Study of upper gastrointestinal tract involvement in pemphigus by esophago-gastro-duodenoscopy.

INTRODUCTION: Involvement of upper gastrointestinal tract in pemphigus vulgaris is not uncommon. AIM: To study the involvement of upper gastrointestinal tract (UGIT) with the help of esophago-gastro-duodenoscopy (EGD) in patients of vesiculobullous dermatoses with emphasis on pemphigus vulgaris. METHODS: Forty-two patients (M-22, F-20) with vesiculobullous dermatoses, diagnosed on the basis of clinical features and skin histopathology as pemphigus vulgaris (PV)-40 patients and pemphigus foliaceus (PF)-2 patients were included in the study. The EGD was performed and mucosa of the esophagus, stomach and first part of the duodenum were examined. Mucosal biopsies were taken from the lower esophagus in 26 patients of PV and studied after H and E staining. RESULTS: On EGD, esophageal involvement was seen in 67% patients of PV (27/40). Of these, Grade I esophagitis was observed in seven, Grade II in 11, Grade III in four and Grade IV involvement was seen in five patients of PV. Three PV patients had associated esophageal candidiasis. Involvement of esophageal mucosa was also observed in one out of two patients of PF. Gastric mucosa was involved in 52% and duodenal mucosa in 20% of PV patients. Acantholysis was observed in seven out of 26 (27%) esophageal biopsies of PV patients. Two patients of PV vomited a tube-like structure, indicative of 'esophagitis dissecans superficialis'. The involvement of the gastric mucosa in patients with history of oral corticosteroid intake (60%) was compared to the group without history of oral corticosteroids (30%). CONCLUSION: Among PV patients under study, significant involvement of oral (87%), esophageal (67%), gastric (52%) and duodenal mucosa (20%) was observed.

Comparison of two systems of classification of leprosy based on number of skin lesions and number of body areas involved--a clinicopathological concordance study.

BACKGROUND AND AIMS: WHO guidelines classify leprosy patients for therapeutic purposes into paucibacillary (PB) and multibacillary (MB) leprosy based on the number of skin lesions. An alternative system of classification has been in practice in Nepal from 1985 onwards, based on the number of body areas involved in patients of leprosy. We attempted a clinicopathological approach for comparison of these two systems of classification in leprosy patients for their ability to demarcate patients into groups of PB and MB leprosy. MATERIALS AND METHODS: The study included 108 leprosy patients (80 males and 28 females). Complete clinical examination and body charting was carried out in each patient noting the count of skin lesions and the number of body areas involved. Slit skin smears and skin biopsies were taken from an active skin lesion in all patients. RESULTS: On analysis, it was observed that there was good clinicopathological correlation between patients with 5 or < 5 skin lesions and 2 or < 2 body areas involved. (Clinical 95% and histological 96%) A similar correlation was also observed in the other group of patients with > 5 skin lesions and > 2 body areas involved, (Clinical 94% and histological 96%). There were almost identical numbers of patients represented in these two groups of classification. CONCLUSIONS: Our findings suggest that patients with involvement of 2 or less body areas can be classified as PB leprosy and those with more than 2 body areas involved can be classified as MB leprosy for the purposes of therapy. The study of areas of involvement in leprosy patients not only provides additional patient information but also adds another parameter as a basis for the study of leprosy patients.

Pulse therapy and its modifications in pemphigus: a six year study.

BACKGROUND: Dexamethasone-cyclophosphamide pulse (DCP) therapy for the pemphigus group of disorders is being widely used in India after its introduction in 1986. However, there are certain limitations to its application due to the serious side effects of cyclophosphamide. METHODS: Between 1995 and 2001, we treated 41 patients of pemphigus with modified pulse therapy. These modifications included certain changes in the DCP therapy protocol and substitution of cyclophosphamide with either azathioprine or methotrexate in a few patients. RESULTS: We observed a good response to pulse therapy in all 34 patients who took treatment regularly. CONCLUSIONS: We found the modifications to the original DCP therapy protocol to be very effective and useful.

Multicentric reticulohistiocytosis.

A 41 -year-old man presented with multiple, painful and tender nodules on the palms, soles, scalp; and on the limbs predominantly around the joints with associated arthropathies. Smaller nodules were seen on the ear helices. There was no other clinically evident or investigative abnormality. Histopathological study confirmed a diagnosis of multicentric reticulo histiocytosis.

Multi focal tuberculosis.

A 19-year-old salesman presented with multiple fusiform, fluctuant, non-tender swellings involving dorsum of the left hand, left index finger and little finger. He also had multiple sinuses with puckered scars on the right thumb, left little finger and right elbow. He was provisionally diagnosed as tuberculous gumma. X-ray chest showed apical cavity and infiltration suggestive of tuberculosis X-ray both hands showed osteolytic lesions with pathological fracture. AFB was cultured on Lowenstein Jensen medium and the patient was given antituberculosis treatment with clinical improvement.

Disseminated superficial porokeratosis.

A 65-year-old male diabetic presented with erythematous and hyperpigmented plaques with scaling and crusting involving the trunk and extensor surfaces of extremities of 10 years duration associated with mild itching. Cutaneous examination revealed large hyperpigmented and erythematous plaques with raised borders over the trunk and extremities. A small plaque on the (L) forearm showed a raised thread like margin with a furrow. A provisional diagnosis of disseminated superficial porokeratosis was made. Skin biopsy confirmed the diagnosis of disseminated superficial porokeratosis.

Multifocal tuberculosis.

A 19 -year-old salesman presented with multiple fusiform, fluctuant, nontender swellings involving dorsum of left hand, left index finger and little finger. He also had multiple sinuses with puckered scars on right thumb, left little finger, and right elbow. He was provisionally diagnosed as tuberculous gumma and was investigated further. X-ray chest showed apical cavity and infiltration suggestive of tuberculosis. X-ray both hands showed osteolytic lesion with pathological fracture. AFB was cultured on Lowenstien Jensen s medium and the patient was put on ATT with clinical improvement.

Primary pachydermoperiostosis associated with Menetrier's disease.

Primary pachydermoperiostosis and Menetrier's disease are both hypertrophic conditions of skin and mucous membrane respectively. Here we report a case of association of these two conditions in a 30 -year-old man.