Infantile tremor syndrome (ITS) is defined by the tetrad of pallor, developmental delay/regression, skin pigmentation, and brown scanty scalp hair. Involuntary movements in the form of tremors supervene in the natural course of the illness in a significant number of cases. The disorder occurs in exclusively breast-fed infants of vegetarian mothers belonging to economically deprived sections of society. Most of the children eventually recover but are frequently left with long-term cognitive and language neurodeficits. ITS continues to be seen in clinical practice, but there has been no consensus on its causation. Last comprehensive review was published in 1987 describing the ITS as a syndrome of unknown or multiple etiologies. Several important papers have been published since then. This review attempts to provide comprehensive and up-to-date information on the subject incorporating recently published studies. In the end, the issue of etiology is objectively re-examined in the light of available evidence some of which has been published in recent years.

Background: Corticosteroids are used as a daily oral therapy or in a pulse form with or without various adjuvant drugs for the treatment of pemphigus. Our long-term experience with the use of dexamethasone-cyclophosphamide pulse therapy as a first-line treatment modality for pemphigus is presented. Methods: A retrospective analysis of records of pemphigus patients treated by dexamethasone-cyclophosphamide therapy was carried out. The treatment regimen consisted of the monthly administration of intravenous dexamethasone (136 mg) for 3 consecutive days with addition of intravenous cyclophosphamide (500 mg) on the second day. Oral cyclophosphamide (50 mg) daily and oral corticosteroids (low tapering doses) were given in the intervals between the pulses, till partial remission was achieved. Pulse therapy was then continued for another 6 months followed by daily oral cyclophosphamide (50 mg) for 1 year, which produced a complete remission. Results: A total of 36 patients, 32 with pemphigus vulgaris and 4 with pemphigus foliaceus, were treated with this regimen. Two to 8 pulses were required to achieve a partial remission, while the total number of pulses given for complete remission ranged from 8 to 32. The duration of pulse therapy correlated with both the disease severity and the time to achieve remission. All patients are now in complete remission with a follow-up of 0.5–12 years. Four patterns of remission were observed, related to the severity of disease. The response to pulse therapy was faster in the milder form of disease. The severe form of disease required more pulses as well as higher doses of intervening oral corticosteroids. Conclusions: Dexamethasone-cyclophosphamide pulse therapy is an effective form of treatment in pemphigus and results in long-lasting remissions.

We studied 27 infants aged 6 to 27 months with vitamin B12 deficiency also known as "infantile tremor syndrome" in India. All were exclusively breast-fed by vegetarian mothers. Developmental delay or regression, pallor, skin hyperpigmentation, and sparse brown hair were present in all. Majority were hypotonic and involuntary movements were encountered in 18. Anemia and macrocytosis was found in 83% and 71% infants, respectively. Low serum vitamin B12 was present in 12 of 21 infants. Seven of the 9 infants with normal serum vitamin B12 had received vitamin B12 before referral. Twelve mothers had low serum vitamin B12. Cerebral atrophy was present in all the 9 infants who underwent neuroimaging. Treatment with vitamin B12 resulted in dramatic improvement in general activity and appetite within 48 to 72 hours followed by return of lost milestones. Tremors resolved in all by 3 to 4 weeks. Nutritional vitamin B12 deficiency is a treatable cause of neurologic dysfunction in infants.

Anticonvulsant hypersensitivity syndrome is an acute, life-threatening, idiosyncratic drug reaction seen with the aromatic antiepileptic drugs, phenytoin, carbamazepine, phenobarbital, and primidone, with frequent cross sensitivity. It usually occurs 2-8 weeks after initiation of therapy and the hallmark clinical features are fever, rash, and lymphadenopathy. Hematologic abnormalities such as eosinophilia, atypical lymphocytes, and internal organ involvement also occur with varying severity. A case of hypersensitivity syndrome due to carbamazepine with cross sensitivity to phenytoin is reported. It is emphasized that this serious drug reaction with diverse clinical presentations should be recognized and treated promptly.

Lichen sclerosus is a depigmenting mucocutaneous disorder that most frequently affects the female genitalia. Lichen sclerosus affecting the oral mucosa is extremely rare. Oral lesions are asymptomatic but cosmetically unacceptable. We report here a case of lichen sclerosus presenting with a linear lesion over the nose that extended to involve the philtrum and the upper lip with intraoral extension up to the gingiva. Treatment with a short course of oral and intralesional corticosteroids resulted in partial resolution of the lesions.