Rajesh Kumar Mandal

Waardenburg syndrome: A report of three cases

Waardenburg syndrome (WS) is a rare autosomally inherited and genetically heterogeneous disorder of neural crest cell development with distinct cutaneous manifestations. Based on the clinical presentations, four subtypes of the disease are recognized. A careful clinical evaluation is required to differentiate various types of WS and other associated auditory-pigmentary syndromes. We describe a case series of WS to highlight the wide spectrum of manifestations of the syndrome including a rare association.

Mucocutaneous features of hand, foot, and mouth disease: A reappraisal from an outbreak in the city of Kolkata

1. Ramam M. Linear epidermolytic acanthoma or adult-onset verrucous epidermal nevus? Indian J Dermatol Venereol Leprol 2010;76:563. 2. Thomas M, George R, Thomas M. Linear epidermolytic acanthoma of vulva: An unusual presentation. Indian J Dermatol Venereol Leprol 2010;76:49-51. 3. Shapiro L, Baraf CS. Isolated epidermolytic acanthoma. Arch Dermatol 1970;101:220-3. 4. Quinn TR, Young RH. Epidermolytic hyperkeratosis in the lower genital tract: An uncommon simulant of mucocutaneous papilloma virus infection: A report of two cases. Int J Gynecol Pathol 1997;16:163-8. 5. Swann MH, Pujals JS, Pillow J, Collier SL, Hiatt K, Smoller BR. Localized epidermolytic hyperkeratosis of the female external genitalia. J Cutan Pathol 2003;30:379-81. 6. Adams BB, Mutasim DF. Adult onset verrucous epidermal nevus. J Am Acad Dermatol 1999;41:824-6. 7. Kawaguchi H, Takeuchi M, Ono H, Nakajima H. Adult onset of inflammatory linear verrucous epidermal nevus. J Dermatol 1999;26:599-602. 8. Kosann MK. Inflammatory linear verrucous epidermal nevus. Dermatol Online J 2003;9:15. 9. Moss C, Shatidulla H. Naevi and other developmental defects. In: Burn T, Breatchnach S, Cox N, Griffiths C, editors. Rook’s Textbook of Dermatology. West Sussex: Blackwell Publishers; 2010. p. 18.1-107. 10. Sarifakioglu E, Yenidunya S. Linear epidermolytic verrucous epidermal nevus of the male genitalia. Pediatr Dermatol 2007;24:447-8.

Keratoacanthoma centrifugum marginatum: a rare variant of keratoacanthoma.

A 42-year-old businessman presented with an asymptomatic, gradually expanding, elevated skin lesion on his right forearm for the duration of 1 year. There was no history of injury or preexisting skin lesion at that site. His personal and family histories were both negative for any form of skin cancer. He had no systemic symptoms. Cutaneous examination revealed a well-demarcated, nontender plaque measuring 6 3 7 cm in size and studded with multiple dome-shaped nodules at the margin (Figure 1). The nodules (approximately 1 3 1.5 cm) were firm in consistency and had central keratin-filled craters. There were multiple small shallow cribriform scars at the center of the plaque. There was no regional lymphadenopathy. Examination of the cutaneous appendages was normal. His physical examination and review of systems were unremarkable. Routine laboratory investigations were normal. Histopathologic examination of an excisional biopsy specimen from a peripheral nodule showed a deep crater invaginating the epidermis containing deeply eosinophilic keratinized cells (Figure 2). Epidermal proliferations buttressed the crater. Plenty of fully keratinized horn pearls were also evident in the sections (Figure 3). No evidence of cellular atypia was seen. The tumor mass was sharply demarcated from the surrounding dermis. There was a moderately dense mononuclear infiltration in the upper dermis around the epidermal proliferations. Abdominal ultrasonography and chest radiography were noncontributory. Based on the clinical evolution with centrifugal expansion in association with central restitution or scarring and histologic features, a diagnosis of keratoacanthoma centrifugum marginatum (KCM) was made. The patient was initially treated with systemic isotretinoin (0.5 mg/kg/d) for 1 month, without any improvement. Following this, liquid nitrogen cryotherapy was instituted at three weekly intervals. Each session consisted of two 1-minute freeze cycles applied to the nodular components of the lesions. Lesional crusting was seen a few days after each session. The lesion significantly regressed (Figure 4) within 2K months, but the patient was subsequently lost to follow-up. Keratoacanthomas (KAs) are relatively rare benign epidermal tumors characterized by rapidly growing, firm, symmetric, dome-shaped nodules with keratin-filled craters in their centers and a tendency toward spontaneous regression. KA presents with either solitary or multiple lesions. Approximately 98% of KAs are of the classic, solitary type, mainly occurring in elderly persons on exposed areas. Several variants of KA with multiple lesions and some rare variants of solitary KA, namely, subungual KA, giant KA, and KCM, have been described in the literature. Kurschat and colleagues note that JC Belisario first described the KCM as a separate entity in 1965. KCM is an extremely rare variant of KA, with less than 50 cases reported in the English literature since it was first described. Moreover, KA is uncommon in dark-skinned people. Although the etiopathogenesis of KA is