Role of Skin Biopsies in the Diagnosis of Hemophagocytic Lymphohistiocytosis

Santos-Arroyo, Aileen E.; Barrera-Llaurador, Julián; Sánchez, Julio E.; Martín‐García, Rafael F.; Sánchez, Jorge

doi:10.1097/dad.0000000000000825

Cited by 9 publications

(6 citation statements)

References 21 publications

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“…Measurement of sCD163, the haptoglobin-hemoglobin scavenger receptor and a marker of macrophage activation, has been examined in several studies of MAS and HLH ( 32 – 34 ). CD163 expression was reported on hemophagocytic macrophages in the skin of patients with HLH, and high serum levels have been noted in patients with HLH and MAS.…”

Section: Resultsmentioning

confidence: 99%

Macrophage Activation Marker Soluble CD163 Associated with Fatal and Severe Ebola Virus Disease in Humans1

McElroy¹,

Shrivastava-Ranjan²,

Harmon³

et al. 2019

Emerg. Infect. Dis.

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Ebola virus disease (EVD) is associated with elevated cytokine levels, and hypercytokinemia is more pronounced in fatal cases. This type of hyperinflammatory state is reminiscent of 2 rheumatologic disorders known as macrophage activation syndrome and hemophagocytic lymphohistiocytosis, which are characterized by macrophage and T-cell activation. An evaluation of 2 cohorts of patients with EVD revealed that a marker of macrophage activation (sCD163) but not T-cell activation (sCD25) was associated with severe and fatal EVD. Furthermore, substantial immunoreactivity of host tissues to a CD163-specific antibody, predominantly in areas of extensive immunostaining for Ebola virus antigens, was observed in fatal cases. These data suggest that host macrophage activation contributes to EVD pathogenesis and that directed antiinflammatory therapies could be beneficial in the treatment of EVD.

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Section: Resultsmentioning

confidence: 99%

Macrophage Activation Marker Soluble CD163 Associated with Fatal and Severe Ebola Virus Disease in Humans1

McElroy¹,

Shrivastava-Ranjan²,

Harmon³

et al. 2019

Emerg. Infect. Dis.

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“…Skin biopsy has been investigated as a potential diagnostic tool for HLH [8]. Hemophagocytosis is the histopathological hallmark but is rarely observed in skin biopsy specimens and it was not observed in this case [9,10].…”

Section: Discussionmentioning

confidence: 90%

Cutaneous Manifestations in a Patient With Reactive Hemophagocytic Lymphohistiocytosis

Dourra

Mussad

Singer

2020

Cureus

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Hemophagocytic lymphohistiocytosis (HLH) is a rare hematologic disease caused by a disordered immune system. We present a case of reactive HLH (RHLH) with uncommon skin findings in a 35-year-old African American female with a history of hidradenitis suppurativa and morbid obesity. Skin findings on physical exam revealed bullous, ecchymotic, hypopigmented, and ulcerated lesions. The literature on the cutaneous lesions of RHLH is limited. Skin findings are an underrecognized feature of RHLH and can help alert to its presence or its recurrence after treatment.

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“…[10][11][12][13][14][15][16][17] Histologic findings are nonspecific and rarely demonstrate hemophagocytosis, which was not present in the skin biopsies from our patient. 11,18,19 Interestingly, clinically different primary eruptions may be similar histologically. 11 Prominent perivascular and interstitial lymphohistiocytic infiltrate is the most common histologic finding.…”

Section: Discussionmentioning

confidence: 99%

A Young Boy With Hemophagocytic Lymphohistiocytosis Presenting With Vaccine-Related Granulomatous Dermatitis: A Case Report and Literature Review

Zengin

Reyes-Barron

Cusick

et al. 2021

The American Journal of Dermatopathology

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Cutaneous eruptions associated with hemophagocytic lymphohistiocytosis (HLH) have been reported in 6%-63% of patients. Clinical findings of these skin lesions vary widely and include maculopapular rashes, ulcers, and violaceous nodules. Corresponding histologic findings are also variable and are considered nonspecific. We report the case of a 4-year-old boy who initially developed a widespread popular-pustular rash 2 weeks after his 12month measles, mumps, and rubella vaccinations. These resolved with scarring then recurred following his 24-month vaccinations. Multiple skin biopsies were negative for infectious organisms and showed a granulomatous infiltrate with perforation and necrobiosis. The differential diagnosis included perforating granuloma annulare, infection, or rheumatoid nodules. At the age of 4, he developed fever, hepatosplenomegaly, pancytopenia and other laboratory abnormalities, requiring hospitalization. A number of studies were performed including biopsies of bone marrow and liver. Molecular testing revealed 2 mutations in UNC13D known to be associated with familial HLH. His prior cutaneous lesions were likely caused by immune dysregulation exacerbated by immunizations because of underlying familial HLH. This case illustrates the importance of recognizing an unusual cutaneous manifestation of a rare disease to arrive at an earlier diagnosis in a pediatric patient. Although cutaneous eruptions usually develop concurrently with other systemic symptoms of HLH, preceding unusual skin lesions may be the first indication of this rare disease.

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Role of Skin Biopsies in the Diagnosis of Hemophagocytic Lymphohistiocytosis

Cited by 9 publications

References 21 publications

Macrophage Activation Marker Soluble CD163 Associated with Fatal and Severe Ebola Virus Disease in Humans1

Macrophage Activation Marker Soluble CD163 Associated with Fatal and Severe Ebola Virus Disease in Humans1

Cutaneous Manifestations in a Patient With Reactive Hemophagocytic Lymphohistiocytosis

A Young Boy With Hemophagocytic Lymphohistiocytosis Presenting With Vaccine-Related Granulomatous Dermatitis: A Case Report and Literature Review

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