Progressive Nodular Histiocytosis: Report of a Case and Review of the Literature

Table 1

Comparison of clinicopathologic features of the different entities in the differential diagnosis of progressive nodular histiocytosis.

Clinicopathologic feature	PNH	BCH [25]	JXG [23]	AXG [39]	GEH [26]	XD [28]	SRH/MRH [40]	Dermatofibroma [41]
Age at onset	Middle-aged to older adults	Typically in the first year of life	Childhood	Adulthood	Adults and children can be affected	Most before 25 years of age	Median age 35 years	Most common in the 20s to 40s
Pattern of cutaneous manifestation	Multiple, widespread cutaneous papules and nodules	Multiple papules most commonly on the head and neck	Solitary cutaneous or subcutaneous nodules in up to 83% of cases	Solitary nodule in up two-thirds of cases	Dozens to hundreds of papules over the trunk and extremities	Multiple and widespread papules	Cutaneous papule(s) or nodule(s)	Usually solitary with predilection for the extremities
Mucous membrane involvement	Yes	Rare	Yes	No	Rare	Yes	No (SRH); yes (MRH)	Yes (benign fibrous histiocytoma) [42]
Visceral involvement	Not usual	No	Yes, in up to 10% of cases	Not usual	Not usual	Yes	No (SRH); yes (MRH)	No
Association with systemic disease	No	Not usual [43]	Can occur	No	Not usual	Yes, 50% of cases associated with diabetes insipidus	MRH may be associated with systemic vasculitis [44] and malignancy [45]	No
Histology	Spindled lesional cells in the dermis	Demarcated infiltrates of tissue macrophages in the reticular dermis	Foamy and spindled mononuclear cells, Touton giant cells	Foamy and spindled mononuclear cells, Touton giant cells	Dermal collection of spindled cells and scattered Langhans giant cells	Macrophages with scalloped nuclei and foamy cytoplasm, as well as Touton and foreign body-type giant cells	Circumscribed aggregate(s) of oncocytic epithelioid cells in the upper and middermis	Poorly circumscribed dermal proliferation of spindled and epithelioid cells
Clinical course	Usually progressive and unremitting	Frequently undergoes spontaneous regression	Spontaneous regression is common in children but less so in adults [39]	Spontaneous regression is uncommon	Frequently undergoes spontaneous regression	Most frequently persistent; spontaneous regression is rare	Does not undergo spontaneous regression	Spontaneous regression is uncommon
Recurrence	Yes	No	No	No	No	No	No	No

PNH: progressive nodular histiocytosis; BCH: benign cephalic histiocytosis; JXG: juvenile xanthogranuloma; AXG: adult xanthogranuloma; GEH: generalized eruptive histiocytosis; XD: xanthoma disseminatum; SRH: solitary reticulohistiocytosis; MRH: multicentric reticulohistiocytosis.