Pachyonychia congenita (PC) is a rare genodermatosis with only 450 cases reported since 1906. It is of two types, type I due to mutation in genes 6a and 16, and 6b and 17 in type II with an autosomal dominant inheritance in both types. A 22 yr old female patient presented in our OPD with hypertrophy of finger and toe nails, palmoplantar keratoderma, oral punctuate leukokeratosis, hyperhidrosis in palms and soles with maceration and malodour since childhood. She had a positive family history with father and grandfather affected but less severely. Microscopy and culture of nail clippings and scrapping were done to rule out fungal infection. On biopsy acanthotic epidermis, parakeratosis, orthokeratosis were seen. No evidence of any associated malignancy was found after thorough workup. She was diagnosed as PC Type 1. She was put on topical steroids and orally on acetretin 25 mg OD. Paring of the nails was done too reduce the thickness of nails & to provide symptomatic relief. She was on a regular treatment for 3-4 months and showed some improvement in the form of reduced palmoplantar hyperkeratosis and reduced oral punctate keratosis but was later lost on followup. She showed no adverse effect to therapy during this period. This case is being reported because of its rarity.

Iron deficiency anaemia (IDA) is common in Indian patients with rheumatoid arthritis (RA). We evaluated red blood cell indices, serum iron related and bone marrow iron stores measurements in diagnosing iron deficiency in patients with RA. Fifty consecutive anaemic patients with RA had their complete blood counts, red cell indices, serum iron, serum ferritin and serum total iron binding capacity (TIBC) measured and underwent posterior iliac crest bone marrow aspiration. Fixed smears were stained for iron with Perl's Prussian blue and patients who had no (grade 0) or minimal stainable iron (grade I) were regarded as iron deficient and rest iron replete (grade II-IV) and hence as having anaemia of chronic disease (ACD). To determine diagnostic power of tests used for diagnosing iron deficiency in addition to positive likelihood ratio, sensitivity, specificity and negative predictive values; receiver operating characteristics (ROC) curves were plotted and areas under the receiver-operating curves were compared. Eighteen patients (36%) had IDA and 32 (64%) had ACD. Correlation between the bone marrow iron stores and serum ferritin was poor in the IDA group (r = -0.09, P = 0.57) and significant in the ACD group (r = 0.79, P < 0.0001). Areas under the ROC curves for mean corpuscular haemoglobin (MCV), serum iron, TIBC and mean corpuscular haemoglobin concentration (MCHC) were relatively low (0.52, 0.71, 0.75 and 0.77, respectively) and these tests had low positive likelihood ratios (1.08, 2.13, 4.62 and 1.5, respectively). Both area under ROC curve [0.98, 95% confidence interval (0.94, 0.99)] and negative predictive value (97%) were highest when cut off serum ferritin was <82 microg/l. In patients with RA serum iron, TIBC, MCV and MCHC have limited value in diagnosing iron deficiency. In this study compared to American and European studies a much higher cut off value of serum ferritin had most diagnostic power for detecting iron deficiency. Bone marrow iron stores measurements appears to be the most reliable method for diagnosing IDA however, it needs to be taken in conjunction with other laboratory findings and the clinical scenario.

Prematurity is a risk factor for hypertension, vascular stiffness, nephron deficit and adult onset cardiorenal disease. The vascular tree and kidneys share morphogenic drivers that promote maturation in utero before 36 weeks of gestation. Vascular elastin accrual terminates after birth leaving collagen to promote vascular stiffness. Our objective was to determine if the histomorphometry of the umbilical artery, an extension of the aorta, parallels nephron mass across gestational age groups. From a cohort of 54 newborns, 32 umbilical cord specimens were adequate for evaluation. The umbilical cord was sectioned, stained with trichrome, and digitalized. Muscular and collagenous areas of the umbilical artery were measured in pixels using the Image J 1.48q software. Total kidney volume was measured by ultrasound and factored by body surface area (TKV/BSA). The umbilical artery total area was significantly greater in term v. preterm infants (9.3±1.3 v. 7.0±2.0 mm2; P<0.05) and increased with gestational age; while the percent muscular and collagen areas were independent of gestational age (R 2=0.04; P=ns). Percent muscular area correlated positively with TKV/BSA (r=0.53; P=0.002); while an increase in collagen correlated inversely with kidney mass (r=-0.53; P=0.002). In conclusion, an enhanced % muscular area and presumed vascular elasticity was associated with increased renal mass in all infants. Umbilical artery histomorphometry provides a link between the intrauterine environment, vascular and kidney development.

ABSTRACT:Oral mucosal health is an integral part of our holistic well-being. Complete skin examination includes those of mucosa, with oral mucosa being the most readily accessible and amenable to examination. Many dermatological diagnoses have an essential mucosal component with the latter adding to the diagnostic information. Apart from this, oral mucosa may harbour incidental abnormalities which either the patient may not be aware of or seems trivial enough to be given due consideration. These findings may nevertheless be quite important and some may need management too. Few studies in the past have attempted to study epidemiological trends of oral mucosal involvement in dermatology outpatient settings. (1,2,3,4) We studied oral mucosa of patients attending dermatology outpatient department. 51.8% patients had oral mucosal findings with 50.1% being incidental not related to the dermatological diagnosis.

ABSTRACT:Contact dermatitis is an inflammatory response of the skin to an exogenous substance (irritant and/or allergen). It can be classified as follows: Irritant contact dermatitis, Allergic contact dermatitis, Photo contact, phototoxic dermatitis, Immediate contact reactions, Non-eczematous reactions Only the superficial regions of the skin are affected in contact dermatitis. Inflammation of the affected tissue is present in the epidermis and the outer dermis. 1 Unlike contact urticaria, in which a rash appears within minutes of exposure and fades away within minutes to hours, contact dermatitis takes days to appear. In our study we have shown the list of most common allergens found positive in our patients. STUDY DESIGN: It was a cross sectional study. METHOD: Study was conducted in mahatma Gandhi hospital Jaipur over a period of 4 months of duration. Patients suspected to have contact dermatitis were subjected to patch testing. Based on the history clinical signs and symptoms. Total 42 patients were short listed in 4 months of duration. RESULT: Our study showed that the most common allergen showing patch test positivity in both males and females were nickel sulphate, cophony, and cobalt sulphate. Patients showed significant improvement in their dermatitis after avoidance of the offending agent. CONCLUSION: Patch test is the method of choice and the "gold standard" in the detection of contact allergy and allergic contact dermatitis.

Evolutionarily, as man developed and manual dexterity increased, nail became an important appendage of the human anatomy. As civilization progressed and social interactions grew, nails, like hair, have also become objects of attention and adornment. However, nail cosmetology as it is evolving today is a fairly recent development. We observed nails of all outpatient attendees at one clinic in Dermatology department, Mahatma Gandhi Hospital, Jaipur over a period of one week in October 2013.

Background:  Low bone mass in an Indian population combined with bone loss secondary to rheumatoid arthritis (RA) would predict high prevalence of osteoporosis in Indian patients with RA. We used quantitative ultrasound (QUS) to assess bone mass in RA patients and compared it with healthy controls. Methods:  One hundred patients with RA and 284 randomly selected healthy controls underwent QUS measurement at the heel. Achilles ultrasonometer was used to measure stiffness index (SI) values and T‐scores. Results:  Both groups did not differ significantly in proportion of female gender and postmenopausal females. Prevalence of low bone mass in both the healthy population and RA patients was high. Both SI and T‐scores were lower in RA patients and they were significantly reduced in postmenopausal patients; 31% of patients with RA had osteoporosis as compared to 14% of controls. There was no statistically significant correlation between disease duration and T‐scores (r = 0.11, P = 0.26) and disease duration and SI (r = 0.12, P = 0.20). Conclusion:  Our study has found that both the healthy population and RA patients had lower bone mass compared to Western studies. Prospective studies are required to establish whether this is associated with fractures and to establish factors responsible for these findings.

INTRODUCTION-Reproductive tract infections (RTIs) and sexually transmitted infections (STIs) represent a major public health problem in developing countries, so their quantification and study is very important. METHODOLOGY-Case records of 231 patients with RTIs and STIs who attended the Department of Dermatology and Venereology, Mahatma Gandhi Medical College and Hospital, Jaipur for a period of seven months from March 2011 to April 2013 were analysed. RESULTS-The total number of cases studied were 231, out of which 137 cases ( 59%) were males and 94 cases (41%) were females , with a ratio of males to females of 1.4 : 1.