Juvenile xanthogranuloma D76.3

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 27.09.2023

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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.

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Occurrence/Epidemiology
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Most common disease within the group of juvenile non-Langerhans cell histiocytoses.

Associations with peripheral neurofibromatosis (NF type I) or juvenile chronic myeloid leukemia have been described.

In up to 50% of patients with NF type I, a juvenile xanthogranuloma gfls. together with a N. anaemicus is found in addition to the typical café-au-lait spots (Ferrari F et al. 2014).

In 40-70% of patients, skin lesions appear already in the 1st year of life.

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-colored, usually completely asymptomatic papules, plaques or nodules (usually 1-3 cm Ø) with a smooth or slightly wrinkled, scale-free surface. The isolated giant xanthogranuloma may grow larger than 5 cm.

JXG occurs with higher than average frequency (in about 3% of cases) together with type I neurofibromatosis (NF1) (Miraglia E et al. 2022).

Histology
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Epidermis flattened, no epidermotropism. Dense infiltrates in papillary and reticular dermis consisting of histiocytes, foam cells, and giant Touton-type cells. Focal lymphohistiocytic infiltrates and neutrophilic and eosinophilic granulocytes. Infiltration of deeper tissues such as fascia and muscle common (30-40% of cases).

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

Differential diagnosis
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Tuberous xanthomas: adult onset.

Cutaneous mastocytoma: Analogous clinical picture. Darier's sign positive.

Urticaria pigmentosa xanthelasmoidea.

Langerhans cell histiocytosis (eosinophilic granuloma): mostly osteolytic bone lesions; skin rarely affected; histology is demonstrative.

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. Am 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. Hügel 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 Surg 50: 425-428.
  9. Miraglia E et al (2022) Juvenile xanthogranuloma in neurofibromatosis type 1. Prevalence and possible correlation with lymphoproliferative diseases: experience of a single center and review of the literature. Clin Ter 173:353-355.
  10. Müller P (1995) Juvenile xanthogranuloma. Z Hautkr 70: 73-74
  11. 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
  12. Utikal J et al (2003) Cutaneous non-Langerhans' cell histiocytoses. J Dtsch Dermatol Ges 1: 471-491
  13. Wolff HH et al (1975) Juvenile xanthogranuloma and organ manifestations. Dermatol 26: 268-272
  14. 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: 27.09.2023