are afflicted with herpes zoster every year. The risk of developing herpes zoster increases considerably with age, reaching 50% in those aged 85 or older. Advanced age and dysfunctional cell-mediated immune responses are two well-established risk factors for varicella zoster virus reactivation. Other risk factors, such as female gender, Caucasian ethnicity, diabetes mellitus, psychological stress, mechanical trauma, heavy metal exposure, as well as family history, have also been postulated. It causes debilitating pain, neuropathy and inflammatory complications.3

Fetal varicella syndrome.

Fetal varicella syndrome is a rare condition of the newborn, presenting with cutaneous scars, limb defects and ocular and central nervous system abnormalities. It is due to varicella or zoster developing in the fetus following maternal varicella infection during early pregnancy. We are reporting one such patient who presented with a linear, depressed, erythematous scar over the left forearm and axillary fold, with a history of maternal chicken pox during the first trimester of pregnancy.

Nail changes and disorders among the elderly.

Nail disorders are frequent among the geriatric population. This is due in part to the impaired circulation and in particular, susceptibility of the senile nail to fungal infections, faulty biomechanics, neoplasms, concurrent dermatological or systemic diseases, and related treatments. With aging, the rate of growth, color, contour, surface, thickness, chemical composition and histology of the nail unit change. Age associated disorders include brittle nails, trachyonychia, onychauxis, pachyonychia, onychogryphosis, onychophosis, onychoclavus, onychocryptosis, onycholysis, infections, infestations, splinter hemorrhages, subungual hematoma, subungual exostosis and malignancies. Awareness of the symptoms, signs and treatment options for these changes and disorders will enable us to assess and manage the conditions involving the nails of this large and growing segment of the population in a better way.

A comparative evaluation of skin and nerve histopathology in single skin lesion leprosy.

BACKGROUND: In spite of leprosy being a disease of nerves, ROM therapy for single skin lesion leprosy was based on clinical trials without much evidence-based studies of nerve pathology. The present study was undertaken to compare the histology of skin and nerve in single skin lesion leprosy, and to assess the scientific rationale and justification of single dose ROM therapy. METHODS: Twenty-seven untreated patients with single skin lesion without significantly thickened peripheral nerves were selected. Skin and nearby pure cutaneous nerve biopsies were studied under both H&E and Fite's stain. RESULTS: All the skin biopsies were negative for AFB and clinico-pathological correlation was positive in 51.85% of skin biopsy specimens. Histopathological diagnosis of leprosy was evident in 55.5% of clinically normal looking nerves, with AFB positivity in 29.6% of nerve biopsy specimens. Correlation between clinical diagnosis and nerve histopathology was poor (26%). CONCLUSIONS: Single skin lesion without thickened peripheral nerves as criteria for single dose ROM therapy is not logical, since the histological diagnosis of leprosy in normal looking nerves with presence of AFB is revealed in this study. Pure cutaneous nerve biopsy is a simple outpatient procedure, without complications. This study emphasizes the need to consider nerve pathology as an important tool for further therapeutic recommendations, than just clinical trials and skin pathology alone. Though single dose ROM therapy has been withdrawn recently, the principle holds good for any future therapeutic recommendations.