Jayanta Kumar Das

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.

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.

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.

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.