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Progressive nodular histiocytoma
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Progressive nodular histiocytoma

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Contributors: David Brodell MD, Susan Burgin MD
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Synopsis

Progressive nodular histiocytoma (PNH) is a rare, progressive disease characterized by the widespread eruption of yellow-brown papules, plaques, and nodules. Lesions are both superficial and deep. The face and mucous membranes may be affected while flexors are often spared. Papules are randomly scattered, while nodules are more likely to be found on the trunk.

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.

Codes

ICD10CM:
D48.1 – Neoplasm of uncertain behavior of connective and other tissue

SNOMEDCT:
302843004 – Histiocytoma

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Diagnostic Pearls

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Differential Diagnosis & Pitfalls

  • Multicentric reticulohistiocytosis – May present with single or multiple papulonodule(s). If multiple, often associated with destructive arthritis and associated malignancy.
  • Generalized eruptive histiocytosis – Recurrent eruptions of small, red-brown papules, most often in adults.
  • Xanthoma disseminatum – Diabetes insipidus and mucous membrane / cutaneous xanthomas. Commonly symmetric eruption of yellow / brown / red papules / plaques.
  • Papular xanthoma (Erdheim-Chester disease) – Associated with bilateral long bone sclerosis with half of patients manifesting cutaneous lesions (red-brown papules / nodules) on presentation. Mortality / morbidity is significant.
  • Sarcoidosis
  • Lepromatous leprosy
  • Nodular mastocytoma
  • Juvenile xanthogranuloma – 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.

Best Tests

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Management Pearls

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Therapy

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References

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Last Reviewed: 01/24/2017
Last Updated: 03/27/2017
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Progressive nodular histiocytoma
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Progressive nodular histiocytoma : Pigmented papule, Smooth nodule
Clinical image of Progressive nodular histiocytoma
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