Abt-letterer-siwe disease C96.0

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 22.10.2022

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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.

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; familial 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 hemorrhages, hemorrhagic-eczematoid, sometimes ulcerative skin lesions, especially capillitium, intertriginous spaces, articular folds affected; aphthous lesions of oral mucosa; thrombocytopenic purpura.

Extracutaneous manifestation: Destructive bone foci (extremities, ribs, skull, iliac blades), scarring. Obligate lymph node swelling, hepatosplenomegaly, fever, increasing anemia. Optional pulmonary involvement (cough, dyspnea, radiographic miliary mottling), 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 renal nuclei as well as lymphocytes and possibly eosinophilic granulocytes with marked 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. Abt AF, Denenholz EJ (1936) Letterer-Siwe disease. Am J Dis Child 51: 499-522.
  2. Feyrter F (1955) On the relations between Abt-Letterer-Siwe disease, eosinophilic granuloma of bone (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. Katayama S et al (2022) An Uncommon Pediatric Rash: Langerhans Cell Histiocytosis. The Journal of Pediatrics 243: 233-234.

  5. Merglová V et al. (2014)Langerhans cell histiocytosis in childhood - Review, symptoms in the oral cavity, differential diagnosis and report of two cases. Journal of Cranio-Maxillofacial Surgery 42: 93-100.
  6. Seward JL et al (2004) Generalized eruptive histiocytosis. J Am Acad Dermatol 50: 116-120.
  7. Siwe SA (1933) Reticuloendotheliosis, a new clinical picture among hepatosplenomegaly. Zschr Pediatr 55: 212
  8. Vade A, Hayani A, Pierce KL (1993) Congenital histiocytosis X. Pediatr Radiol 23: 181-182.
  9. Widodo I et al (2020) Case series of cutaneousLangerhans cell histiocytosis in Indonesian children; The clinicopathological spectrum. Dermatology Reports 12:8777
  10. Willman CL et al (1994) Langerhans cell histiocytosis (histiocytosis X)--a clonal proliferative disease. N Engl J Med 331: 154-160
  11. Wolff HH, Janka GE (1978) Abt-Letterer-Siwe's disease. On diagnosis and therapy. Mschr Pediatr 126: 425-430.


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Last updated on: 22.10.2022