Aetiopathological and clinical study of erythroderma.

Erythoderma first described by Hebra in 1868 is a reaction pattern, characterised by generalised and confluent erythema with desquamation affecting more than 90% of body surface and is usually accompanied by systemic manifestation including some metabolic changes. Tile study was envisaged to assess their profile of aetiology, clinical features and to correlate with histopathological findings. Twenty-four patients (18 males and 6 females) with erythroderma were considered for the study. All patients were hospitalised, detailed history was taken and were subjected to relevant investigations--haemogram, serum proteins, liver and renal function tests, routine and microscopic urine tests, HIV screening, x-ray chest, ultrasound abdomen, FNAC of enlarged lymph nodes and skin biopsy. The age group affected ranged between 7 and 70 years, peak incidence between 50 and 60 years. The male to female ratio was 3:1. Scaling, erythema and pruritus were predominant clinical manifestations. In 12 clinically suspected psoriatic erythroderma, only in 6 cases the histopathological features were correlated to psoriasis and remaining showed non-specific chronic dermatitis. Two patients were found to be HIV-positive. There is good clinicopathological correlation in erythroderma caused by lichen planus, atopic dermatitis, seborroeic dermatitis, non-bullous congenital ichthyosiform erythroderma. Drugs were found to be aetiological factor in 3 cases. The study was conducted to assess variations in clinical, aetiological and histopathological profile of erythroderma. The present series had high percentage of erythroderma secondary to preexisting dermatoses. The onset of disease was insidious except in drug-induced erythroderma where it was acute.

Forehead plaque: a cutaneous marker of CNS involvement in tuberous sclerosis.

BACKGROUND: Tuberous sclerosis complex (TSC) is a neurocutaneous genodermatosis characterized by hamartoma formation in multiple organs. There are no definite cutaneous markers suggestive of central nervous system (CNS) involvement in TSC. AIMS: To study association of forehead plaque seen in tuberous sclerosis patients and CNS involvement in TSC. METHODS: This is a retrospective study of 15 cases of tuberous sclerosis in varying age groups - from 1.5 to 50 years. All the cases were thoroughly evaluated with detailed history; clinical examination; and relevant investigations like X-rays of chest, skull, hands and feet; ultrasound abdomen and computed tomography of brain. RESULTS: Out of the 15 cases, CNS involvement was seen in 8 cases. Seizures were present in 8 cases (53.33%) and mental retardation was seen in 6 cases (40%). Computerized tomography of brain revealed subependymal nodules (SENs) in eight cases (53.33%). In addition to SENs, subependymal giant cell astrocytomas and cortical tubers were seen in 2 cases each. Out of these 8 cases having CNS involvement, in 7 cases forehead plaque was observed. In 1 case, no forehead plaque was observed (X 2 = 1.07, P < 0.05). CONCLUSION: This study shows that there is a statistically significant relationship between the presence of a forehead plaque and CNS involvement in TSC. Therefore, forehead plaque may be considered as a novel cutaneous marker to know the CNS involvement in TSC at an early stage.

Tuberous xanthomas in type IIA hyperlipoproteinemia.

A 4-year-old obese girl with multiple yellowish, plaques and nodular lesions showed features of tuberous xanthomato in Type IIa hyperlipoproteinemia.

Dyschromatosis Universalis.

Hyperpigmented macules all over the body intermignled with mottled depigmentation were observed in two brothers from Varanasi. Hyperpigmented macules were present since birth and increased ill number and size during childhood. Depigmentation developed at the age of eigh, years and increased during adolescence. This entity described as dyschromatosis universalis in the Japanese and the Europeans has not been described in the Indians so far.