The Indeterminate Cell Proliferative Disorder: Report of a Case Manifesting as an Unusual Cutaneous Histiocytosis

Abstract

A patient with an unusual, distinctive cutaneous histiocytosis is described. Extensive morphologic, antigenic, and enzymatic studies indicate that this histiocytosis represents a proliferative disorder of cutaneous indeterminate cells. Features that distinguish this disorder from other histiocytoses are discussed.

Unusual variants of non-Langerhans cell histiocytoses

“…5,16,18,20,24 In summary, these cells display similar histologic and antigenic features to LC. Ultrastructurally, however, they differ in that Birbeck granules are absent.…”

“…26 In addition to the cases mentioned above, chemotherapy regimens, including vinblastine and 2-chlorodeoxyadenosine, respectively, were necessary in two other cases with extensive and disfiguring cutaneous disease despite the absence of visceral involvement. 5,17,22 We observed two adult male patients with ICH having widespread cutaneous eruptions without evidence of systemic disease at onset. 29 In these patients, oral cyclophosphamide gave a good clinical response.…”

“…On the other hand, 24 cases with widespread cutaneous lesions have been described, 3,5,6,[8][9][10]15,[17][18][19][20][21][22][23][24][25][26] the majority of which having an indolent or selflimited course; indeed, in a number of reported cases treatment and follow-up were not stated. 3 Table I.…”

“…Two clinical subtypes seem to predominate: a solitary nodular form [11][12][13][14]16 (Fig 1, A) and a multiple papulonodular form 3,5,6,[8][9][10]15,[17][18][19][20][21][22][23][24][25][26] (Fig 1, B). The former is usually characterized by a single, soft red asymptomatic nodule about 1 cm in diameter; the latter presents with a widespread eruption of multiple, firm asymptomatic lesions ranging in size from a few millimeters to 1 cm, varying in color from dark red to brownish and covered by intact skin.…”

“…7 Age of onset and incidence ICH affects predominantly adults, showing no sex-or age-specific predilection, although about one third of the cases so far reported in the literature are pediatric. 4,[8][9][10][11][12][13][14][15] Since the original description of Wood et al, 5 only about 20 cases of ICH had been reported, until a recent study on a large collection that included 18 patients with ICH. 4 …”