Malignant blue nevus.

Blue nevus represents an aberrant collection of functioning benign dermal melanocytes. Its malignant degeneration is rare and is regarded as a form of malignant melanoma. We report a case of 35-year old male with this rare condition whose primary lesion over left foot ulcerated and patient later succumbed to multiple metastases.

Darier's disease with warty dyskeratoma and basal cell epithelioma.

A 48-year old man with warty dirty papules over the seborrhoeic areas, also had a nodule with a central keratotic crater over the right cheek. Biopsy of this nodule revealed features of Darier's disease, warty dyskeratoma and basal cell epithelioma. Even though Darier's disease and warty dyskeratoma are considered as a distinct entities, the presence in the same lesion emphasizes the need for further studies on this association.

Mutilating acral keratoderma.

A 15-year-old male, born of non-consanguineous marriage started developing thickening of skin of sole of the both feet at the age of 7 which progressed to involve whole of both feet by 11th year. Patient later developed painful autoamputation of little toes of both feet due to pseudoainhum. Involvement of the palms was limited to a localised area over palmar aspect of right middle finger and was non-progressive. No other family member was affected. This may represent a variant of Vohwinkel's syndrome or the rarer form of acral keratoderma.

Tuberous sclerosis.

Although tuberous sclerosis has been described with a diagnostic triad, it is not present consistently in all cases. Variety of skin manifestations were reported in tuberous sclerosis. This studay was undertaken to assess the frequency of various skin changes in tuberous sclerosis. Ten consecutive cases of tuberous sclerosis were studied. Angiofibroma was the commonest cutaneous manifestation. Atypical fibroxanthoma, dermatofibroma and neurofibroma were also noticed as interesting associations.

Aggressive infantile fibromatosis.

An 8-month-old infant presented with 2 ulcerated indurated plaques in the right lower limb. The lesions grew rapidly initially but for the last 2 months, they have been stationary. Biopsy showed proliferation of fusiform fibroblasts some of which were arranged in whorls. The clinical picture and the histopathology suggested the diagnosis of an aggressive infantile fibromatosis. This is one of the types of juvenile fibromatoses, a group of tumours characterised by fibrous tissue proliferation present at birth or acquired early in childhood.

Waardenburg's syndrome.

Three children in a family of five presented with heterochromia iridis, lateral displacement of inner canthi and varying degrees of sensorineural deafness. All the 3 showed iris atrophy. The father of the children had only heterochromia iridis. A diagnosis of Waardenburg's syndrome Type I was made in the children with the father probably representing a forme fruste of the condition.

Atypical gorlin's syndrome.

A 21-year-old woman presented with complaints of skin lesions on her face, palms and soles. On examination, 8 to 10 well-defined, pigmented nodules with raised and pearly borders were seen on the face. Multiple pits were present on the palms and soles. Biopsy of both lesions revealed the presence of basal cell epitheliomas. The patient also gave history of removal of a keratocyst of mandible 10 years back. The case was diagnosed as Gorlin's syndrome. It is atypical due to the low number of basal cell epitheliomas, the occurrence of only a single odontogenic cyst and the absence of other features usually associated with this condition.