Panniculitis associated with severe alpha 1-antitrypsin deficiency. Treatment and review of the literature

Smith, Kevin

doi:10.1001/archderm.123.12.1655

Cited by 40 publications

(46 citation statements)

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“…The drug has been especially useful in patients who do not respond to other medication, including in those with associated alpha-1-antitrypsin deficiency. [17][18][19][20] Of note is that panniculitis in alpha-1-antitrypsin deficiency is usually observed in severe forms of the disease and such patients would also present symptoms related to lung and/or liver involvement. 1,4,19 No such symptoms were noted in our patient, who underwent no formal work-up for alpha-1-antitrypsin deficiency after her positive response to corticosteroids.…”

Section: Discussionmentioning

confidence: 99%

“…[17][18][19][20] Of note is that panniculitis in alpha-1-antitrypsin deficiency is usually observed in severe forms of the disease and such patients would also present symptoms related to lung and/or liver involvement. 1,4,19 No such symptoms were noted in our patient, who underwent no formal work-up for alpha-1-antitrypsin deficiency after her positive response to corticosteroids. Considering current treatment options, it should be also mentioned that a standardized therapy protocol has yet to be established and this might be related to developing a universally accepted consensus about the diagnostic criteria, disease staging, and classification.…”

Section: Discussionmentioning

confidence: 99%

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Nonsuppurative Nodular Panniculitis of the Breast

Rotaru

Punga

Codreanu

et al. 2015

Clinical Breast Cancer

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Nonsuppurative Nodular Panniculitis of the Breast

Rotaru

Punga

Codreanu

et al. 2015

Clinical Breast Cancer

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“…In summarizing possible pathogenetic mechanisms of panniculitis in AAT deficiency, Smith et al [26] have proposed several mechanisms: (1) insufficient inhibition of membrane-bound serine proteases; (2) increased elastin degradation promoted by the large amounts of available fatty acids; (3) insufficient inhibition of complement activation; (4) neutrophil accumulation at sites of inflammation that may result in the release of serine proteases with subsequent damage to surrounding connective tissue structures; (5) oxidation of the active site of the AAT molecule by myeloperoxidase which reduces antiprotease activity. Keeping in mind that skin biopsies from our patient with AAT deficiency-associated panniculitis showed, for the first time, the co-accumulation of neutrophils and Z AAT polymers (fig.…”

Section: Discussionmentioning

confidence: 99%

New Findings in PiZZ α<sub>1</sub>-Antitrypsin Deficiency-Related Panniculitis

et al. 2009

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Panniculitis is a recognized but unusual complication of a severe deficiency of α₁-antitrypsin (AAT), with fewer than 100 cases described to date. Like the pathogenesis of emphysema in severe PiZZ deficiency of AAT, panniculitis has been hypothesized to be an inflammatory process, possibly related to Z AAT polymer formation and to an unopposed anti-inflammatory screen in the context of deficient serum levels of AAT. The current report presents a 31-year-old woman with PiZZ AAT deficiency-associated panniculitis. Our case extends current knowledge of AAT-associated panniculitis in 2 ways: (1) we demonstrate Z-type AAT polymers in the skin, which supports the inflammatory pathogenesis of panniculitis and the potential pro-inflammatory role of polymers; (2) we show that a high dose and long-term use of intravenous augmentation therapy (90 mg/kg body weight once weekly during 3 years) can ameliorate the frequency and severity of panniculitis associated with AAT deficiency.

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“…The lesions typically contain an abundance of neutrophilic aggregates between collagen bundles in the reticular dermis, with edema and elastolysis, and adjacent to necrotic fat lobules and septa. [23][24][25][26] The differentiating histopathologic features from other forms of inflammatory panniculitis are the presence of large areas of normal fat adjacent to necrotic areas that contain intense polymorphonuclear and macrophage infiltration.…”

Section: Discussionmentioning

confidence: 99%

Fatal Subcutaneous Panniculitis-Like T-Cell Lymphoma γ/δ Subtype (Cutaneous γ/δ T-Cell Lymphoma): Report of a Case and Review of the Literature

Kao¹,

Resh

McMahon

et al. 2008

The American Journal of Dermatopathology

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Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is a distinct type of peripheral T-cell lymphoma, which has gained recognition over the past decade. The disease presents complex clinical, pathologic, and immunohistochemical features, which warrant awareness by dermatologists, dermatopathologists, hematopathologists, hematologists, oncologists, and internists. SPTCL was initially included as a provisional entity in the Revised European-American Lymphoma classification, followed by the European Organization for Research and Treatment of Cancer classification as a primary cutaneous lymphoma, and subsequently as a distinct entity by the World Health Organization classification. It is known that patients diagnosed with SPTCL usually respond poorly to therapy, and the tumor progresses aggressively. Data from recent studies in a series of cases of SPTCL by the European Organization for Research and Treatment of Cancer Cutaneous Lymphoma Group have further identified SPTCL as a heterogeneous disease entity, which comprises an alpha/beta subtype (SPTCL-AB) and a gamma/delta subtype (SPTCL-GD). The latter has recently been included in the entity of "cutaneous gamma/delta T-cell lymphoma" by the World Health Organization, Pathology and Genetics of Skin Tumours. The clinical, histologic, and immunophenotypic data, treatment, and prognosis, appear different in the 2 subtypes of SPTCL. We report a case of fatal SPTCL-GD (cutaneous gamma/delta T-cell lymphoma), with detailed clinicopathologic features, immunohistochemical studies, treatment, and clinical course. In view of its aggressive behavior, identification of this disease is critical for proper management and treatment.

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Panniculitis associated with severe alpha 1-antitrypsin deficiency. Treatment and review of the literature

Cited by 40 publications

References 0 publications

Nonsuppurative Nodular Panniculitis of the Breast

Nonsuppurative Nodular Panniculitis of the Breast

New Findings in PiZZ α<sub>1</sub>-Antitrypsin Deficiency-Related Panniculitis

Fatal Subcutaneous Panniculitis-Like T-Cell Lymphoma γ/δ Subtype (Cutaneous γ/δ T-Cell Lymphoma): Report of a Case and Review of the Literature

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