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Case Report/Case Series |

Acquired Cutis Laxa Associated With Heavy Chain Deposition Disease Involving Dermal Elastic Fibers ONLINE FIRST

John T. O’Malley, MD, PhD1; Vivette D. D’Agati, MD2; William H. Sherman, MD3; Marc E. Grossman, MD1
[+] Author Affiliations
1Department of Dermatology, Columbia University Medical Center, New York, New York
2Department of Pathology, Columbia University Medical Center, New York, New York
3Department of and Medicine, Columbia University Medical Center, New York, New York
JAMA Dermatol. Published online August 13, 2014. doi:10.1001/jamadermatol.2014.725
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Importance  Acquired cutis laxa is a rare cutaneous manifestation of hematologic malignancy. We report a case of γ heavy chain deposition disease (HCDD) associated with acquired cutis laxa, renal involvement, and hypocomplementemia and propose a mechanism of elastic fiber degradation in the skin of this patient with HCDD.

Observations  To determine the localization of immunoglobulin heavy chains and complement activation in the skin of a patient with HCDD, we examined her skin biopsy specimens under light and electron microscopy. Analysis demonstrated the deposition of γ heavy chain and complement components C1q and C3 on the surfaces of dermal elastic fibers, indicating complement fixation by the deposited heavy chains. Electron microscopy revealed finely granular electron-dense deposits coating the surfaces of frayed dermal elastic fibers.

Conclusions and Relevance  The pathogenesis of cutis laxa in this condition is poorly understood. We hypothesize a mechanism of elastic tissue destruction by complement fixation with resultant activation of the complement cascade ultimately causing elastolysis. Based on our findings and those of other reports, we propose that skin heavy chain deposition can serve as a marker of plasma cell secretory activity in HCDD, although further studies are needed.

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Figure 1.
Patient’s Clinical Appearance Consistent With Cutis Laxa, But Histopathologic Analysis Did Not Demonstrate Abnormalities in the Elastic Fibers

A, Markedly aged appearance of patient compared with her 2 sisters, who are 7 (left) and 9 (right) years younger. B, Upper arm biopsy specimen showing a normal dermis (hematoxylin-eosin, original magnification ×20). C, No obvious abnormalities in the distribution or size of the dermal elastic fibers (Orcein stain, original magnification ×20).

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Figure 2.
Immunofluorescence Demonstrates γ Heavy Chain Deposition and Activated Complement Colocalizing Around Elastic Fibers

A-D, The IgG heavy chain panels show strong staining for γ heavy chain (IgG) at low power (A, original magnification ×200) and high (B, original magnification ×400), colocalizing around the dermal elastic fibers, blood vessel walls (C, original magnification ×400), and eccrine glands (D, original magnification ×400). E and F, In the complement images, there are codeposits of complement components C1q (E, original magnification ×200) and C3c (F, original magnification ×400) on dermal elastic fibers.

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Figure 3.
Electron Microscopy Shows Fragmented Dermal Elastic Fibers Intermingled With Collagen Fibers

A, At low power (original magnification ×4000), electron-dense deposits are seen surrounding the periphery of fragmented dermal elastic fibers (arrowheads). B, At higher power (original magnification ×15 000), a layer of electron-dense granular deposits is evident on the surface of the elastic fibers, with sparing of the adjacent banded collagen fibers. C, At highest power (original magnification ×50 000), the surfaces of the elastic fibers appear frayed and irregular.

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