A Rare Association of Hydrops of Gall Bladder with Hepatitis E Infection.

Gall bladder distension with acute viral acalculous cholecystitis is an extremely rare event especially with Hepatitis E infection in paediatric cases with a high incidence of perforation, gallbladder necrosis and mortality. We report a four year old male child presenting with fever, vomiting, pain abdomen, mild hepatosplenomegaly and tenderness in right hypochondrium. Laboratory investigations revealed hyperbilirubinemia and elevated liver enzymes, but there was no evidence of bacterial or parasitic infection. Serology for viral hepatitis suggested acute Hepatitis E infection. Ultrasonographically distended inflamed gallbladder without calculous was observed. Finally acute acalculous cholecystitis due to Hepatitis E virus was diagnosed and the child responded to the conservative management.

Tuberous sclerosis with portal vein thrombosis, protein C and S deficiency.

A 29-year-old lady with a bad obstetric history and portal vein thrombosis, presented to the Skin OPD for facial lesions. On examination, angiofibromas on face, shagreen patch and periungual fibromas were observed. She also had dental pits and a retinal hamartoma. Investigations revealed hamartomas in the brain and kidney. Hematological work-up showed protein C and S deficiency with Factor V Leiden positivity. Except for the cutaneous symptoms, the patient did not have any clinical manifestations in other organs affected by tuberous sclerosis. A similar association of tuberous sclerosis with protein C deficiency has been reported in only one case in literature.

Undiagnosed purpura: a case of autoerythrocyte sensitization syndrome associated with dermatitis artefacta and pseudo-ainhum.

A 23-year-old young woman presented with recurrent episodes of painful bruising along with linear erosions on the accessible areas of the body of nine years duration with a pseudo-ainhum of her left nipple for the past three months. Her case history included repeated visits to various physicians at different centers and an extensive investigative profile. A diagnosis of autoerythrocyte sensitization was made on the basis of the clinical history, dermatological examination complemented by a positive autoerythrocyte sensitization test, psychiatric evaluation and absence of any organic cause for her ailment. She was placed on psychiatric management and has remained symptom-free after six months follow-up. The case is reported for its rarity, as well as for the association of autoerythrocyte sensitization syndrome with frank dermatitis artefacta and pseudo-ainhum, which to the best of our knowledge has not yet been reported in the literature.

Macular degeneration in a case of Reiter's disease.

Posterior uveitis is not a documented feature of Reiter's disease. We describe here a patient of Reiter's disease, who after a bout of dysentery in January 2002 developed macular edema of the right eye with visual acuity of 6/36. In December 2002, she was found to have phlyctenular conjunctivitis. In June 2004, when she reported with scaly plaques over both palms, repeat ophthalmological examination revealed macular degeneration of the right eye. The simultaneous occurrence of Reiter's disease and macular degeneration in this patient may be fortuitous but the immunological basis of both diseases suggests a possible association.

Systemic sclerosis, localized morphea, en coup de sabre and aortic regurgitation: a rare association.

A 24-year-old male presented with Raynaud's phenomenon, digital infarcts, salt and pepper pigmentation and characteristic facies. There was gastrointestinal involvement clinically, endoscopically as well radiologically. In view of these findings and the demonstration of anti-nuclear antibodies with a homogeneous pattern of immunofluorescence and ScL-70 antibodies, he was diagnosed as a case of diffuse cutaneous systemic sclerosis. In addition, the patient had plaques of localized morphea, en coup de sabre with facial hemiatrophy on the left side, and mild aortic regurgitation (detected on echocardiography). The occurrence of these rare associations of localized plaques of morphea, en coup de sabre and aortic regurgitation in a single case of systemic sclerosis is quite an exceptional and interesting occurrence.

Segmental anhidrosis with hyporeflexia associated with congenital spinal deformity: a Ross's syndrome variant or inverse Horner's syndrome?

A 39-year-old soldier presented with anhidrosis affecting both upper extremities below the shoulders, the right side of the trunk below the third rib in front and the third vertebra on the back, and the left lower extremity below the inguinal ligament since 1992. Ten years later in 2002, he was also found to have bilateral absence of Achilles reflex and decreased right knee jerk. In addition, the patient was found to have congenital spinal abnormalities in the form of block of vertebrae C3-C4; decreased disc space C4-C5; and break in pars interarticularis L5-S1 with decreased disc space. A total of seven cases of Ross syndrome, Holmes-Adie syndrome (tonic pupil with lost tendon jerks) with segmental anhidrosis, have been described in the literature. Our case, however, did not have any pupillary abnormality. A case of progressive isolated segmental anhidrosis has also been described. The association of congenital spinal abnormality, which may be pathognomonic in the causation of this progressive sudomotor degeneration, is quite interesting in our case. The distribution of anhidrosis on the right side is just below the level of sweating loss sometimes described in lesions of superior sympathetic cervical ganglion in Horner's syndrome.

Chlamydia trachomatis seropositivity during pregnancy.

BACKGROUND: Chlamydia trachomatis infection in pregnant women can lead to serious maternal/neonatal antenatal or postnatal complications. The aim of this study was to find out the quantum of this problem in antenatal cases of Command Hospital, Kolkata. METHODS: Antibodies to C. trachomatis by ELISA method were tested in sera of 24 pregnant women and 10 nonpregnant controls. RESULTS: Five (20.83%) and 3 (12.5%) were positive and borderline positive for IgM and 1 (4.17%) and 2 (8.33%) were positive and borderline positive respectively for IgG antibodies i.e. an overall positivity of 45.83%. Only 1 (10%) of the controls was positive for IgA antibodies. CONCLUSIONS: This study highlights the importance of performing this simple blood test for detecting C. trachomatis infection in antenatal cases, to treat the infected and prevent the complications.

Ichthyosiform sarcoidosis following chemotherapy of Hodgkin's disease.

Acquired ichthyosis in adults may reflect internal disease, viz. internal malignancy, especially Hodgkin's lymphoma or sarcoidosis. We report a patient who developed ichthyosiform sarcoidosis over a year after completion of 6 cycles of combination chemotherapy for Hodgkin's disease. To our knowledge, the development of ichthyosiform sarcoidosis after chemotherapy for Hodgkin's disease has not been described earlier.

Ladakhi koilonychia.

A total of 176 highlander Ladakhis staying at an altitude of 3445 meters were examined for nail changes. Mean age of the subjects was 22.28 years (range 3-58 years). Koilonychia was seen in 47.16% of the subjects. It was most common during fourth (80.56%) and fifth (80%) decade. Males (49.60%) were slightly more commonly affected than females (41.34%). Soldiers (69.57%) were most commonly affected. Peasant and labourers (64.26%) were also equally affected. Most of the soldiers were also involved in forming during their leave period. Recruits (39.29%) and students (30.30%) were less commonly affected. Right index finger (36.36%), right middle finger (30.68%) and right thumb (29.55%) finger nails were most commonly affected followed by left thumb (13.64%), left index finger (10.23%), right ring finger (8.52%) and left middle finger nails (7.95%). Mean haemoglobin levels in those with or without koilonychia were 14.17 and 14.12 gm % respectively. Chronic hypoxia of high of high altitude causing increased erythropoiesis and depletion of iron stores leads to thinning of nail plate and atrophy of the distal nail bed with superadded mechanical trauma of farming or hard labour is the most likely cause of Ladakhi koilonychia. Dietary iron supplementation as a public health programme should be started in Ladakh to meet the demands of increased erythropoiesis in chronic hypoxic conditions.

High altitude and nail growth.

Linear nail growth studies were carried out in 22 highlanders Ladakhi (3445 m) and 6 lowlander male troops, in 4 during their fresh induction into high altitude (3445 m) and in 2 during their stay in plains while on leave from high altitude. The average age of highlander Ladakhis was 21.82 years (range 16-36 years) and lowlanders was 34 years (range 29-40 years). There was significant decrease in nail growth in age matched (average age 34.25) highlander Ladakhis (93.11/day, SD 7.24, P < 0.05) and in freshly inducted lowlanders (88.71/day, SD 10.7 mu, P < 0.05) in contrast to average nail growth in plains (1191/day, SD 1.41) Although the average nail growth in highlander Ladakhis (99.34/day, SD 13.91) was more than the lowlanders inducted into high altitude (88.7 s/day, SD 10.7 mu) the difference was not found statistically significant (P > 0.05). However it does suggest some degree of acclimatization in highlanders. Hypoxic conditions and extreme cold conditions both appear to be factors responsible for decreased nail growth in high altitude areas.

Patch test with ether extracts in salicaceae allergy.

A total of 23 cases suggestive of airborne contact dermatitis were patch tested with ether extracts of flowers and leaves of populus sp. and salix sp. in a study conducted in Ladakh at an altitude of 3445 meters above sea level. Overall positivity was found in 12 (52.17%), with populus sp. alone in 7 (30.43%), salix sp. alone in 4 (17.39%) and to both in one (8.33%).

Chronic actinic dermopathy--a clinical study in Ladakh.

A total of 176 highlander Ladakhis staying at Leh (Ladakh) at an altitude of 3445 meters were examined for skin changes on the exposed parts of the body. 111 (63.07%) had pigmentation over forehead, cheeks, nose or chin, 53 (30.11%) had telangiectasia over nose, cheeks or ear lobules, 21 (11.93%) had thickening and furrowing over forehead or lateral aspect of the eyes and only 5 (2.82%) had solar keratosis. Pigmentation and telangiectasia though seen in the first decade of life with prevalence rate seen as 48% and 24% respectively, the maximum prevalence has been seen in the second and third decade (79.22% and 25.97% and 62.96% and 48.15% respectively). Thickening and furrowing is seen most commonly in the fifth and sixth decade, which also leads to decreased prevalence of pigmentation and telangiectasia. Telangiectasia as a skin change to prolonged exposure to short wave length UV radiation has not been described by Eguren in 1972. Dilatation of the blood vessels in the dermis described by him correlates well with our finding of telangiectasia. Thus skin changes of pigmentation, telangiectasia, thickening and furrowing of the sun exposed skin and histopathological changes of Egurer s HA Dermopathy should be included in the syndrome of chronic actinic dermopathy.