Sweet's syndrome leading to acquired cutis laxa (Marshall's syndrome) in an infant with alpha 1-antitrypsin deficiency

Hwang, S. T.

doi:10.1001/archderm.131.10.1175

Cited by 21 publications

(30 citation statements)

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“…GSS may be classified under the general heading ‘cutis laxa’ (dermatochalasia), a group of elastolysis syndromes [39, 44]. The pathophysiology of most of these syndromes remains unknown.…”

Section: Discussionmentioning

confidence: 99%

“…The pathophysiology of most of these syndromes remains unknown. In acquired cutis laxa syndromes infiltrates in the skin may directly or indirectly affect the structure of elastic fibers [44]. …”

Section: Discussionmentioning

confidence: 99%

“…A third explanation is given by Hwang et al [44] who postulated that acquired cutis laxa in general and possibly syndromes such as MDE may be caused, at least in part, by α 1 -antitrypsin deficiency leading to a localized (or generalized) increase in granulocyte elastase activity. However, in GSS the presence of this enzyme has not yet been investigated.…”

Section: Discussionmentioning

confidence: 99%

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Granulomatous Slack Skin

et al. 1998

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Purpose: Granulomatous slack skin (GSS) is a rare cutaneous disorder characterized clinically by the evolution of circumscribed erythematous lax skin masses, especially in the body folds, and histologically by a granulomatous T-cell infiltrate and loss of elastic fibers. GSS is often associated with preceding or subsequent lymphoproliferative malignancies, especially mycosis fungoides (MF) and Hodgkin’s disease (HD). No effective treatment is known yet. Whether this entity is a benign disorder, a peculiar host reaction to a malignant lymphoma, a precursor of malignant lymphoma or an indolent cutaneous T-cell lymphoma (CTCL) in itself is still a matter of debate. Patients and Methods: The results of the patients with GSS from the Netherlands are compared with the cases reported in the world literature. Results: A female patient had had GSS for 8 years without developing a secondary malignancy. In a second female patient with a histologically confirmed diagnosis of MF, GSS developed 18 years later in the axillary and inguinal folds which had previously been affected by plaque-stage MF lesions. A third male patient with a 6-year history of erythematosquamous skin disease diagnosed as CTCL developed GSS. Moreover, granuloma formation was also found in a facial basal cell carcinoma, in a cervical lymph node and the spleen. Clonal rearrangements of the T-cell receptor β genes were found in the 2 female patients; the male patient could not be tested. Conclusion: GSS is a rare clinicopathological entity. Only 34 patients have been described so far. The development of GSS within plaque MF lesions has not been reported before. Our third case developed very extensive skin lesions and showed a strong propensity to develop granulomas as compared to cases reported before. The presence of a clonal T-cell population was demonstrated in all cases tested. Our cases support the idea that GSS is a very rare and rather indolent type of CTCL. Apparently, the disease is associated with a peculiar immune response, characterized by granuloma formation and disappearance of elastic fibers resulting in the lax skin. The relationship between GSS and other preexisting or subsequent lymphoproliferative diseases (diagnosed in approximately 50% of the cases) warrants a life-long follow-up.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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Granulomatous Slack Skin

et al. 1998

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“…Fibroblasts from acquired cutis laxa express elastolytic activity, unlike control skin fibroblasts or fibroblasts from inherited cutis laxa [22]. Dysfunction of elastase/protease inhibitors has also been reported in association with acquired cutis laxa [3, 23]. Elastases are a group of proteases produced by several cell types including polynuclear neutrophils, macrophages and fibroblasts.…”

Section: Discussionmentioning

confidence: 99%

“…Acquired cutis laxa differs from inherited cutis laxa which is more common and can be transmitted as an autosomal dominant, autosomal recessive or X-linked recessive trait [1]. Acquired cutis laxa has been reported de novo or following inflammatory skin conditions such as Sweet’s syndrome [2, 3], drug reactions [4]or urticarial eruption [5, 6, 7, 8, 9]. It has also been described in association with neoplasia such as myeloma [10]or lymphoma [11].…”

Section: Introductionmentioning

confidence: 99%

Increased Fibroblast Elastase Activity in Acquired Cutis laxa

Bouloc¹,

Godeau

Zeller³

et al. 1999

Dermatology

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Background: Acquired cutis laxa is a rare disease characterized by sagging skin, premature wrinkling and reduced skin elasticity. Observation: We report a 21-year-old woman, who presented with acquired cutis laxa on the face and the ear lobes. Urticarial papules had preceded for 6 years. There was no systemic involvement. Skin specimens were obtained from lax skin and urticarial papules, and from healthy controls. Histology showed only few perivascular lymphocytes in lax ear skin and a dense inflammatory infiltrate in urticarial skin. In both biopsies elastic fibres were decreased as demonstrated by computerized morphometric analyses. Elastase activities of fibroblasts in culture were evaluated. There was a 2- to 3-fold increase in elastase activity in urticarial skin fibroblasts, contrasting with a normal elastase activity in lax ear skin. Conclusion: Our findings suggest that the inflammatory cells could play a significant role in the destruction of elastic fibres.

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Generalized Acquired Cutis Laxa Associated With Multiple Myeloma With Biphenotypic IgG- λ and IgA-κ Gammopathy Following Treatment of a Nodal Plasmacytoma

New

Callen²

2011

Arch Dermatol

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Background: Cutis laxa is a rare dermatosis that can be inherited or acquired. The acquired form is rare and has been associated with various conditions, including multiple myeloma, monoclonal gammopathy of undetermined significance, and heavy chain deposition disease. Observations: We describe a 48-year-old man who developed generalized cutis laxa over a 4-year duration. There were no preceding skin changes except for a history of erythematous plaques with granuloma annulare-like features on his buttocks and lateral hips. He underwent treatment of an axillary lymph node plasmacytoma with surgery and radiation 4 years prior to his cutaneous changes and had been clinically monitored with a diagnosis of monoclonal gammopathy of unknown significance (MGUS). Cutaneous manifestations prompted a systemic evaluation demonstrating a persistent monoclonal IgG-M-spike on immunofixation electrophoresis and lytic bone lesions. He was later found to have biphenotypic IgG-and IgA-multiple myeloma. Conclusions: Multiple myeloma, plasma cell dyscrasia, and heavy-chain deposition disease have been very rarely reported to be associated with acquired cutis laxa (ACL). Findings in our patient support the hypothesis that paraproteinemia is a cause of ACL through immunologic destruction of elastic fibers manifesting as granuloma annulare-like plaques. Evaluation for an underlying gammopathy is essential for the workup of a patient with new-onset ACL.

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Sweet's syndrome leading to acquired cutis laxa (Marshall's syndrome) in an infant with alpha 1-antitrypsin deficiency

Cited by 21 publications

References 0 publications

Granulomatous Slack Skin

Granulomatous Slack Skin

Increased Fibroblast Elastase Activity in Acquired Cutis laxa

Generalized Acquired Cutis Laxa Associated With Multiple Myeloma With Biphenotypic IgG- λ and IgA-κ Gammopathy Following Treatment of a Nodal Plasmacytoma

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