Open Access Open Access  Restricted Access Subscription or Fee Access

A Rare Case Report—Xeroderma Pigmentosum Associated Skin Cancer

Shweta Parmar, Saumya P. Srivastava, L. Gopichandran

Abstract


Xeroderma pigmentosum (XP) is a rare autosomal recessive disease, in which the ability of the DNA repair is impaired, symptom presentation is majorly observed over the skin exposed to sunlight and can range from minor hyperpigmentation to ulcerative nodules. We report a rare case of skin cancer associated with xeroderma pigmentosum, in a 6-year-old male child, admitted in a cancer hospital. Initially, the patient was treated for xeroderma pigmentosum. The condition exaggerated and took a form of skin cancer with presentation of distributed pattern of hypo and hyper pigmented macules. Lesions were closely aggregated over the face with ulcerative nodules over scalp, nose, near inner canthus of the eyes. The patient underwent excision followed by reconstruction and post-procedure external beam radiotherapy (EBRT) with the dose of 54Gy/30 in fraction for 6 weeks. The condition did not resolved; patient then underwent radiofrequency excision of scalp nodule with electrosurgical excision of outgrowing lesion in medial canthus under general anesthesia (GA), and was maintained on isotretinoin 10 mg with photoprotection. Procedure of electro chemotherapy was done, with intra-tumoral injection (2000 IU Bleomycin) at multiple sites covering entire tumor area in 8 pulses of 100 µs on left facial lesion and wide local excision (WLE) with full thickness graft of medial canthus lesion was performed using diathermy

Full Text:

PDF

References


L Feller, NH Wood, MH Motswaledi. Xeroderma pigmentosum: a case report and review of the literature. J Prev Med Hyg.2010;51(2):87–91.

S Jasuja, A Mathur, SS Bhakal, et al. Xeroderma pigmentosum with squamous cell carcinoma of face. A rare case report of two siblings. Indian J Cancer.2015;52(4):567–567.

Genetics Home References, Xeroderma pigmentosum. [Online] Available from: https://ghr.nlm.nih.gov/condition/xeroderma-pigmentosum

Hinkle, Brunner & Suddarth’s Textbook of Medical-surgical Nursing, 13th edition. United States: Wolters Kluwer India Pvt. Ltd;2014.

Saumya Panda, Nonmelanoma skin cancer in India: current scenario. Indian J Dermatol. 2010;55(4):373–8.

NORD (National Organization for Rare Disorders), (2020, Jul.) Xeroderma Pigmentosum. [Online] Available from: https://rarediseases.

org/rare-diseases/xeroderma-pigmentosum/

Medscape, (2019, Dec.) Xeroderma Pigmentosum Differential Diagnoses. [Online]. Available from: https://emedicine.medscape.com/article/1119902-differential

DeVita Jr Vincent T Lawrence, et al. Devita, Hellman, and Rosenberg’s Cancer: Principles & Practice of Oncology. 10th edition. Philadelphia: Wolters Kluwer;2015.

Potter PA, Perry AG, Hall A, et al. Fundamentals of nursing. 9th ed.St. Louis, Mo: Mosby Elsevier;2017.

Danette Langbecker, Abbey Diaz, Raymond J Chan, et al. Educational programmes for primary prevention of skin cancer. Cochrane Database Syst Rev. 2018(3):CD011061.

Sudhir M Naik, Ashok M Shenoy, A Nanjundappa. Cutaneous malignancies in xeroderma pigmentosum: earlier management improves survival. Indian J Otolaryngol Head Neck Surg. 2013;65(2):162–7.


Refbacks

  • There are currently no refbacks.