Asok Gangopadhyay

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.

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.

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.

Dermatophyte infection of the male genitalia

Dermatophyte infection is a very common disease, but that of the male genitalia is said to be rare. Here we report four cases of dermatophyte infection of the male genitalia, of which one had lesion on the penis alone, one had lesions on the penis and the groins, one had lesions on penis and scrotum, and the other had involvement of scrotum, penis and groins. Two patients gave history of application of steroid-containing preparations and another had diabetes mellitus. Culture of the scraping of the lesional skin yielded Trichophyton rubrum in two cases and Epidermophyton floccosum in the other two. All cases resolved completely with topical terbinafine with or without oral antifungals.

Extragenital lichen sclerosus et atrophicus

Lichen sclerosus et atrophicus (LSA) is a chronic inflammatory dermatosis with anogenital and extragenital presentations. Extragenital lichen sclerosus is most common on the neck, shoulders and upper trunk. Linear lesions are uncommon in LSA. We report a case of linear extragenital LSA involving forehead and scalp, along with grouped white papules of LSA in the right side of the back in a postmenopausal woman. The patient showed atypical clinical presentation of LSA in face which clinically mimicked ‘en coup de sabre’ as seen in morphea, but other clinical features suggested the diagnosis of LSA and the histopathological findings confirmed it.

Naevoid Psoriasis and ILVEN: Same Coin, Two Faces?

The true existence of naevoid psoriasis and inflammatory linear verrucous naevus as distinct entities has been a debatable issue. Each has been opined to be a variant of the other. Considerable clinical and histological resemblance is seen between the two conditions. We describe three cases which attempt to throw more light on this issue.

Squamous cell carcinoma in a patient with vitiligo of photo-covered skin

Ultraviolet radiation is an important risk factor for the development of skin cancers. Melanin is known to protect the skin against harmful effects of ultraviolet radiation. Hence, vitiligo patients are at higher risk of developing such malignancies. But few reports of occurrence of skin cancers in vitiligo lesions suggest that their occurrence in vitiligo is rare. There are reports of development of Squamous cell carcinoma (SCC) in vitiligo lesions, following long-term Psoralen and Ultraviolet-A (PUVA) therapy. But there are no reports of occurrence of SCC in a vitiligo patch located in photo-covered area. We describe a patient with SCC in a vitiligo patch present in photo-covered skin.

Cutis laxa: A report of two interesting cases

Cutis laxa is a rare disease that may be either inherited or acquired. The acquired form is rarer than the inherited form. Pathogenesis of this disease is largely unknown. Two cases of acquired cutis laxa are reported here and neither of them had any systemic involvement or any history of drug intake. One of them had localized disease with history of preceding cutaneous inflammation. The other patient with generalized lesion lacked any history of preceding illness. The patient with localized lesion was treated satisfactorily by reconstructive surgery. The other patient had generalized involvement, for which no satisfactory treatment could be offered.

Hyperpigmented keratotic plaques below the elbow and above the knee: An unexplored entity?

Background: Hyperpigmented keratotic lesions below the elbow and above the knee are common, but not yet described in the literature. Aim: To clinicopathologically analyze the lesions to find out their etiology, aggravating factors, associated diseases and histopathological features. Materials and Methods: Thirty patients having such lesions were subjected to detailed history-taking and clinical examination. A biopsy from the local area was performed in all the cases. The data obtained was statistically analyzed. Results: The commonest age group affected comprised of middle-aged females. In 50% cases, friction played an important role, and in the rest, no cause was found. Majority of patients had no other dermatological or systemic disorders. Nonspecific histopathologic changes were observed in 60%. Conclusion: Our study points towards the existence of an idiopathic hyperpigmentation around the elbow and knee that is common and not associated with any other skin disorder and is histologically nonspecific.

Annualr elastolytic giant cell granuloma with penile involvement

Annular elastolytic giant cell granuloma is a recently described entity of unknown origin that presents as indolently growing papules and plaques over sun-exposed skin. Histology shows a granulomatous infiltrate with loss of elastin in the center of the lesion. We came across a patient who had penile lesions and a paucity of literature about the involvement of the external genitalia in this disorder.