Afsar Fatma Sule, Ergin Malik, Ozek Gulcihan, Vergin Canan, Karakuzu Ali, Seremet Sila
Ataturk Research and Training Hospital, Esmirna, Turquia.
Dr. Behcet Uz Children's Hospital, Esmirna, Turquia.
Rev Paul Pediatr. 2017 Jan-Mar;35(1):115-119. doi: 10.1590/1984-0462/;2017;35;1;00015.
To report a case of late-onset self-healing Langerhans cell histiocytosis.
A 4½-month-old female patient presenting with an eythematopurpuric eruption underwent a skin biopsy for histopathology and was first diagnosed with isolated cutaneous Langerhans cell histiocytosis. Her lesions regressed within a few months and she was retrospectively diagnosed with late-onset self-healing Langerhans cell histiocytosis after being without skin or systemic involvement in a follow-up four years later.
Self-healing Langerhans cell histiocytosis, which is characterized by clonal proliferation of Langerhans cells and presents with cutaneous lesions, is a rare self-limited variant of histiocytosis and can only be diagnosed retrospectively, after the patient remains free from systemic involvement for several years. Although it presents at birth or during the neonatal period, only a few cases of its late-onset type regarding the age of onset have been reported. Purpuric lesions that appear after the neonatal period serve as a clue for late-onset self-healing Langerhans cell histiocytosis and the patients should be monitored regularly for systemic involvement if the diagnosis is confirmed by a cutaneous biopsy.
报告一例迟发性自愈性朗格汉斯细胞组织细胞增多症病例。
一名4个半月大的女性患者，出现红斑紫癜性皮疹，接受了皮肤活检以进行组织病理学检查，最初被诊断为孤立性皮肤朗格汉斯细胞组织细胞增多症。她的皮损在几个月内消退，四年后的随访中未出现皮肤或全身受累情况，随后她被回顾性诊断为迟发性自愈性朗格汉斯细胞组织细胞增多症。
自愈性朗格汉斯细胞组织细胞增多症以朗格汉斯细胞的克隆性增殖为特征，表现为皮肤损害，是一种罕见的组织细胞增多症的自限性变体，只有在患者数年无全身受累后才能进行回顾性诊断。虽然它在出生时或新生儿期出现，但关于发病年龄的迟发型病例仅有少数报道。新生儿期后出现的紫癜性损害是迟发性自愈性朗格汉斯细胞组织细胞增多症的线索，如果皮肤活检确诊，应对患者进行定期监测以防全身受累。
