Idiopathic chilblains in myelomonocytic leukemia: not a simple association

Nazzaro, Gianluca; Genovese, Giovanni; Marzano, Angelo Valerio

doi:10.1111/ijd.13896

Cited by 3 publications

(3 citation statements)

References 8 publications

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“…Similarly, the high rate of positive-result direct immunofluorescence showing vascular deposits of IgM, IgA, or C3 is an original finding. Indeed, direct immunofluorescence result was negative in a case of COVID-19eassociated chilblain, 17 showed a lupus band test in 21% of cases of autoimmune-related chilblains, 22 and showed nonspecific findings in cases of idiopathic chilblains associated with myelomonocytic leukemia 23 ; these studies did not mention specific vascular deposits.…”

Section: Discussionmentioning

confidence: 94%

Chilblain-like acral lesions during the COVID-19 pandemic (“COVID toes”): Histologic, immunofluorescence, and immunohistochemical study of 17 cases

Kanitakis

Lesort

Danset

et al. 2020

Journal of the American Academy of Dermatology

129

168

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Background: During the coronavirus disease 2019 pandemic, several acral chilblain-like lesions were observed in young patients with suspected, but mostly unconfirmed, infection with severe acute respiratory syndrome coronavirus 2. The histopathologic aspect of these lesions is as yet poorly known.Objective: To investigate the pathologic features of chilblain-like lesions.Methods: Biopsies were obtained from 17 cases of chilblain-like lesions during the coronavirus disease 2019 pandemic in France and were studied by routine histologic examination, immunohistochemistry, and direct immunofluorescence. The patients had suspected but unconfirmed infection with severe acute respiratory syndrome coronavirus 2 (negative nasopharyngeal polymerase chain reaction and serologic test results).Results: Chilblain-like lesions showed many features in common with those reported in idiopathic and autoimmune-related chilblains, including epidermal necrotic keratinocytes, dermal edema, perivascular and perieccrine sweat gland lymphocytic (predominantly CD3/CD4 1 ) inflammation, and frequent vascular changes (endothelialitis, microthromboses, fibrin deposition, and immunoreactant deposits on vessels).Conclusions: Chilblain-like lesions show histopathologic features similar to those of idiopathic and autoimmune-related chilblains, with a high rate of vascular changes and direct immunofluorescence positivity. The role of severe acute respiratory syndrome coronavirus 2 in the development of these puzzling lesions remains to be elucidated.

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Section: Discussionmentioning

confidence: 94%

Chilblain-like acral lesions during the COVID-19 pandemic (“COVID toes”): Histologic, immunofluorescence, and immunohistochemical study of 17 cases

Kanitakis

Lesort

Danset

et al. 2020

Journal of the American Academy of Dermatology

129

168

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“…In myelomonocytic leukemia, malignant myocytic cells and hypergammaglobulinemia may interfere with vascular microcirculation, inducing hyperviscosity and vascular stasis and promoting chilblain onset [36].…”

Section: Physiopathologymentioning

confidence: 99%

Chilblains

Nyssen

Benhadou

Magnée

et al. 2020

Vasa

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Summary: Idiopathic chilblain is a relatively common yet poorly recognized acrosyndrome. This literature review aims to better understand and draw attention to this disorder. Chilblain is a localized inflammation of the skin that occurs on exposure to cold but non-freezing wet weather. It usually resolves spontaneously. The etiology is uncertain, but vasospasm seems to play a role in this abnormal reaction to cold. Diagnosis is most often based on clinical presentation, but a skin biopsy can be useful in dubious cases. In histology, dermal edema and an inflammatory infiltrate are usually present. A distribution of the infiltrate particularly around the eccrine gland is typical. Systemic symptoms and underlying autoimmune disease should be screened. Avoiding cold and keeping extremities warm is the first recommendation for management, as well as smoking cessation. Calcium channel blockers (in particular nifedipine) seems to be the treatment that has been most evaluated in chilblains. However, their effectiveness is not confirmed by all studies. Topical betamethasone is often used but its effect has not been confirmed by randomized clinical trials. Other treatments, such as pentoxifylline, hydrochloroquine and topical nitroglycerin have shown positive effects only in a reduced number of patients. Acupuncture seems to bring a benefit.

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“…Some authors postulated three possible mechanisms for these lesions: (1) The presence of malignant monocytic cells, because of their rigidity, large diameter and difficulty in passing through the lumen may cause modifications in vascular flow in the microcirculation; (2) The polyclonal activation of B lymphocytes with polyclonal hypergammaglobulinemia with autoantibodies secretion; and finally (3) The role of cold, which has an influence on blood viscosity [82]. The immunohistochemical demonstration of the lymphoid nature of the infiltrate and ruling out specific leukemic infiltrates allows the histopathological differential diagnosis of the lesions of chilblain-like leukemia cutis described before [83].…”

Section: Other Cutaneous Disorders In Mds/mpn Patients 41 Cutaneous M...mentioning

confidence: 99%

Clinical, Histopathological and Molecular Spectrum of Cutaneous Lesions in Myelodysplastic Syndrome and Myeloproliferative Neoplasms (MDS/MPN): An Integrative Review

Prieto-Torres,

Requena,

Rodríguez-Pinilla

2023

Cancers

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Myeloid neoplasms and acute leukemias include different entities that have been recently re-classified taking into account molecular and clinicopathological features. The myelodysplastic syndrome/myeloproliferative neoplasm (MDS/MPN) category comprises a heterogeneous group of hybrid neoplastic myeloid diseases characterized by the co-occurrence of clinical and pathological features of both myelodysplastic and myeloproliferative neoplasms. The most frequent entity in this category is chronic myelomonocytic leukemia (CMML) which is, after acute myeloid leukemia (AML), the main myeloid disorder prone to develop cutaneous manifestations. Skin lesions associated with myelodysplastic and myeloproliferative neoplasms include a broad clinical, histopathological and molecular spectrum of lesions, poorly understood and without a clear-cut classification in the current medical literature. The aim of this review is to describe and classify the main clinical, histopathological and molecular patterns of cutaneous lesions in the setting of MDS/MPN in order to improve the diagnostic skills of the dermatologists, hematologists and pathologists who deal with these patients.

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Idiopathic chilblains in myelomonocytic leukemia: not a simple association

Cited by 3 publications

References 8 publications

Chilblain-like acral lesions during the COVID-19 pandemic (“COVID toes”): Histologic, immunofluorescence, and immunohistochemical study of 17 cases

Chilblain-like acral lesions during the COVID-19 pandemic (“COVID toes”): Histologic, immunofluorescence, and immunohistochemical study of 17 cases

Chilblains

Clinical, Histopathological and Molecular Spectrum of Cutaneous Lesions in Myelodysplastic Syndrome and Myeloproliferative Neoplasms (MDS/MPN): An Integrative Review

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