Surgical Excision with Rotational Flap Reconstruction for Basosquamous Cell Carcinoma in Xeroderma Pigmentosa: A Case Report

Abstract

Xeroderma pigmentosa (XP) is a rare autosomal recessive disorder which is characterized by a defect in nucleotide excision repair of DNA following exposure to UV radiation. This leads to hypersensitivity to sunlight causing pigmented skin lesions, photophobia and a 1000-fold increase in risk of developing cutaneous malignancies like basal cell carcinomas, squamous cell carcinomas and melanomas of head and neck. We present an interesting case of a 28 year old man with basosquamous cell carcinoma secondary to XP treated successfully with surgical excision and reconstruction with rotational flap technique.

Keywords: Basal cell carcinoma; Rotational flap; Xeroderma pigmentosa.

Fig. 1

Pre op photo with generalized freckles and hyperpigmented macules. Note the brownish black, circular ulcerated lesions on both sides of nose

Fig. 2

Biospy of the Right sided lesion shows tissue lined by stratified squamous epithelium with round to oval tumor cells seen in sub-epithelium arranged in sheets and nests with containing hyperchromatic nuclei, moderate eosinophilic cytoplasm, peripheral palisading and retraction artifact. Mitotic cells 1–2/10HPF

Fig. 3

Post op 8th day with surgical excision and rotational flap reconstruction of the left sided lesion

Fig. 4

Biopsy of left sided lesion showing tissue arranged in solid nests with peripheral palisading and retraction artifact. Individual tumor cells are pleomorphic with round to oval hyperchromatic nucleus and irregular nuclear membrane with scant cytoplasm. Melanin pigment incontinence at dermoepidermal junction seen

Fig. 5

6 months follow up picture after Right sided excision biopsy and reconstruction with rotational flap. Note that the margins are well healed and structural intricacy is maintained