Progressive nodular histiocytoma
Patients aged 40-60 are most commonly affected, but PNH may present at any age. There are no definitive associations with other diseases. Nodule histiocytes may accumulate iron, and PNH has been anecdotally associated with iron-deficiency anemia.
PNH is a type of non-Langerhans cell histiocytosis. It is not associated with Langerhans histiocytosis or abnormal serum lipids. Histologically, juvenile xanthogranuloma and PNH share many characteristics.
PNH lesions may be very painful; ulceration and bleeding may occur. There is no spontaneous remission, and new lesions continue to arise throughout the disease course. Management of skin lesions is notoriously difficult; multiple surgeries may be necessary. That being said, PNH is not associated with any visceral changes.
D48.19 – Other specified neoplasm of uncertain behavior of connective and other soft tissue
302843004 – Histiocytoma
Differential Diagnosis & Pitfalls
- – May present with single or multiple papulonodule(s). If multiple, often associated with destructive arthritis and associated malignancy.
- Generalized – Recurrent eruptions of small, red-brown papules, most often in adults.
- – Diabetes insipidus and mucous membrane / cutaneous xanthomas. Commonly symmetric eruption of yellow / brown / red papules / plaques.
- Papular xanthoma () – Associated with bilateral long bone sclerosis with half of patients manifesting cutaneous lesions (red-brown papules / nodules) on presentation. Mortality / morbidity is significant.
- – About 80%-90% of all non-Langerhans cell histiocytosis cases. Generally characterized by a single papule / nodule in an infant or child. Shares many histologic features with PNH and is often considered on the same spectrum.