Pramila Bajaj

Nail changes in Langerhans cell histiocytosis

Nail changes in Langerhans cell histiocytosis are distinctly uncommon. Paronychial erythema, swelling and subungual pustules of the fingernails and toenails were cardinal, and were supported by diffuse as well as dense collections of mononuclear Langerhans cells evidenced by microscopic investigation. Oral administration of co‐trimoxazole (800 mg sulphamethoxazole + 160 mg trimethoprim) every 12 h, 50 mg/d cyclophosphamide and 80 mg/d predinisolone were the mainstay of treatment, supported by scalp tar shampoo and local betamethasone lotion application.

Association of gastroesophageal reflux disease in young children with persistent respiratory symptoms.

Forty children aged between 3 months and 3 years (median age 14 months) with persistent respiratory symptoms beyond 4 weeks or recurrence of respiratory symptoms were investigated for gastroesophageal reflux (GER). Diagnostic tests included upper gastrointestinal endoscopy, oesophageal biopsy, gastroesophageal scintiscan and 24 h ambulatory oesophageal pH monitoring. GER was detected in 14 (35 per cent) of these patients; which included 38 per cent of the enrolled cases of recurrent bronchopneumonia, 40 per cent cases of reactive airway disease, and 22 per cent cases of persistent cough. Amongst the cases detected to have GER, the age of onset of respiratory symptoms was less than 1 year in 86 per cent of cases (p < 0.01), nocturnal symptoms of cough and wheeze were reported in 78 per cent (p < 0.05), and 86 per cent cases did not present with typical gastrointestinal symptoms (p < 0.01). Family history of asthma was absent in all cases of GER-related reactive airway disease (p < 0.01). Cases detected to have GER were followed for 3-6 months after starting anti-reflux therapy. A significant (p < 0.01) decrease was noticed in the number of further episodes in children with GER-related recurrent bronchopneumonia and reactive airway disease after starting anti-reflux therapy. Improvement was also noticed in nocturnal symptoms and nutritional status after anti-reflux therapy was started. Our results suggest that GER may be one of the possible contributing factors in any child with recurrent and persistent respiratory complaints. Early diagnosis and anti-reflux therapy in cases with GER-related respiratory complaints can result in significant improvement in symptoms.

Diagnostic Modalities for Gastroesophageal Reflux

Objective : To evaluate commonly utilized diagnostic modalities to detecting Gastroesophageal Reflux (GER).Methods : Sixty children aged 1–72 months (mean age 14.7 months) with symptoms suggestive of Gastroesosphageal Reflux (GER) were investigated and subjected to upper gastrointestinal endoscopy and esophageal biopsy (EB), gastroesophageal scintiscanning (GS) and 24 hour ambulatory pH monitoring.Result : GER was detected in 28 (46.7 %) cases by one or more diagnostic modalities. Ambulatory 24 hour pH monitoring was positive in higher proportion (43.3 %) of cases in comparison to other modalities, followed by EB (38.3%) and GS (30%). Considering 24 hour pH monitoring as the gold standard, esophageal biopsy was positive in 22/26 cases (84.6%) detected by 24 hour pH monitoring with a specificity of 97.1% as compared to 17/26 cases (65.4%) by gastroesophageal scintiscanning with a specificity of 97.1%. When compared with EB results, amongst various parameters measured during 24 hour pH monitoring, Reflux index (Rl) ranked highest (sensitivity 95.6 % and specificity 89.2 %) followed by duration of longest episode > 20 minutes and Euler Byrne score. Oscillatory index, calculated from tracings of pH monitoring, even though ranked lower because of its low sensitivity helped to pick up 2 cases missed by EB and Rl.Conclusion : Our results suggest that a combination of diagnostic modalities may be required to diagnose GER in young children. Ambulatory 24 hour pH monitoring appears to be the single best investigation and combining it with EB and/or GS can help to detect maximum number of cases.

Sebaceous Hyperplasia in Youngsters

To the Editor: A 23-year-old man had progressively noticed several yellowish asymptomatic eruptions varying from pin-head to pea size over the left side of his face, including the lower lid and the nasolabial fold, in a linear fashion for the past eight years. Examination of the skin surface revealed multiple papules 2-4 mm in diameter. A few of them had central depressions. They were distributed in a linear fashion from the left lower lid to the upper lid. A few lesions were also present on the forehead. The distribution of the lesions was striking (Fig. I). Mild electro-dessication was used to destroy the lesions. Similar lesions were later seen in a 24-year-old woman, on whom 0.05 per cent tretinoin cream was used with excellent results. Hematoxylin-eosin stained sections prepared from the typical papules revealed multiple greatly enlarged sebaceous glands Fig. 1. Yellowish, multiple, flat-topped papules of varying sizes, disposed in a linear fashion over only the left side of the face

Malakoplakia of bone: a case report

BACKGROUND Malakoplakia is an uncommon but distinctive granulomatous disease, characterized by an accumulation of histiocytes or Von Hansemann cells containing intracytoplasmic, laminated Michaelis-Gutmann bodies. CASE A 3-year-old male presented with a tender swelling in the left gluteal region that had been present for one month. Smears made from a fine needle aspirate showed large histiocytic cells containing intracytoplasmic, basophilic, laminated, targetoid Michaelis-Gutmann bodies resembling Von Hansemann cells in malakoplakia. Histopathology confirmed the diagnosis of malakoplakia of bone. CONCLUSION This case, histologically proven to be malakoplakia, demonstrated regression of the lesion following therapy. The characteristic cytologic features and presence of Von Hansemann cells may in themselves be diagnostic and obviate the need for biopsy.

Free oxygen radicals and immune profile in newborns with lung diseases

Respiratory difficulties constitute the commonest cause of morbidity in new born infants and pulmonary pathology is the most frequent autopsy finding. Various components, such as free oxygen radicals, immunoglobulins, and compliment systems, play an important role in immunopathology and immune defence. The aim of the present study was to assess free oxygen radical markers in neonatal lung diseases, studying their relationship with immune profile and ascertaining their relevance as predictors of clinical outcome. Full term newborns with respiratory distress and X-ray proven lung disease formed the study group. Blood samples were assessed for lipid peroxide (LPO), superoxide dismutase (SOD), glutathione peroxidase (GPx), immunoglobulins IgM and G, CD4 and CD8 subpopulations of T-lymphocytes. Levels of free radical markers were significantly higher in the study group. The humoral immune response was seen in terms of raised IgM levels in the study group which were still within the normal range. The cellular immune response was demonstrated by a raised percentage of CD4 T-lymphocytes which in turn accentuated the CD4:CD8 ratio. Higher levels of reactive oxygen species (ROS) were associated with a prolonged duration of respiratory distress and oxygen dependence. Since the free radicals have emerged as the major final common pathway of tissue injury, free radical ablation offers substantial potential for treatment; but whether antioxidants, scavengers and other modalities would have a significant impact on clinical outcome, remains to be investigated.

Pityriasis Rubra Pilaris (PRP): Report of Four Cases

Four patients with pityriasis rubra pilaris are reported. The diagnosis in each was based upon well‐recognized clinical features. Two of them, a mother and son, had the disease since childhood and were marked by relative remission in spring and exacerbation in autumn. Moderate to severe pruritus was a common dominator. Erythroderma was a presenting feature in one case. Although histopathology was considered imperative, it only supplemented the clinical expression. Vitamin A in heavy dosage, supplemented by vitamin E and stanozolol in tandem, was the mainstay of treatment.

Clinico-histopathological correlation of skin and nerve in leprosy.

The histopathological features of skin tissue sections in patients clinically diagnosed as leprosy were correlated with the histopathological features of nerve specimens obtained from the same patients. Fifty untreated leprosy patients attending the Outpatient Department of the Department of Dermatology and Sexually Transmitted Diseases of Smt. Sucheta Kriplani and Kalawati Saran Childrens Hospitals, New Delhi, India were included in the study. On correlating the histological features of skin and nerve tissue sections, concordant findings were found in 24 out of the 50 patients (48%) but discordance between the histopathological features of skin and nerve tissue sections were found in 26 out of 50 cases (52%). Of these 26 cases, the nerve tissue histology when compared with the skin histology showed features lower down the disease spectrum in 17 (34%) cases. Seven of the 50 patients (14%) showed histological features of leprosy higher in the disease spectrum in the nerve tissue sections than in the skin biopsy sections. One patient clinically LL leprosy demonstrated histopathological features of Histoid leprosy in the skin sections and LL in the nerve sections. The remaining one patient had features of TT leprosy in the skin tissue sections while the nerve tissue histopathology showed non‐specific changes. Histological features of the skin tissue sections were consistent with the clinical diagnosis in 33 out of 50 cases (66%). When the clinical groups were correlated with the histological features of the nerve tissue sections, concordance was found in 30 of the 50 cases (60%). On comparison of the histological features of skin and nerve tissue sections with the clinical diagnosis, concordance was still lower i.e., 19 out of 50 cases (38%). Thus the histological features of the skin tissue sections correlated more frequently with the clinical diagnosis than did those of the nerve sections. The importance of neural histology lies in the fact that it shows a higher BI and a lower histological grading in some cases and if not performed the lapse can result in inadequate treatment, drug resistance and even relapse.