Histiocytomas, generalized eruptive D76.3

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 29.10.2020

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Synonym(s)

disseminated adult xanthogranulomas

History
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Müller and Winkelmann, 1963

Definition
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Very rare, spontaneously healing (benign) non-Langerhans cell histiocytosis

Etiopathogenesis
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Unknown; self-limited benign histiocytic systemic disease.

Manifestation
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Mainly occurring in adults, very rarely in children. So far, less than 20 cases have been described in the literature.

Efflorescence(s)
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Disseminated, brown smooth, non-confluent, asymptomatic papules.

Localization
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Face, trunk, proximal extremities.

Clinical features
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Progression in phases (phases of activity lasting from a few weeks to months). Generalised clinical picture with disseminated, skin-coloured or reddish-brown, smooth, mostly asymptomatic (rarely slight itching), 0.3-1.0 cm large papules which do not tend to confluence and appear on unmodified skin. At full manifestation usually 50 to 1000 efflorescences occur. Mucous membranes, palmae and plantae are almost always free. Recess of the large bends.

Histology
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  • Regular layered surface epithelium with orthokeratotic keratinization. In the upper and middle dermis, dense patchy infiltrate consists of slightly vacuolated predominantly mononuclear macrophages (CD68+, only very occasionally S 100+, CD1a-) and lymphocytes; no foam or giant cells. Infiltrate-free border zone below the epidermis.
  • Electron microscopy: Large amounts of electron dense, regularly structured "laminated bodies" appear in the tumor cells, but no Langerhans granules.
  • Usually no foam cells or giant cells are present.
  • Immunohistology

    Note! Histiocytes are positive for stabilin-1 and CD68 markers, negative for CD1a and S100 markers (S 100 only occasionally positive).

Differential diagnosis
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Other non-Langerhans cell histiocytoses, in particular multicentric reticulohistiocytosis and multinodular, storing, partly giant cell reticulohistiocytosis Differentiation from congenital self-healing reticulohistiocytosis (Birbeck granules negative; S100 positive; CD1a negative) is possible by ultrastructural means and immunohistology.

Complication(s)
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Therapy
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Waiting, because of benign course with spontaneous remissions.

Radiation therapy
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Individual case reports on positive effects of PUVA therapy are available (systemic PUVA with meladinine 0.5 mg/kg bw; GD 1.25 J/cm2).

Progression/forecast
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Later development of acute leukaemia is possible, therefore regular checks of the differential blood count.

Literature
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  1. Lan Ma H et al (2006) Successful treatment of generalized eruptive histiocytomas with PUVA. J Dtsch Dermatol Ges 5: 131-114
  2. Marzano AV et al (2002) Guess what! Generalized eruptive histiocytosis (histiocytoma). Eur J Dermatol 12: 205-206
  3. Matsushima Y et al (1999) Generalized eruptive Histiocytoma of childhood associated with rheumatic fever. Eur J Dermatol 9: 548-550
  4. Misery L et al (2001) Generalized eruptive histiocytoma in an infant with healing in summer: long-term follow-up. Br J Dermatol 144: 435-437
  5. Muller SA et al (1967) Generalized eruptive histiocytoma. Enzymes histochemistry and electron microscopy. Arch Dermatol 96: 11-17
  6. Utikal J et al (2003) Cutaneous non-Langerhans' cell histiocytoses. J Dtsch Dermatol Ges 1: 471-491
  7. Winkelmann RK et al (1963) Generalized eruptive histiocytoma. A benign papular histiocytic reticulosis. Arch Dermatol 88: 586-596
  8. Winkelmann RK, Kossard S, Fraga S (1980) Eruptive histiocytoma of childhood. Arch Dermatol 116: 565-570
  9. Zelger BW et al (1996) Non-Langerhans cell histiocytoses. A new unifying concept. At J Dermatopathol 18: 490-504

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Please ask your physician for a reliable diagnosis. This website is only meant as a reference.

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Last updated on: 29.10.2020