Congenital Self-healing Histiocytosis

Solitary cutaneous dendritic cell tumor in a child: Role of dendritic cell markers for the diagnosis of skin Langerhans cell histiocytosis

“…22 In contrast, depending on the study, only 10% to 30% (or even none) of the dermal tumor cells of CSHRH exhibit these granules. 7,[23][24][25][26] In accordance with these studies, in our case S-100 protein and CD1a-positive tumor cells were negative for Langerin, thereby excluding the presence of Birbeck granules. Therefore the tumor cell cannot definitely be identified as belonging to the LC subset of skin DCs.…”

“…23 Studies with the Lag antibody demonstrated that skin lesions of classic LCH are predominantly composed of Birbeck granules containing LC, which are rarely found in other organs, such as lymph nodes, liver, and spleen. 27 Rather than the presence of two populations, it appears more likely that local factors, such as the tumor influencing cytokine milieu, may lead to different states of cell differentiation.…”

“…29 However, it was later demonstrated that these lysosome-like bodies may be deformed Birbeck granules. This led to the assumption that tumor cells containing such dense bodies may be in a state of regression 23,25 and may therefore be correlated with the self-limited course of the disease.…”

A Solitary Variant of Congenital Self‐healing Reticulohistiocytosis: Solitary Hasimoto‐Prltzker Disease

“…The unique features of this disorder were presence at birth or perinatally of numerous cutaneous nodules, absence of internal involvement, and universal spontaneous and permanent healing. Several cases have since been described (4)(5)(6)(7)(8)(9)(10), allowing the following list of criteria to be proposed for this entity:…”

“…Disorders of Langerhans' cells have been difficult to study, since LC are not specifically identifiable in routine histologic material. Histiocytosis X (3) in all its forms and congenital self-healing reticulohistiocytosis (CSHR) (4)(5)(6)(7)(8)(9)(10) are the only two diseases identified to date as proliferations of the cells. Cutaneous lesions in both conditions usually are multiple.…”