Progressive nodular histiocytosis D76.3

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 19.10.2022

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Histiocytosis nodular cutaneous reactive; Nodular cutaneous reactive histiocytosis; Nodular non-Langerhans cell histiocytosis; Non-Langerhans cell histiocytosis nodular; PNH; progressive nodular histiocytoma; progressive nodular histiocytosis

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Robinson et al. 1963; Taunton, 1978;

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Rare, benign progressive histiocytosis of the non-Langerhans cell group confined to the skin, the entity of which is still controversial. A variant of multicentric reticulohistiocytosis is discussed.

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Unknown. In some cases the association with chonic-myeloid leukemia or brain tumors is discussed.

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Generalized, typical facial infestation.

Clinical features
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Integument: Generalized and symmetrical yellow-brown or skin-colored papules and nodules with characteristic involvement of the face. In rare cases manifestation as facies leontina.

General: Good clinical general condition. Involvement of joints or internal organs not confirmed.

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Accumulation of irregular histiocytes (foam cells and giant cells) mixed with lymphocytes. No mitotic figures.

Electron microscopy: Pleomorphic inclusion bodies as in multicentric reticulohistiocytosis.

Immunohistology: Posivity for Ki-M1p, CD68, CD13, often alphaSMA.

Differential diagnosis
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multicentre reticulohistiocytosis, further clinical pictures from the so-called xanthogranuloma group of non-Langerhans cell histiocytoses.

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Due to the rarity of the disease there are no generally applicable guidelines. The most gentle surgical removal of the nodules is highly recommended (see case report). Successes are described under cytostatic therapy with vinblastine and therapy with fast electrons.

Case report(s)
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29-year-old patient with generalized, coarse papules and nodules on the entire integument which had been increasing continuously for 10 years. Recurrent tendency after excision of single nodules, sometimes spontaneous regression.

Findings: Reduced AZ with normal EZ; evidence of several hundreds of papules and painless, red to red-brown nodules up to 3.0 cm in size. Generalized distribution pattern with preference of legs and buttocks. Here, areal infestation.

Laboratory: Severe microcytic anemia (Hb 7.7 g/dL, MCV 62.8 fl). Serum iron markedly decreased.

Histology: Proliferations of histiocytes, with foamy loosened cytoplasm. Berlin blue stain: Hemosiderin-storing CD68+, CD1A- macrophages.

Bone marrow biopsy: Severe microcytic anemia, picture as in iron deficiency.

Therapy and course: Blood transfusions up to a HB of 9.0. Excision of all nodes. After 6 months, some new nodules had appeared on the extremities, otherwise stable condition. Hb continuously at 9-10 g/dl.

Comment: One of the rarest non-Langerhans cell histiocytoses is progressive nodular histiocytosis. The nodular lesions often involve the entire integument and possibly the mucous membranes. Excision of the nodules may be considered an effective therapy for severe microcytic anemia (siderophagia).

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  1. Beswick SJ et al (2002) Progressive nodular histiocytosis in a child with a hypothalamic tumor. Br J Dermatol 146: 138-140
  2. Gianotti F et al (1985) Histiocytic syndrome: A review. J Am Acad Dermatol 13: 383-404.
  3. Gonzales RA et al (2000) Progressive nodular histiocytosis accompanied by systemic disorders. Br J Dermatol 143: 628-631.
  4. Hilker O (2011) Progressive nodular histiocytosis with severe microcytic anemia. Abstract CD 46th DDG Conference DK02/04.
  5. Hilker O et al (2013) Progressive nodular histiocytosis. J Dtsch Dermatol Ges 11:301-317.
  6. Kiyohara T et al (2018) Progressive nodular histiocytosis with large nodules and a bulky mass. Content 99: Issue 6

  7. Kumar BC (2011) Generalized eruptive histiocytosis mimicking leprosy 77: 498-502
  8. Nofal A et al (2011) Progressive nodular histiocytosis: a case report and literature review. Int J Dermatol 50:1546-1551
  9. Taunton OD et al (1978) Progressive nodular histiocytoma. Arch Dermatol 114: 1505-1508.


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Last updated on: 19.10.2022