Calcific elastosis in the setting of weight gain
Authors: Monica Enamandram, Kathryn Schoch, Daniel D. Miller, Thomas D. Horn
Journal: Journal of the American Academy of Dermatology (2014)

To the Editor: A 51-year-old man presented for evaluation of stable, asymptomatic skin lesions that had been present for several years. His history was notable for Graves disease treated with thyroidectomy, and significant ensuing weight gain that predated the onset of his cutaneous findings. The patient was otherwise healthy, and his only prior medical treatment was levothyroxine. He was adopted and his family history was unknown.Physical examination revealed an overweight man with asymptomatic hyperpigmented reticulated plaques and longitudinal striae over his bilateral flanks and legs (Fig 1). No other concerning cutaneous lesions were present. A 4-mm punch biopsy was performed of a right lower flank hyperpigmented plaque. Hematoxylin-eosin and elastic stains showed fragmented, mineralized elastic fibers in the reticular dermis. Elastic fibers with calcium deposition were present on von Kossa stain (Fig 2).Fig 2Localized calcific elastosis. Histology of lesional skin, right lower flank. A, Hematoxylin-eosin stain with fragmented elastic fibers in the reticular dermis. B, von Kossa stain revealing calcium deposition on dermal elastic fibers. (Original magnifications: A, ×40; B, ×10.)View Large Image Figure ViewerDownload Hi-res image Download (PPT)The patient's histologic examination, lesional morphology, and lack of systemic symptoms suggested a diagnosis of acquired calcific elastosis in the setting of weight gain. The differential diagnosis also included pseudoxanthoma elasticum (PXE), a rare heritable disorder associated with defects of the ABCC6 gene, causing progressive mineralization of elastic fibers and complications of the skin, eye, and cardiovascular system.1Marchione R. Kim N. Kirsner R.S. Pseudoxanthoma elasticum: new insights.J Invest Dermatol. 2009; 129: 258Crossref PubMed Scopus (6) Google Scholar, 2Lewis K.G. Bercovitch L. Dill S.W. Robinson-Bostom L. Acquired disorders of elastic tissue: part I. Increased elastic tissue and solar elastotic syndromes.J Am Acad Dermatol. 2004; 51 (quiz 22-4): 1-21Abstract Full Text Full Text PDF PubMed Scopus (144) Google Scholar Cutaneous lesions may be the presenting sign; however, 85% of patients with inherited PXE demonstrate angioid streaks, the characteristic retinal findings resulting from breaks in Bruch's elastic membrane.3James W.D. Berger T.G. Elston D.M. Abnormalities of the dermal fibrous and elastic tissue. Andrews' Disease of the Skin. WB Saunders, Philadelphia2011Google Scholar Vascular involvement is also common, manifesting as hemorrhage, intermittent claudication, and/or hypertension.PXE-like features have been reported in calciphylaxis and inflammatory conditions, including lipodermatosclerosis, granuloma annulare, lichen sclerosis, morphea profunda, erythema nodosum, septal panniculitis, and nephrogenic systemic fibrosis. More broadly, 4 major types of cutaneous calcification exist—dystrophic, metastatic, idiopathic, and iatrogenic—according to the original etiology of the symptoms. Dystrophic calcinosis cutis, including that of PXE and calcific elastosis, occurs in damaged or inflamed skin secondary to mechanical, chemical, or other factors; serum calcium and phosphate remain within normal limits in such cases.4Li Q. Jiang Q. Pfendner E. Váradi A. Uitto J. Pseudoxanthoma elasticum: clinical phenotypes, molecular genetics and putative pathomechanisms.Exp Dermatol. 2009; 18: 1-11Crossref PubMed Scopus (124) Google Scholar Localized calcific elastosis, although histologically indistinguishable from PXE, is a distinct, often skin-limited entity.2Lewis K.G. Bercovitch L. Dill S.W. Robinson-Bostom L. Acquired disorders of elastic tissue: part I. Increased elastic tissue and solar elastotic syndromes.J Am Acad Dermatol. 2004; 51 (quiz 22-4): 1-21Abstract Full Text Full Text PDF PubMed Scopus (144) Google Scholar It is most frequently described as lax, reticulated, or cobble-stoned plaques often in the periumbilical region of obese multiparous women. Mechanical stress, such as that of pregnancy and/or surgery, with subsequent elastic fiber degeneration is thought to produce localized disease.2Lewis K.G. Bercovitch L. Dill S.W. Robinson-Bostom L. Acquired disorders of elastic tissue: part I. Increased elastic tissue and solar elastotic syndromes.J Am Acad Dermatol. 2004; 51 (quiz 22-4): 1-21Abstract Full Text Full Text PDF PubMed Scopus (144) Google Scholar, 3James W.D. Berger T.G. Elston D.M. Abnormalities of the dermal fibrous and elastic tissue. Andrews' Disease of the Skin. WB Saunders, Philadelphia2011Google Scholar Calcific elastosis has been reported in the setting of ascites, anasarca, chronic renal failure, and tumefactive lipedema.2Lewis K.G. Bercovitch L. Dill S.W. Robinson-Bostom L. Acquired disorders of elastic tissue: part I. Increased elastic tissue and solar elastotic syndromes.J Am Acad Dermatol. 2004; 51 (quiz 22-4): 1-21Abstract Full Text Full Text PDF PubMed Scopus (144) Google Scholar, 5Taylor N.E. Foster W.C. Wick M.R. Patterson J.W. Tumefactive lipedema with pseudoxanthoma elasticum-like microscopic changes.J Cutan Pathol. 2004; 31: 205-209Crossref PubMed Scopus (13) Google Scholar Certain chemicals, such as Norwegian saltpeter (calcium-ammonium-nitrate salts) fertilizer and penicillamine have also been associated with calcific elastosis.To definitively rule out PXE, the patient underwent ophthalmologic evaluation, cardiovascular consultation, and routine health screening. Molecular analysis of ABCC6 was not performed in this case, in that these clinical evaluations were all within normal limits.6Plomp A.S. Toonstra J. Bergen A.A. van Dijk M.R. de Jong P.T. Proposal for updating the pseudoxanthoma elasticum classification system and a review of the clinical findings.Am J Med Genet A. 2010; 152A: 1049-1058Crossref PubMed Scopus (101) Google Scholar Localized calcific elastosis has not been previously described secondary to weight gain alone. As evidenced by our findings, when confronted with cutaneous elastic tissue abnormalities, it is important that clinicians consider and pursue a comprehensive diagnostic workup to rule out underlying systemic involvement when appropriate for long-term clinical management and counseling of affected patients. To the Editor: A 51-year-old man presented for evaluation of stable, asymptomatic skin lesions that had been present for several years. His history was notable for Graves disease treated with thyroidectomy, and significant ensuing weight gain that predated the onset of his cutaneous findings. The patient was otherwise healthy, and his only prior medical treatment was levothyroxine. He was adopted and his family history was unknown. Physical examination revealed an overweight man with asymptomatic hyperpigmented reticulated plaques and longitudinal striae over his bilateral flanks and legs (Fig 1). No other concerning cutaneous lesions were present. A 4-mm punch biopsy was performed of a right lower flank hyperpigmented plaque. Hematoxylin-eosin and elastic stains showed fragmented, mineralized elastic fibers in the reticular dermis. Elastic fibers with calcium deposition were present on von Kossa stain (Fig 2). The patient's histologic examination, lesional morphology, and lack of systemic symptoms suggested a diagnosis of acquired calcific elastosis in the setting of weight gain. The differential diagnosis also included pseudoxanthoma elasticum (PXE), a rare heritable disorder associated with defects of the ABCC6 gene, causing progressive mineralization of elastic fibers and complications of the skin, eye, and cardiovascular system.1Marchione R. Kim N. Kirsner R.S. Pseudoxanthoma elasticum: new insights.J Invest Dermatol. 2009; 129: 258Crossref PubMed Scopus (6) Google Scholar, 2Lewis K.G. Bercovitch L. Dill S.W. Robinson-Bostom L. Acquired disorders of elastic tissue: part I. Increased elastic tissue and solar elastotic syndromes.J Am Acad Dermatol. 2004; 51 (quiz 22-4): 1-21Abstract Full Text Full Text PDF PubMed Scopus (144) Google Scholar Cutaneous lesions may be the presenting sign; however, 85% of patients with inherited PXE demonstrate angioid streaks, the characteristic retinal findings resulting from breaks in Bruch's elastic membrane.3James W.D. Berger T.G. Elston D.M. Abnormalities of the dermal fibrous and elastic tissue. Andrews' Disease of the Skin. WB Saunders, Philadelphia2011Google Scholar Vascular involvement is also common, manifesting as hemorrhage, intermittent claudication, and/or hypertension. PXE-like features have been reported in calciphylaxis and inflammatory conditions, including lipodermatosclerosis, granuloma annulare, lichen sclerosis, morphea profunda, erythema nodosum, septal panniculitis, and nephrogenic systemic fibrosis. More broadly, 4 major types of cutaneous calcification exist—dystrophic, metastatic, idiopathic, and iatrogenic—according to the original etiology of the symptoms. Dystrophic calcinosis cutis, including that of PXE and calcific elastosis, occurs in damaged or inflamed skin secondary to mechanical, chemical, or other factors; serum calcium and phosphate remain within normal limits in such cases.4Li Q. Jiang Q. Pfendner E. Váradi A. Uitto J. Pseudoxanthoma elasticum: clinical phenotypes, molecular genetics and putative pathomechanisms.Exp Dermatol. 2009; 18: 1-11Crossref PubMed Scopus (124) Google Scholar Localized calcific elastosis, although histologically indistinguishable from PXE, is a distinct, often skin-limited entity.2Lewis K.G. Bercovitch L. Dill S.W. Robinson-Bostom L. Acquired disorders of elastic tissue: part I. Increased elastic tissue and solar elastotic syndromes.J Am Acad Dermatol. 2004; 51 (quiz 22-4): 1-21Abstract Full Text Full Text PDF PubMed Scopus (144) Google Scholar It is most frequently described as lax, reticulated, or cobble-stoned plaques often in the periumbilical region of obese multiparous women. Mechanical stress, such as that of pregnancy and/or surgery, with subsequent elastic fiber degeneration is thought to produce localized disease.2Lewis K.G. Bercovitch L. Dill S.W. Robinson-Bostom L. Acquired disorders of elastic tissue: part I. Increased elastic tissue and solar elastotic syndromes.J Am Acad Dermatol. 2004; 51 (quiz 22-4): 1-21Abstract Full Text Full Text PDF PubMed Scopus (144) Google Scholar, 3James W.D. Berger T.G. Elston D.M. Abnormalities of the dermal fibrous and elastic tissue. Andrews' Disease of the Skin. WB Saunders, Philadelphia2011Google Scholar Calcific elastosis has been reported in the setting of ascites, anasarca, chronic renal failure, and tumefactive lipedema.2Lewis K.G. Bercovitch L. Dill S.W. Robinson-Bostom L. Acquired disorders of elastic tissue: part I. Increased elastic tissue and solar elastotic syndromes.J Am Acad Dermatol. 2004; 51 (quiz 22-4): 1-21Abstract Full Text Full Text PDF PubMed Scopus (144) Google Scholar, 5Taylor N.E. Foster W.C. Wick M.R. Patterson J.W. Tumefactive lipedema with pseudoxanthoma elasticum-like microscopic changes.J Cutan Pathol. 2004; 31: 205-209Crossref PubMed Scopus (13) Google Scholar Certain chemicals, such as Norwegian saltpeter (calcium-ammonium-nitrate salts) fertilizer and penicillamine have also been associated with calcific elastosis. To definitively rule out PXE, the patient underwent ophthalmologic evaluation, cardiovascular consultation, and routine health screening. Molecular analysis of ABCC6 was not performed in this case, in that these clinical evaluations were all within normal limits.6Plomp A.S. Toonstra J. Bergen A.A. van Dijk M.R. de Jong P.T. Proposal for updating the pseudoxanthoma elasticum classification system and a review of the clinical findings.Am J Med Genet A. 2010; 152A: 1049-1058Crossref PubMed Scopus (101) Google Scholar Localized calcific elastosis has not been previously described secondary to weight gain alone. As evidenced by our findings, when confronted with cutaneous elastic tissue abnormalities, it is important that clinicians consider and pursue a comprehensive diagnostic workup to rule out underlying systemic involvement when appropriate for long-term clinical management and counseling of affected patients.