Al-Hassani Fawaz, Davies James, Pang Calver, Knight Simon
Leeds General Infirmary, Plastic Surgery, Great George St, Leeds LS1 3EX, UK.
Bradford Royal Infirmary, Plastic Surgery, Duckworth Ln, Bradford BD9 6RJ, UK.
JPRAS Open. 2017 Mar;11:33-36. doi: 10.1016/j.jpra.2017.01.002.
Incontinentia pigmenti (IP) is a rare genetic skin disorder with an X-linked dominant inheritance that is seen almost exclusively in females. Subungual keratoacanthoma (SUKA) is a rare benign neoplasm of the nail bed associated with IP. The recommended initial treatment of SUKA is conservative thereby sparing the digit in the form of excision and curettage. However, definitive treatment involves terminalisation of the affected digit. We report a case in a 54-year-old woman with IP who developed SUKA who was treated with local subungual excision and subsequent skin grafting who had previous terminalisation of other digits for the same condition. Subungual keratoacanthoma is a rare benign neoplasm that is typically treated with curettage and excision at the initial stages with terminalisation as the definitive treatment. Given the highly positive outcome in this case with minimal sacrifice to the dexterity of the patient and preservation of digit length, we feel that local subungual excision and skin grafting should be strongly considered as an alternative to amputation in moderate to severe cases of the condition.
色素失禁症（IP）是一种罕见的遗传性皮肤病，呈X连锁显性遗传，几乎仅见于女性。甲下角化棘皮瘤（SUKA）是一种与色素失禁症相关的罕见的甲床良性肿瘤。SUKA的推荐初始治疗是保守治疗，即通过切除和刮除术保留手指。然而，确定性治疗包括截除患指。我们报告一例54岁患有色素失禁症并发生SUKA的女性病例，该患者接受了甲下局部切除及随后的植皮治疗，其其他手指此前因相同情况已被截除。甲下角化棘皮瘤是一种罕见的良性肿瘤，初始阶段通常采用刮除术和切除术治疗，最终治疗为截除术。鉴于该病例取得了高度积极的结果，对患者灵活性的影响最小且保留了手指长度，我们认为在该疾病的中重度病例中，应强烈考虑将甲下局部切除和植皮作为截肢的替代方案。
