PROGRESSIVE NODULAR HISTIOCYTOSIS

By Gary M. White, MD


Progressive nodular histiocytosis (PNH) is a very rare type of non-Langerhans cell histiocytosis. It is characterized by progressive appearance of papules and nodules without spontaneous resolution.

Clinical

Multiple, yellowish papules to dermal nodules measuring from 5.0 mm to 5.0 cm is seen in PNH. Larger lesions may be painful and disfiguring. Infiltration of the face may produce a leonine fancies. Mucosal involvement is rare, but one patient with laryngeal involvement died due to airway obstruction [Indian Dermatol Online J 2016;7:516-9].

Differential Diagnosis

PNH and generalized eruptive histiocytoma are very similar but PNH tends to be more progressive and lesions are larger. Xanthoma disseminatum usually prefers the flexures.

It should be noted that various overlap cases occur and some patients seem to have one condition that evolves into another. Thus, GEH, PMH, BCH and disseminated JXGs may lie along a spectrum and all derive from the same cell, the dermal dendritic cell. Xanthoma disseminatum may be considered, but lesions of XD prefer the flexures.

Similar and Potentially Overlapping Non-Langerhans Cell Histiocytoses

Benign Cephalic Histiocytosis
Disseminate Juvenile Xanthogranuloma
Generalized Eruptive Histiocytoma
Progressive Nodular Histiocytosis

Treatment

There is no reliably effective treatment.

References

Dermatology Online Journal, 19(6)

JAAD December 2007 Volume 57, Issue 6, Pages 1031–1045

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