A Singular Case of Congenital Self-healing Histiocytosis with Skin, Liver and Atypical Eye Involvement

Parentin, Fulvio; Ventura, Giovanna; Pastore, Serena; Kiren, Valentina; Bibalo, Chiara; Pensiero, Stefano; Lepore, Loredana

doi:10.3109/09273948.2010.605538

Cited by 4 publications

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“…1-3 Four clinical subtypes, which share significant clinical overlap, are known: Letterer-Siwe disease, Hand-Schüller-Christian disease, eosinophilic granuloma and congenital self-healing reticulohistiocytosis (CSHRH) or Hashimoto-Pritzker disease. 1,4,5 Histopathological and immunohistochemical studies are essential for diagnosis showing a dermal infiltrate with predominance of large, round histiocytic cells with dense eosinophilic cytoplasm with eccentric, reniform nuclei and Langerhans cells stain positive for S100 and CD1a (Figure 3A). 1-8 …”

Section: Discussionmentioning

confidence: 99%

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Case for diagnosis

Zanuncio¹,

Carvalho²,

Guedes³

et al. 2013

An. Bras. Dermatol.

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Langerhans cell histiocytosis is a rare, clinically heterogeneous desease. Since there is considerable clinical overlap among the four described variants (Hand-Schüller-Christian, eosinophilic granuloma, Letterer-Siwe and Hashimoto-Pritzker), the concept of spectral disease applies to this entity. The Hashimoto-Pritzker variant was first described in 1973. Characteristically, it is present at birth or during the first days of life, impairment is limited to the skin and prognosis is favorable with spontaneous resolution. We report a newborn male patient with Hashimoto-Pritzker disease presenting as a S100 + and CD1a + single congenital perianal lesion with rapid involution in two months.

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Section: Discussionmentioning

confidence: 99%

“…4-5 Development of lesions in adulthood, as well as pulmonary and ocular involvement, are extremely rare. 9 CSHRH may eventually show multisystem recurrence with considerable increase in morbidity and mortality.…”

Section: Discussionmentioning

confidence: 99%