Michel J, Gentil-Perret A, Gebska E, Cambazard F
Service de Dermatologie, CHU, 42055 Saint-Etienne Cedex 02.
Ann Dermatol Venereol. 2000 Jun-Jul;127(6-7):624-8.
Solitary histiocytoma is an uncommon form of Hashimoto-Pritzker syndrome and an exceptional type of histiocytosis with cells of undetermined origin. A solitary often ulcerated congenital nodule is generally observed. We report two cases, one of each form.
Both cases presented an ulcerative budding congenital tumefaction of the plantar aspect of the right foot for the first child and the parieto-axillary region in the second. Histology disclosed a granulomatous infiltrate of histiocytes positive for specific immunolabels (protein S100 and CD1a). In the first case, electron microscopy revealed histiocytes devoid of Birbeck granules and myelinoid bodies leading to the diagnosis of Langerhans histiocytosis with cells of unknown origin. In the second case, 18 p. 100 of the cells contained Birbeck granules. There has been no recurrence after a 5-year follow-up in a case.
These cases recall the congenital nature of some types of solitary histiocytomas. Indeed, congenital Langerhans histiocytoma can occur as a unique nodule. The tumefaction may lie in any localization. Histological diagnosis is required. The benign nature of these lesions is confirmed by the absence of distant lesions and the lack of recurrence after complete excision. About a dozen cases have been reported. Most have been Hashimoto-Pritzker syndromes. Only one case has been reported with cells of undetermined origin. The diagnosis of histiocytosis with cells of undetermined origin is made when the ultrastructure study demonstrates the vacuity of the histiocyte cytoplasm. This condition is similar to Hashimoto-Pritzker syndrome by the absence of recurrence and systemic diffusion. It can however be observed in adults. The undetermined cell types would correspond different phases of Langerhans cell maturation or involution.
孤立性组织细胞瘤是桥本-普利茨克综合征的一种罕见形式，是一种起源不明细胞的特殊组织细胞增多症类型。通常可见一个孤立的、常发生溃疡的先天性结节。我们报告两例，每种形式各一例。
第一例患儿表现为右足底溃疡性隆起性先天性肿物，第二例表现为顶腋区肿物。组织学检查显示组织细胞肉芽肿性浸润，特异性免疫标记物（蛋白S100和CD1a）呈阳性。第一例中，电子显微镜检查显示组织细胞无Birbeck颗粒和髓样小体，从而诊断为起源不明细胞的朗格汉斯细胞组织细胞增多症。第二例中，18%的细胞含有Birbeck颗粒。其中一例经5年随访无复发。
这些病例提示某些类型的孤立性组织细胞瘤具有先天性。实际上，先天性朗格汉斯细胞组织细胞增多症可表现为单个结节。肿物可位于任何部位。需要进行组织学诊断。这些病变的良性性质通过无远处病变及完整切除后无复发得以证实。已报道约十几例。大多数为桥本-普利茨克综合征。仅报道过一例起源不明细胞的病例。当超微结构研究显示组织细胞胞质空虚时，可诊断为起源不明细胞的组织细胞增多症。这种情况与桥本-普利茨克综合征相似，无复发及全身扩散。然而，也可在成人中观察到。起源不明的细胞类型可能对应朗格汉斯细胞成熟或退化的不同阶段。
