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Biomedical subjects

M Ramam

Publications and source records attributed to M Ramam.

At least 19 recordsLinked to original sources

Griscelli syndrome.

A 4-month-old child had silvery gray hair, light-colored skin, recurrent chest infections, hepatosplenomegaly, and episodes of pancytopenia and hemophagocytosis in the liver, spleen, and bone marrow. Light microscopy of hair showed characteristic large aggregates of pigment granules distributed irregularly along the hair shaft. Peripheral blood smear examination did not show giant granules in granulocytes. Enlarged hyperpigmented basal melanocytes with sparsely pigmented adjacent keratinocytes were seen on the skin biopsy specimen. On the basis of these clinical and laboratory findings, Griscelli syndrome was diagnosed. The child succumbed to infection during an accelerated phase of the disease.

Bone Marrow↗

Cyclophosphamide pulses with oral prednisolone in the treatment of pemphigus: a pilot study.

An open labeled clinical trial aimed at assessing the efficacy and safety of pulse intravenous cyclophosphamide with daily oral prednisolone in the treatment of pemphigus was carried out. Twenty-six patients (12 men, 14 women; mean age, 48.4 years), comprising 25 cases with pemphigus vulgaris and 1 with pemphigus vegetans (< 10% body surface area involvement) who did not achieve adequate control on corticosteroids with or without other adjuvants were included. After baseline evaluation, monthly intravenous boluses of cyclophosphamide (15 mg/kg) along with daily oral prednisolone (starting dose 1 mg/kg/day, tapered according to clinical response) were administered. Patients were assessed monthly for clinical activity and side-effects. All patients experienced significant clinical improvement within 1 month of starting treatment. Healing of skin and mucosal lesions occurred respectively at mean durations of 2.1 and 3.6 months. Three weeks to 8 months later, 9 patients had recurrences of activity on tapering/withdrawal of prednisolone, mainly in the oral mucosa. Side effects of treatment included amenorrhea (3 patients), microscopic hematuria (3) which cleared with co-administration of mesna, vomiting (1), weight gain (10), gastritis (1), and cataract (2). It is concluded that treatment with monthly intravenous cyclophosphamide boluses along with daily oral prednisolone clears lesions of pemphigus with < 10 percent body surface involvement, and this may be an alternative regimen for pemphigus. Monitoring for adverse effects is essential.

Administration, Oral↗

A randomized placebo-controlled double-blind study of levamisole in the treatment of limited and slowly spreading vitiligo.

BACKGROUND: A previous uncontrolled, open trial of levamisole in patients with limited and slowly spreading vitiligo had shown that new lesions did not develop in 94% of patients after 2-4 months of treatment with the drug. OBJECTIVES: To assess the efficacy of levamisole in the treatment of slowly spreading, limited vitiligo. METHODS: In a randomized double-blind trial at the Department of Dermatology and Venereology, All India Institute of Medical Sciences, New Delhi, India, 60 patients with vitiligo involving < 2% of the body surface area and with slowly spreading disease (defined as one to five new lesions in the previous month or six to 15 new lesions in the previous 3 months) were randomly allocated to receive oral levamisole 150 mg or placebo on two consecutive days in a week. Children received oral levamisole 100 mg. All patients applied mometasone furoate 0.1% cream on the depigmented macules once daily. Patients were evaluated monthly for 6 months. The main outcome measure was the occurrence of new lesions, counted at each monthly visit. The secondary outcome measures comprised: (i) a dermatology-specific instrument, the Dermatology Life Quality Index or Children's Dermatology Life Quality Index questionnaires, which were completed by the patients at baseline and at every visit, and (ii) a general health questionnaire, the World Health Organization Quality of Life Brief Questionnaire, which was completed at baseline and at the end of the study. RESULTS: Forty-three patients completed 6 months of follow-up. The mean +/- SD number of new lesions that developed during the study period of 6 months was 1.9 +/- 2.0 (range 0-8) in the levamisole group and 1.8 +/- 2.0 (range 0-7) in the placebo group (P = 0.92). The proportion of patients who did not develop any further new lesions for the remainder of the study period was higher in the levamisole group at all the monthly evaluation points, although it was statistically significant (P = 0.05) only at the fourth month. Improvement in quality of life was similar in both groups. CONCLUSIONS: The study indicates that levamisole is not as effective in arresting disease progression as was observed in a previous open study. A study with a larger sample size is necessary to determine if levamisole is truly superior to placebo in this respect.

Adult↗

Diagnostic clinical features of pentazocine-induced ulcers.

BACKGROUND: Pentazocine was introduced in 1967 as a "non-narcotic, nonaddicting" analgesic. However, the abuse potential of this medication was soon recognized, and cutaneous and muscular complications of pentazocine abuse have been reported. METHODS: Demographic and clinical data on 10 patients with pentazocine-induced ulcers attending the Dermatology Outpatient Department of the All India Institute of Medical Sciences (AIIMS), New Delhi, India between November 2000 and October 2002 have been compiled. RESULTS: Ten patients with pentazocine-induced ulcers were seen at AIIMS between November 2000 and October 2002, six of whom were female. The average age of these patients was 32 years. The duration of the complaints ranged from 10 days to 7 years (average 17.5 months). Nine of the 10 patients had past history of painful medical conditions for which they had received pentazocine injections. All the patients presented with deep ulcers and sinuses over the accessible sites. The margins of these ulcers were hyperpigmented and indurated. Six patients had scars along the superficial vein access sites. Three patients had puffy-hand syndrome, while two had muscle contractures. No underlying psychiatric disorders were found in any of these patients. Urine screening for pentazocine was positive in two patients. Antinuclear antibody (ANA), antineutrophil cytoplasmic antibody (ANCA) and antibody against DNA (antidsDNA) tests and screening for infections such as human immunodeficiency virus (HIV), hepatitis B virus (HBV) and hepatitis C virus (HCV) were negative in all patients. CONCLUSIONS: Pentazocine abuse can be suspected from cutaneous findings, even when the patient does not volunteer a history of self-medication. Recognition of the condition will prevent misdirected investigations and treatment. The patient should be encouraged to seek treatment for drug dependence.

Adult↗

How soon does cutaneous tuberculosis respond to treatment? Implications for a therapeutic test of diagnosis.

BACKGROUND: It is difficult to demonstrate Mycobacterium tuberculosis in smears or biopsies and to grow it in culture in cutaneous tuberculosis because most cases are paucibacillary. A therapeutic trial of antitubercular drugs is frequently used to confirm the diagnosis in difficult cases. Information is lacking on the response to antitubercular therapy in cutaneous tuberculosis; consequently there are no clear guidelines on when to expect a response and also when to abandon a therapeutic trial. METHODS: We studied the records of 60 patients treated for cutaneous tuberculosis at our hospital to study the time course of the therapeutic response. All patients were treated with a short-course antitubercular regimen consisting of isoniazid 300 mg daily, rifampicin 450 mg daily, ethambutol 800 mg daily and pyrazinamide 1500 mg daily for 2 months followed by isoniazid and rifampicin in the same doses for 4 months. At follow-up visits, each patient was assessed by a dermatologist who recorded the presence or absence of clinical improvement in the skin lesions. RESULTS: Of the 60 patients seen, eight patients did not follow up after the initial consultation, 48 patients improved with treatment and four patients were classified as treatment failures. The timing of the first visit varied from 3 days to 15 months (median 27.5 days, mean 58.96 +/- 94.50) after initiation of treatment. Twenty-one patients were recorded to have improved within the first month of therapy. Twenty-seven patients who first reported more than 30 days after initiation of treatment were found to have improved. Four patients failed to respond during follow up ranging from 3 to 17 months. CONCLUSION: When a therapeutic trial is undertaken in cutaneous tuberculosis, 6 weeks of therapy with four drugs appears adequate to prove (or disprove) the diagnosis.

Adolescent↗

Ichthyosis follicularis, alopecia and photophobia (IFAP) syndrome treated with acitretin.

We describe a 3-year-old male patient with the ichthyosis follicularis, alopecia and photophobia (IFAP) syndrome, who developed cutaneous and ocular involvement in infancy. In addition, he had growth retardation and borderline intelligence; no other systemic involvement was found on detailed investigation. A moderate response to acitretin therapy (1 mg/kg) administered for 6 months was observed, with improvement in cutaneous features and corneal erosions and no change in alopecia or photophobia.

Abnormalities, Multiple↗

Strawberry gingiva: a distinctive sign in Wegener's granulomatosis.

A 37-year-old man presented with gingival hyperplasia accompanied by an ulcer on the eyelid, nasal obstruction, ear discharge, and discharging nodules on the cheek and back. An evaluation for infectious diseases and leukemia was negative. During his hospital stay, he had fever and migratory joint pains develop, suggesting a diagnosis of Wegener's granulomatosis, which was confirmed by a strongly positive cytoplasmic antineutrophil cytoplasmic antibody test. He responded promptly to treatment with oral prednisolone and cyclophosphamide. In retrospect, the diagnosis could have been suspected earlier because of the distinctive red, friable, and granular appearance of the gingiva.

Adult↗

Phaeohyphomycosis caused by Exophiala spinifera in India.

The second case of phaeohyphomycosis due to Exophiala spinifera in India has been diagnosed 46 years after the initial case. The present case involved a 12-year-old female patient with no known immunocompromising conditions. She presented with multiple verrucous, well-defined plaques encompassing phaeohyphomycotic lesions of varying sizes on her face, chest, arms and thighs. Lymph node involvement in dissemination was confirmed by demonstrating pigmented fungal elements in histopathology of the left axillary node. The infection responded positively to prolonged administration of itraconazole. The original case involved a young boy and was similarly disseminated but was more severe, with bone involvement, and had a fatal outcome. It is likely that other such cases have occurred in the intervening time but have not been reported.

Antifungal Agents↗

Morphometric study of lymphoid cells in mycosis fungoides and its simulators.

The distinction of early stages of mycosis fungoides from benign lymphoid disorders of skin is difficult by conventional histological techniques. We studied 10 cases of mycosis fungoides, 10 cases of large plaque parapsoriasis, 10 cases of other benign lymphoid disorders of skin and 5 cases of lymph nodes. Nuclear area, perimeter of the nucleus, nuclear contour index, cytoplasmic area, form factor and nuclear cytoplasmic ratio as well as DNA-ploidy were determined by image analysis. There were statistically significant difference (P value < 0.05) between all parameters except nuclear cytoplasmic ratio of the lymphoid cells of Mycosis Fungoides and benign lymphoid disorders of skin. Aneuploidy was found in 50% cases of Mycosis Fungoides. Histopathological parameters like epidermotropism pautrier micro-abscess and atypical lymphocytic infiltrate in both epidermis and dermis were more marked in aneuploid than diploid cases. So, the determination of nuclear contour index and DNA-ploidy is of importance to differentiate between Mycosis Fungoides and benign lymphoid disorders of skin.

Adult↗

Parthenium dermatitis presenting as photosensitive lichenoid eruption. A new clinical variant.

Parthenium hysterophorus is the commonest cause of airborne contact dermatitis (ABCD) in India. The disease usually manifests as itchy erythematous, papular, papulovesicular and plaque lesions on exposed areas of the body. Rarely, however, the disease may present as actinic reticuloid or photocontact dermatitis. We have observed a different clinical variant of this disease where certain patients with Parthenium dermatitis have presented with discrete, flat, violaceous papules and plaques on exposed areas of the body closely simulating photosensitive lichenoid eruption. We had 8 patients, 6 males and 2 females between 30 and 62 years of age, with itchy, violaceous, papules and plaques on the face, neck, ears, upper chest and dorsa of the hands for 6 months to 6.5 years. Four of these patients had a history of improvement of the lesions up to 30% in winter and aggravation of lesions on exposure to sunlight. There was no personal or family history of atopy. Cutaneous examination in all patients revealed multiple flat, violaceous, mildly erythematous papules and plaques on the forehead, sides and nape of neck, ears, 'V' area of the chest, and extensor aspects of the forearms and hands. Skin biopsies from these lesions showed features of chronic non-specific dermatitis. Patch testing with standardized plant antigens showed a positive patch test reaction to Parthenium hysterophorus in all patients, with a titre of contact hypersensitivity (TCH) varying from undiluted to 1 : 100. We conclude that Parthenium dermatitis may occasionally present with lesions very similar to the lesions of photosensitive lichenoid eruption in morphology and distribution. This clinical presentation of Parthenium dermatitis needs to be recognized to avoid misdiagnosis.

Adult↗

Panniculitides--a clinicopathologic study.

OBJECTIVES: To describe the clinical and pathologic features of panniculitides, an uncommon group of diseases, where the available Indian literature is sparse. MATERIAL AND METHODS: Prospective four years study of patients presenting with panniculitis to a large teaching hospital in North India. RESULTS: Erythema nodosum (EN) was the commonest panniculitis (79.3% cases) in our cohort. Other causes included erythema induratum, Weber Christian disease, cutaneous polyarteritis nodosa and panniculitis associated with Takayasu's arteritis and dermatomyositis-lupus overlap. Elevated ESR and leukocytosis were the common laboratory abnormalities encountered. Septal panniculitis without vasculitis was the most frequent histopathologic pattern observed. Patients of EN did well on nonsteroidal anti-inflammatory drugs while corticosteroids with or without azathioprine were used to treat other panniculitides. CONCLUSIONS: Physician awareness, clinical pattern recognition and histopathological confirmation, all play a key role in the identification and management of panniculitis.

Adipose Tissue↗

Pituitary-adrenal function following dexamethasone-cyclophosphamide pulse therapy for pemphigus.

BACKGROUND: Systemic corticosteroid therapy is known to lead to pituitary-adrenal (PA) suppression. Although patients treated for pemphigus with dexamethasone-cyclophosphamide pulse (DCP) therapy have shown no evidence of PA suppression, no study has been conducted to study this possible side-effect of DCP therapy. OBJECTIVES: To study the effect of DCP therapy for pemphigus on PA function. METHODS: A cross-sectional study was carried out in 33 patients who completed phase II of DCP therapy in a single centre, a large teaching hospital in New Delhi, India. Serum cortisol levels following adrenocorticotrophic hormone stimulation were analysed in 31 patients. RESULTS: Seventeen (55%) patients showed suppressed PA function. Suppressed function occurred more frequently in patients who had received pulses of dexamethasone in addition to DCP therapy in the first phase of treatment (P = 0.055). CONCLUSIONS: Suppressed PA function occurs in about half of patients who receive DCP therapy for pemphigus. These patients probably do not require routine replacement therapy with corticosteroids but may need supplementation during periods of stress.

Adolescent↗