Juvenile xanthogranuloma D76.3

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 29.10.2020

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Synonym(s)

giant cell granuloma juveniles; Juveniles giant cell granuloma; Naevoxanthoendothelioma; Naevoxanthoma; neviform xanthelasma; Peripheral giant cell granuloma; Xanthoma juvenile

History
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Adamson, 1905; Mc Donagh, 1909

Definition
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Benign, predominantly integumentary, less frequently systemic, self-healing, granulomatous disease of unclear etiology.

Occurrence/Epidemiology
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Etiopathogenesis
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Associations with neurofibromatosis type I or juvenile chronic myeloid leukemia are discussed.

Manifestation
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Predominantly begins within the first year of life (about 80% of cases)

Occurs in 5-17% of cases at birth.

Can (very rarely) occur in adulthood (20-30 years).

Localization
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Mostly head and trunk, more rarely extremity extension, oral mucosa, eyes (iris). Rarely infestation of internal organs (liver, lungs, spleen, pericardium, bones).

Clinical features
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Predominantly solitary, more rarely multilocular or disseminated, soft, elastic, initially red, later yellow or skin-coloured, usually completely asymptomatic papules, plaques or nodules (usually 1-3 cm Ø) with smooth or slightly wrinkled, scale-free surface.

Histology
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Epidermis flattened, no epidermotropism. Dense infiltrates in the papillary and reticular dermis consisting of histiocytes, foam cells and giant cells of the touton type. Focal lymphohistiocytic infiltrates as well as neutrophil and eosinophil granulocytes. Infiltration of deeper tissues such as fascia and muscles frequent (30-40% of cases).

Immunohistology: Stabilin-1(MS-1 HMWP), CD68 positive multinuclear cells and CD163 positive histiocytes (S100, CD1a negative).

Differential diagnosis
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Complication(s)
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If neurofibromatosis generalisata type I is present at the same time, the risk of developing juvenile chronic leukemia is 20-30 times higher.

Therapy
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Spontaneous regression possible between the ages of 1 and 6 years (40% of cases). Healing with residual pigmentation, on the capillitium possibly with alopecia.

Excision of single nodules if necessary, especially in case of disturbing localization (e.g. eye).

Alternative: treatment with CO 2 laser.

In case of rapid progression systemic glucocorticoids, e.g. prednisolone (e.g. Solu Decortin H) 10 mg/day p.o., especially in case of eye involvement to prevent glaucoma or loss of vision.

Progression/forecast
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Spontaneous regression between the ages of 1 and 6. Residual pigmentation. With eye involvement danger of blindness.

Literature
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  1. Adamson NF (1905) Congenital xanthoma multiplex in a child. Br J Dermatol 17: 222-223
  2. Mc Donagh JER (1909) Spontaneous disappearance of an endothelioma (nevo-xanthoma). Br J Dermatol 21: 254-256
  3. Dehen L et al (1990) Xanthogranuloma, type I neurofibromatosis and myelomonocytic leukemia with spontaneous regression. Ann Dermatol Venereol 117: 812-814
  4. Dehner LP (2003) Juvenile xanthogranulomas in the first two decades of life: a clinicopathologic study of 174 cases with cutaneous and extracutaneous manifestations. At J Surg Pathol 27: 579-593
  5. Ferrari F et al (2014) Juvenile xanthogranuloma and nevus anemicus in the diagnosis of neurofibromatosis type 1 JAMA Dermatol 150:42-46
  6. Hill H (2006) Fibrohistiocytic skin tumors. J Dtsch Dermatol Ges 4: 544-555
  7. Jung T (2000) Congenital manifestations of juvenile xanthogranuloma (large nodular form). dermatologist 51: 423-426
  8. Margulis A (2003) Juvenile xanthogranuloma invading the muscles in the head and neck: clinicopathological case report. Ann Plast Surgery 50: 425-428
  9. Müller P (1995) Juveniles xanthogranuloma. Z Hautkr 70: 73-74
  10. Torok E et al (1985) Juvenile xanthogranuloma: an analysis of 45 cases by clinical follow-up, light and electron microscopy. Acta Derm Venereol 65: 167-169
  11. Utikal J et al (2003) Cutaneous non-Langerhans' cell histiocytoses. J Dtsch Dermatol Ges 1: 471-491
  12. Wolff HH et al (1975) Juvenile xanthogranuloma and organ manifestations. Dermatologist 26: 268-272
  13. Zvulunov A et al (1995) Juvenile xanthogranuloma, neurofibromatosis, and juvenile chronic myelogenous leukemia. World statistical analysis. Arch Dermatol 131: 904-908

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Last updated on: 29.10.2020