Nevus Unius Lateris with Bilateral Oral Mucosal Lesions: An Unusual Presentation.

Verrucous epidermal nevi (VEN) are cutaneous hamartomas characterized by keratinocytic hyperplasia. Majority are linear in distribution and tend to follow the Blaschko lines; however, some may have zosteriform (segmental) or systematized distribution involving widespread areas of skin. The systematized ones are further classified into "Nevus Unius Lateris" when one-half of the body is affected, and "Ichthyosis Hystrix" showing bilateral distribution, both being the uncommon forms. Although it can affect any body part, it rarely involves the head and neck region with seldom involvement of mucosae, scalp, and ear lobes. We saw a 6-year-old child with multiple hyperpigmented verrucous plaques predominantly present over left half of the body, ipsilateral alopecia scalp, and verrucous lesions involving mucosae of palate and tongue, which were present bilaterally. Previously, case reports of oral lesions related to VEN had demonstrated segmental, midline, or unilateral distribution. Hereby, we report this peculiar case of Nevus Unius Lateris with bilateral oral mucosal involvement, owing to its rarity.

Graham little piccardi lasseur syndrome: a rare case report with concomitant hypertrophic lichen planus.

Graham Little Piccardi Lasseur Syndrome (GLPLS) is a rare variant of lichen planopilaris comprising of a triad of multifocal and patchy cicatricial alopecia of scalp, non-cicatricial alopecia of axillae and pubic region and keratotic follicular papules over a body. Its exact etiology is not known until date, but primarily involves an immune mediated inflammatory reaction against the bulge region of hair follicles resulting in cicatricial alopecia. We report an extensive and diffuse presentation of this rare syndrome in a middle-aged female with hypertrophic lichen planus (LP) present on malar region of face, etiologically relating GLPLS to LP.

Bardet-Biedl syndrome: a rare case report from North India.

The Bardet-Biedl syndrome (BBS) is a rare ciliopathic human autosomal-recessive disorder, affecting multiple organ systems. Less than 15 cases have been reported from India. The authors present a classical case of BBS presenting to dermatology outpatient with hypogonadism and features such as marked central obesity, retinal dystrophy, polydactyly, structural renal abnormalities and mental retardation, along with a brief review of the literature. This case exemplifies the need for multidisciplinary management in such cases.