Abt-letterer-siwe disease C96.0

Last updated on: 29.10.2020

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Synonym(s)

acute disseminated juvenile form; acute disseminated Langerhans cell histiocytosis; acute disseminated LCD; Acute reticuloendotheliosis; aleukemic reticulosis; Histiocytosis X; Histiocytosis X acute disseminated juvenile form; Letterer-Siwe disease; Letterer-Siwe M.; Reticuloendotheliosis acute; Reticulosis aleukemic

History
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Department and Letterer, 1924; Siwe, 1933

Definition
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Acute, generalized, malignant course of Langerhans cell histiocytosis in early childhood.

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Occurrence/Epidemiology
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The incidence of LCH in childhood is 2.0-5/1,000,000 children. f:m=1.8:1.0

Etiopathogenesis
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  • Unknown; family cases with autosomal recessive inheritance have been described.
  • Pathogenetic: Proliferation of dendritic cells (Langerhans cells) with expression of CD1 antigen, S-100 protein and neuron-specific endase (NSE).

Manifestation
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Mostly infants and toddlers, rarely adults.

Localization
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Preferably seborrhoeic zones: hairy head, auditory canals, nasolabial and perioral region, upper and middle parts of the trunk, especially in the area of the welding channels.

Clinical features
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  • Integument: Eruption of disseminated small, flat, yellow-brownish papules with scaly to crusty surface. Tendency to necrotic disintegration and petechial haemorrhages, haemorrhagic eczematoid, sometimes ulcerated lesions, especially capillitium, intertriginous spaces, joint folds affected; aphthous lesions of the oral mucosa; thrombocytopenic purpura.
  • Extracutaneous manifestation: Destructive bone foci (extremities, ribs, skull, iliac crest), scarring. Obligatory swelling of lymph nodes, hepatosplenomegaly, fever, increasing anemia. Optional pulmonary involvement (cough, dyspnoea, radiological miliary speckling), osteolysis.

Laboratory
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Leukocytosis or leukopenia, thrombopenia, hypochromic anemia.

Histology
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  • Dense subepidermal infiltrate of large, atypical histiocytoid cells with kidney-shaped nuclei as well as lymphocytes and possibly eosinophil granulocytes with distinct epidermotropism. Immunohistochemically, the cells react with S100, CD1a, and Langerin-CD207.
  • Electron microscopy: detection of Langerhans cell granules (tennis racket-like Birbeck granules)

Differential diagnosis
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External therapy
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Symptomatic, e.g. with lotio zinci, topical glucocorticoids.

Internal therapy
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Cytostatic agents in combination with high-dose glucocorticoids, antibiotics, blood transfusions, vitamins.

Progression/forecast
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The more acute the start and the more extensive the internal co-participation, the worse the prognosis. Untreated lethal, long-term remissions possible with cytostatic therapy.

Literature
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  1. Abbot AF, Denenholz EJ (1936) Letterer-Siwe disease. On J Dis Child 51: 499-522
  2. Feyrter F (1955) On the relationships between Abt-Letterer-Siweschen disease, eosinophilic bone granuloma (eosinophilic granulomatosis) and Hand-Schüller-Christian disease. Medical, Stuttgart: 1019-1025
  3. Letterer E (1924) Aleukemic reticulosis. A contribution to the proliferative diseases of the reticuloendothelial apparatus. Frankf Zschr Path 30: 377
  4. Seward JL et al (2004) Generalized eruptive histiocytosis. J Am Acad Dermatol 50: 116-120
  5. Siwe SA (1933) Reticuloendotheliosis, a new clinical picture among hepatosplenomegaly Zschr Pediatrics 55: 212
  6. Vade A, Hayani A, Pierce KL (1993) Congenital histiocytosis X. Pediatric radiol 23: 181-182
  7. Wolff HH, Janka GE (1978) Abt-Letterer-Siwe disease. For diagnosis and therapy. Mschr pediatrics 126: 425-430
  8. Willman CL et al (1994) Langerhans-cell histiocytosis (histiocytosis X)--a clonal proliferative disease. N Engl J Med 331: 154-160

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Last updated on: 29.10.2020