Sujata Sengupta

Epidemiological and clinicopathological study of oral leukoplakia

BACKGROUND Oral white lesions that cannot be clinically or pathologically characterized by any specific disease are referred to as leukoplakia. Such lesions are well known for their propensity for malignant transformation to the extent of 10-20%. Exfoliative cytology is a simple and useful screening tool for detection of malignant or dysplastic changes in such lesions. AIMS A clinico-epidemiological and cytological study of oral leukoplakia was undertaken to detect their malignant potential and value of cytology in diagnosis. METHODS This 2 year duration multicentre study was undertaken on all patients presenting with oral white lesions to the out patient department of the two institutions. Those cases in which a specific cause (infective, systemic disease or specific disease entity) for the white lesions were elicited were excluded from the study. The group with idiopathic white lesions was included in the study and was subjected to periodic exfoliative cytological study at three monthly intervals to detect any malignant change. Patients presenting less than two times for follow up were excluded from the final analysis of the study. RESULTS Out of total 2920 patients studied, 89.53% showed benign, 9.93% showed dysplastic and, 0.72% showed malignant cells on exfoliative cytological study. All the dysplastic and malignant lesions were subjected to histopathological study by incisional biopsy. Among the dysplastic lesions 13.79% proved benign and the rest true dysplastic. Among the cytologically malignant group 4.76% showed dysplasia and the rest true malignant lesions. CONCLUSION Persistent leukoplakia has a potential for malignant transformation and exfoliative cytology could be a simple method for early detection of dysplastic and malignant changes.

Pityriasis versicolor in the pediatric age group

BACKGROUND Pityriasis versicolor (PV) is a mild chronic infection of the skin caused by Malassezia yeasts. Although it is primarily seen in adults, children are often affected in the tropics . METHODS Over a period of 2 years, children (up to the age of 14 years) who were clinically and mycologically diagnosed as PV were included in the study. The clinical and epidemiological pattern in different age groups was noted. RESULTS PV in this age group formed about 31% of the total cases of PV; 4.8% cases presented in infancy. The commonest site of involvement was the face in 39.9% of the cases. Most of the cases presented in summer months. CONCLUSIONS PV is not an uncommon disease among children in the tropics. There is a sudden resurgence of cases in the hot monsoons and even infants are not spared.

An open randomized comparative study of oral itraconazole pulse and terbinafine pulse in the treatment of onychomycosis

BACKGROUND Onychomycosis is a recalcitrant disease of the nails caused by dermatophytes, yeasts, and molds. AIMS To compare the clinical efficacy of oral itraconazole pulse therapy and oral terbinafine pulse therapy in onychomycosis. METHODS A randomized single-blind clinical comparative study was undertaken on 120 patients of onychomycosis during the period March 1999-February 2002. Sixty patients were randomly assigned to receive oral itraconazole 100 mg, two capsules twice daily for seven days a month and the other group of sixty patients received oral terbinafine 250 mg, one tablet twice daily for seven days every month. Four such monthly pulses were administered for each drug. The patients were evaluated at 4-weekly intervals till sixteen weeks and then at 24, 36 and 48 weeks. RESULTS We observed a clinical cure rate of 82% and mycological cure rate of 90% in the group of patients treated with itraconazole while the group with terbinafine showed clinical and mycological cure rates of 79% and 87% respectively. This difference was not statistically significant. CONCLUSIONS Both oral itraconazole and terbinafine are effective in the treatment of onychomycosis when administered in the pulse dosage form. Terbinafine is more cost effective while itraconazole has a broader spectrum of antimycotic activity.

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.

Nevus lipomatosus superficialis over neck, an atypical site

The most common histologic type of malignancy of the gall bladder is adenocarcinoma. The occurrence of metastatic adenocarcinoma from the gall bladder to the skin is rare, with only nine reported cases, including four in the English language. In the latter reports, the cutaneous metastases were located on the preauricular area, upper lip, umbilicus, scalp, neck, upper arms, and back. The lesions were described as nodular, smooth, and red-brown. Only one case showed epidermal invasion with ulceration. The presence of cutaneous metastases is a grave prognostic indicator, with an average survival time of 7.5 months.

Pityriasis rubra pilaris with Koebner's isomorphic phenomenon.

1. DiGiovanna JJ, Bale SJ. Clinical heterogeneity in epidermolytic hyperkeratosis. Arch Dermatol 1994;130:1026-35. 2. Irvine AD, McLean WH. Human keratin diseases:the increasing spectrum of disease and subtlety of the phenotype-genotype correction. Br J Dermatol 1999;140:815-28. 3. Sethuraman G, Khaitan BK, Dash SS, Chandramohan K, Sharma VK, Kabra M, et al. Icthyosiform erythroderma with rickets: report of five cases. Br J Dermatol 2007;158:603-6. 4. Bhagat SB, Bhagat SS, Sharma HK, Naik M, Amin P, Pandit J. Severe bilateral rachitic genu valgum in patients with nonbullous congenital icthyosiform erythroderma: a report of two cases and review of literature. J Pediatr Orthop B 2007;16:423-8. 5. Nayak S, Behera SK, Acharjya B, Sahu A, Mishra D. Epidermolytic hyperkeratosis with rickets. Indian J Dermatol Venereol Leprol 2006;72:139-42. 6. DiGiovanna JJ, Bale SJ. Epidermolytic hyperkeratosis: Applied molecular genetics. J Invest Dermatol 1994;102:390-4. 7. Judge MR, McLean WH, Munro CS. Disorders of Keratinization. In: Burns T, Breathnach S, Cox N, Griffiths C, editors. Rook’s Textbook of Dermatology. 7th ed. Blackwell Science: Oxford; 2004. p. 34.1-110. 8. Paller AS, Syder AJ, Chan YM, Yu QC, Hutton E, Tadini G, et al. Genetic and clinical mosaicism in a type of epidermal nevus. N Eng J Med 1994;331:1408-15. 9. Nomura K, Umeki K, Hatayama I, Kuronuma T. Phenotypic heterogeneity in bullous congenital ichthyosiform erythroderma: possible somatic mosaicism for keratin gene mutation in the mildly affected mother of the proband. Arch Dermatol 2001;137:1192-5. 10. Verma SB. Epidermolytic hyperkeratosis and ricketsa rare association. Ind J Dermatol 2005;50:107-9. hyperkeratotic ridges in flexural areas, normal palmoplantar surfaces and naevoid epidermolytic hyperkeratosis in a parent, diagnosis of NPS-1 epidermolytic hyperkeratosis with nutritional rickets was made. Epidermolytic hyperkeratosis includes a spectrum comprisinglinear epidermal verrucous naevus, icthyosis hystrix, icthyosis bullosa of Siemens and BCIE. Bullous congenital icthyosiform erythroderma is a chronic and disfiguring disease which has a tremendous impact on family and social life. An underlying genetic defect of keratin synthesis or degradation involving keratin K1 and/or keratin 10 has been suggested.[2,6] Mutations perturb keratin alignment, oligomerization and filament assembly, weakening the cytoskeleton compromising mechanical strength and cellular integrity of the epidermis, and leading to cytolysis and blistering.[6] The barrier function of the skin is markedly disturbed, leading to increased transepidermal water loss and bacterial colonization of the stratum corneum.[7] The mosaic form of BCIE is characterized by unilateral or bilateral streaks of hyperkeratosis that follow the lines of Blaschko.[8] A patient with an epidermolytic verrucous epidermal naevus is likely to have gonadal mosaicism as well as cutaneous mosaicism, and can therefore produce offspring with generalized BIE.[9] Our patient’s father had an epidermolytic verrucous epidermal naevus on right arm, indicating mosaic form of BCIE, leading to full-blown generalized disease in the child.

Coexistence of papulonecrotic tuberculide with lichen scrofulosorum.

Tuberculides, the supposedly immunologic reactions to the products of dead Mycobacterium tuberculosis bacilli deposited in the skin from distant foci of tubercular infection, are presently considered to be of two types-papulonecrotic tuberculide and lichen scrofulosorum. Simultaneous occurrence of both the types in the same patient is very rare. We report the case of an adult male without any known internal tubercular focus who showed two types of skin lesions, clinically typical and histopathologically consistent with the diagnoses of papulonecrotic tuberculide and lichen scrofulosorum, occurring simultaneously. Polymerase chain reaction showed the presence of Mycobacterium tuberculosis DNA in papulonecrotic tuberculide type of lesion, and both types of lesions responded promptly to anti-tubercular drugs.