Cutaneous infiltrates – non-lymphoid

Weedon, David

doi:10.1016/b978-0-7020-3485-5.00041-3

Cited by 18 publications

(40 citation statements)

References 1,042 publications

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“…The CD68+, S-100+, and CD1a2 immunophenotype of the infiltrating cells in our case characterizes also the phagocytic cells present in RosaiDorfman disease (RDD) in which the cells may also demonstrate emperipolesis. 15 602782), which is an autosomal recessively inherited form of histiocytosis with similarities to RDD; and in 2 families reported to have familial RDD. This may explain the immunophenotype similarity to RDD in our H syndrome case.…”

Section: Discussionmentioning

confidence: 98%

A Case of H Syndrome Showing Immunophenotye Similarities to Rosai–Dorfman Disease

Avitan‐Hersh¹,

Mandel²,

Indelman³

et al. 2011

The American Journal of Dermatopathology

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H syndrome (OMIM 612391) is a recently described autosomal recessive genodermatosis characterized by indurated, hyperpigmented, and hypertrichotic skin and systemic manifestations including hepatosplenomegaly, cardiac anomalies, hearing loss, hypogonadism, low height, hypertriglyceridemia, hallux valgus, and flexion contractures. H syndrome results from mutations in the SLC29A3 gene, which encodes the human equilibrative nucleoside transporter hENT3. The cutaneous histopathology is characterized by a striking mononuclear cell infiltrate in the dermis consisting of CD68+ monocyte-derived cells and CD34+ and factor XIIIa+ dendrocytes. We describe a case of H syndrome in which the infiltrating mononuclear cells were CD68+, CD163+, S-100+, and CD1a-, thus simulating the immunophenotype observed in Rosai-Dorfman disease (RDD). The immunostaining for CD21, fascin, and CD34 were negative, and there were also many factor XIIIa+ dendrocytes interspersed within the dense mononuclear cell infiltrate. Recent findings of biallelic mutations in SLC29A3 in 2 families reported to have familial RDD and in a kindred with Faisalabad histiocytosis (OMIM 602782), which is an autosomal inherited form of histiocytosis with similarities to RDD, may explain the RDD-like immunophenotype in our H syndrome case.

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Section: Discussionmentioning

confidence: 98%

A Case of H Syndrome Showing Immunophenotye Similarities to Rosai–Dorfman Disease

Avitan‐Hersh¹,

Mandel²,

Indelman³

et al. 2011

The American Journal of Dermatopathology

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“…Melanosit kökenini göstermenin en kesin yolu da immünohistokimyadır. HMB-45 ve Melan-A, sensitivitesi oldukça yüksek melanositik markerlardır (1).…”

Section: Olgu Sunumuunclassified

“…Plak ve tümör evrelerinde ise, epidermis içinde yuvalar oluşturmuş atipik lenfositlerin yanı sıra, dermiste de belirgin lenfoidhücre popülasyonu görülür. İmmünhistokimyasal olarak yapılan T hücre belirteçleri (CD3, CD5 gibi) ayırıcı tanıda önemlidir (1).…”

Section: Olgu Sunumuunclassified

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Bes Aylik Bebekte Deri Tutulumu ile Saptanan Langerhans Hücreli Histiyositoz: Olgu Sunumu

Tepeoğlu¹

2013

gmj

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“…2 Xanthomas, on the contrary, correspond to the deposit of lipids in macrophages as a secondary event 1 and can be classified according to clinical features and association with dyslipidaemia. The papular xanthoma (PX) first described by Winkelmann 3 has features of both a secondary and primary macrophagic disorder and therefore its place among the xanthomatosis or the n-LCD histiocytosis is not completely clarified.…”

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confidence: 99%