Porokeratosis confined to the genital area: a report of three cases.

Genital involvement in porokeratosis (PK) is a rare occurrence even in disseminated forms. We encountered three patients who had porokeratosis affecting only the genital area. Two of them were male with involvement of the penis and scrotum and only the scrotum respectively. The lady with vulvar involvement is a hitherto unreported instance of porokeratosis confined to female genitalia. None of the cases were very easy to diagnose clinically but biopsies proved confirmatory. The male patients were advised light electrocautery under local anesthesia while the female patient underwent surgical excision.No malignant change has been reported in them till date.

Multicentric squamous cell carcinoma over lesions of porokeratosis palmaris et plantaris disseminata and giant porokeratosis.

Porokeratosis is a specific disorder of keratinization that has five clinical types and shows a characteristic 'cornoid lamella' on histopathology. Malignant degeneration has been described in all forms of porokeratosis. To the best of our knowledge, this is the first Indian report of multicentric squamous cell carcinoma complicating porokeratosis.

Alagille syndrome with prominent skin manifestations.

Alagille syndrome, a rare genetic disorder with autosomal dominant transmission, manifests 5 major features: paucity of interlobular bile ducts, characteristic facies, posterior embryotoxon, vertebral defects and peripheral pulmonic stenosis. We report a 6-year-old male child who presented with a history of progressive jaundice since infancy, generalized pruritus and widespread cutaneous xanthomata. He was also found to have obstructive jaundice, pulmonary stenosis with ventricular septal defect and paucity of bile ducts in liver biopsy. Histopathology confirmed skin lesions as xanthomata. The child was diagnosed as a case of Alagille syndrome. This particular syndrome with prominent cutaneous manifestations has been rarely reported in the Indian literature.

Epidermolysis bullosa pruriginosa--report of three cases.

Epidermolysis bullosa pruriginosa, a genetic mechanobullous disease, is characterized by pruritus, lichenified or nodular prurigo-like lesions, occasional trauma-induced blistering, excoriations, milia, nail dystrophy and albopapuloid lesions, appearing at birth or later. Scarring and prurigo are most prominent on the shins. Treatment is unsatisfactory. We report three such cases: two of them first cousins, are described with history of blisters since childhood, followed by intensely pruritic lesions predominantly on the shins, and dystrophy of toenails, but no albopapuloid lesions or milia. Intact blisters were present in one case, and excoriations were seen in the other two. All of them showed encouraging response to cryotherapy.

Pigmented xerodermoid--report of three cases.

Pigmented xerodermoid, a rare genodermatosis, presents with clinical features and pathology similar to xeroderma pigmentosum, but at a later age. DNA repair replication is normal, but there is total depression of DNA synthesis after exposure to UV radiation. Two siblings in their teens and a man in his thirties with features of pigmented xerodermoid, e.g. photophobia, freckle-like lesions, keratoses, dryness of skin, and hypo- and hyper-pigmentation, are described. Although classically the onset of pigmented xerodermoid is said to be delayed till third to fourth decade of life, it seems the disease may appear earlier in the tropics. Early diagnosis and management could be life-saving.

Mycosis fungoides with unusual vitiligo-like presentation.

Mycosis fungoides (MF), the commonest variant of primary cutaneous T cell lymphoma (CTCL), is relatively uncommon among the Asians. Hypopigmented mycosis fungoides is a rare variant usually observed in dark-skinned individuals, especially children. Hypopigmented MF usually responds well to therapy, particularly to PUVA, and has a comparatively benign course. Mycosis fungoides in a 16-year-old boy, with extensive asymptomatic hypopigmented lesions developing gradually all over the body over eight years and vitiligo-like skin lesions developing for seven years, with no systemic features, is presented for its unusual clinical features and conspicuous histopathological findings of prominent epidermotropism. The case showed fairly good response to PUVASOL therapy.

Erythrokeratoderma variabilis.

A case of erythrokeratoderma variabilis with classical clinical and histological features is being presented here.