Progressive nodular histiocytosis accompanied by systemic disorders

Abstract

Progressive nodular histiocytosis is a proliferative process of histiocytes, the main element of which is the dermal dendrocyte. It is considered to form part of a group of histiocytic disorders related to juvenile xanthogranuloma, which also includes xanthoma disseminatum, benign cephalic histiocytosis, spindle cell xanthogranuloma and generalized eruptive histiocytosis; disorders which perhaps represent the spectrum of one single entity. We present the case of a 57-year-old man who, for 26 years, had had a progressively deforming process of cutaneous lesions, with systemic involvement, including chronic myeloid leukaemia, hepatosplenomegaly, hypothyroidism, hyperuricaemia and hypocholesterolaemia. We have not been able to establish precisely the relationship between these features.

Papular Xanthoma Associated with Angiokeratoma of Fordyce: Considerations on the Nosography of This Rare Non-Langerhans Cell Histiocytoxanthomatosis

“…Furthermore, the similarity of the histological picture and of the immunocytochemical findings of PNH with those of PX and other histiocytoxanthomatoses [18, 19, 20]supports the hypothesis that the progressive forms of PX and PNH can actually represent the same entity.…”

“…To conclude, it is important to remember that the possible association of non-LC histiocytoxanthomatoses with hematological disorders [10, 20]suggests the need for a long and accurate follow-up.…”

“…Since the histological and immunocytochemical features of many xanthomized non-LC histiocytoses (juvenile xanthogranuloma, PX, xanthoma disseminatum and PNH) may be very similar [18, 19, 20]and since nonxanthomized non-LC histiocytoses like benign cephalic histiocytosis and generalized eruptive histiocytosis can transform into xanthomized non-LC histiocytosis [22, 23, 24, 25, 26], the exact diagnosis of these overlapping diseases is in many cases mainly based on clinical findings. Clinically, xanthoma disseminatum differs from PX because of (a) the tendency of the lesions to become confluent and to merge into plaques; (b) the involvement of skin folds and mucous membranes and (c) the association with diabetes insipidus [27].…”

Progressive Histiocytosis: Description of a Case of Slow-Course Non-Langerhans Cell Histiocytosis

“…In our patient, no systemic disorders were observed, differently from the case reported by Ruiz et al [8]. The lack of systemic involvement rules out multicentric reticulohistiocytosis but not its cutaneous form, diffuse cutaneous reticulohistiocytosis (DCR), which usually appears at a later age [11, 12].…”

“…Among the various types of NLCH, progressive nodular histiocytosis (PNH) [1, 4, 5, 6, 7, 8, 9, 10]is clinically the most similar to our case. In our patient, however, both the nodular lesions typical of PNH and the facies leonina sometimes associated with this disease were absent.…”