Preceding Annular Skin Lesions in a Patient with Hemophagocytic Lymphohistiocytosis

Jun, Hee-Jin; Kim, Hyung Ok; Lee, Jun Young; Park, Young Min

doi:10.5021/ad.2015.27.5.608

Cited by 10 publications

(11 citation statements)

References 7 publications

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“…HLH, on its own, has been found in up to 20% of haematological malignancies 8. While evidence of HLH can be seen histologically, a recent case report of an HLH presentation with multiple annular hyperpigmented plaques,9 identical to those seen on our patient, would suggest that cutaneous manifestations of HLH may also be present and should not be missed. Our experience has demonstrated that SPTCL should be kept as part of the panel of rare panniculitis mimics and should be considered in refractory cases of panniculitis, more so in patients with evidence of HLH.…”

Section: Discussionsupporting

confidence: 63%

A case of deadly panniculitis

Yuson¹,

Maiolo

Hissaria

2018

BMJ Case Reports

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While the majority of panniculitides are benign in nature, there are rare instances when panniculitis presents as the initial sign of a complex disease state. We describe a case of panniculitis initially diagnosed as lupus profundus that was challenged when features of haemophagocytic lymphohistiocytosis became apparent. We illustrate how some key clinical features and newer investigations can help differentiate between benign and malignant panniculitis.

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Section: Discussionsupporting

confidence: 63%

A case of deadly panniculitis

Yuson¹,

Maiolo

Hissaria

2018

BMJ Case Reports

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“…6,7,11,13,15,17,19,20 In addition, spongiosis, hyperkeratosis, dying keratinocytes, vacuolization at the dermoepidermal junction, and tuberculoid granulomas can also be seen on the histologic sections. 7,[10][11][12]17 These findings can present at the time of HLH onset, yet preceding isolated skin lesions are rarely reported. 7,10 Similar to our case, Brinkmeier et al 7 reported Ebstein-Barr-Virus-associated HLH that initially presented with hyperkeratotic granulomatous nodules.…”

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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“…However, skin eruption can be an important clinical feature in patients with HLH. According to the literature, 6–65% of patients with HLH have skin involvement during the course of disease [ 3 , 5 , 24 ]. Also in MAS/HLH, skin findings have been reported [ 7 , 25 , 26 ].…”

Section: Discussionmentioning

confidence: 99%

Macrophage activation syndrome in a newborn: report of a case associated with neonatal lupus erythematosus and a summary of the literature

et al. 2021

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Background Macrophage activation syndrome (MAS) is a life-threatening hyperinflammatory syndrome and is caused by a severely dysregulated immune response. It has rarely been associated with neonatal lupus. Case presentation We present a female neonate with MAS born to a mother who had cutaneous lupus erythematosus with circulating anti-nuclear antibodies (ANA), anti-SSA, anti-SSB and anti-extractable nuclear antigen (anti-ENA) antibodies. Because of neonatal lupus (NLE) with a total atrioventricular block, epicardial pacemaker implantation was required on the sixth day of life. Following surgery she developed non-remitting fever and disseminated erythematous skin lesions. A diagnosis of MAS was made based on these symptoms, with hyperferritinemia, elevated transaminases, hypertriglyceridemia, and a skin biopsy that showed hemophagocytosis. Our patient was treated with steroids for 3 months with good effect. No relapse has occurred. Conclusions MAS is a rare complication of neonatal lupus that may be difficult to diagnose, but needs to be treated promptly. In this article, pathogenesis and overlap of MAS and hemophagocytic lymphohistiocytosis (HLH) has been described. Diagnosis of MAS can be difficult. Different diagnostic criteria are used in both diagnosing MAS and HLH. Validated criteria for diagnosis of MAS in other disease than systemic onset JIA have not been validated yet. In NLE, diagnosing MAS is even more difficult, since skin lesions are already common in NLE. We show the potential additional value of skin biopsy in diagnosing MAS.

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Preceding Annular Skin Lesions in a Patient with Hemophagocytic Lymphohistiocytosis

Cited by 10 publications

References 7 publications

A case of deadly panniculitis

A case of deadly panniculitis

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

Macrophage activation syndrome in a newborn: report of a case associated with neonatal lupus erythematosus and a summary of the literature

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