Lymph node abscess and cardiac involvement in a patient with nodular lepromatous leprosy (LL) with erythema nodosum leprosum (ENL): a rare occurrence.

With the world's focus on reducing the leprosy patient load to the extent of elimination, finding and reporting the rarer presentations of leprosy becomes important for prompt treatment. Also, these untreated patients may serve as a potential source of infection in community. We report a 35-year old man diagnosed to have lepromatous leprosy and erythema nodosum leprosum with inguinal lymph node abscess and suspected cardiac involvement that proved fatal. We stress the importance of detailed workup to look for associated systemic involvement for timely intervention and favourable outcome.

Co-existence of extramammary Paget's disease and Bowen's disease of vulva.

Extramammary Paget's disease and Bowen's disease are histologically similar and immunohistochemistry is often required to make the diagnosis. We present a case of vulval Paget's disease with Bowen's disease in an elderly female. Strong positivity for cytokeratin 7, anti CAM 5.2, carcinoembryonic antigen (CEA) and periodic acid-Schiff (PAS) stain in clitoral, left labial and interface regions of the vulvectomy specimen confirmed the diagnosis of Paget's disease (PD) while positive staining for p63 in the right labial and interface regions helped in establishing the diagnosis of concurrent Bowen's disease (BD).

Familial atrophia maculosa varioliformis cutis: first case report from the Indian subcontinent with pedigree analysis.

Familial atrophia maculosa varioliformis cutis is a very rare disorder with less than 28 cases being reported in the literature worldwide and remains a mystery both as far as genetics and the virtue of its pathogenesis is concerned. We present a case of mother and son, both having this disorder with presentations unique in terms of sites involved and try to draw a five generations pedigree chart for the same. We further support its inheritance pattern as autosomal dominant. Also, we propose oral isotretinoin as an effective treatment modality for the same.

Phacomatosis cesioflammea: first case report from India.

Phacomatosis cesioflammea is a rare condition defined by the simultaneous presence of both vascular and pigmentary nevus in the same patient. We report a case of a 4-year-old Indian female child who presented with diffuse dermal melanosis on the upper shoulders, upper anterior chest and lower back and extending to involve both sides of the arms and forearms, generalized port-wine stain on the back, shoulders and both upper limbs with sparing of the right palm. At places, the two types of lesions were superimposed on each other and were also present discretely on the back, but in close proximity to each other. There was the presence of melanosis bulbi on the right side of the eye. She was otherwise normal. She was clinically diagnosed as a case of phacomatosis pigmentovascularis cesioflammea. The nonallelic twin spotting phenomenon has been proposed in the pathogenesis of this disorder.