Fig 2. Histopathology of a biopsy specimen of the lowerventral aspect of the arm. Glandlikemasses of gram-positivecocci (arrows) typical of staphylococcal botryomycosiswere detected by Accustain Gram stain (Sigma, Steinheim,Germany). (Original magnification:3100.)

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(5 �g) was administered intravenously to enhanceblood flow to the arm. Physiotherapy includedmanual lymphatic drainage of the right arm. Inaddition, compression bandages were applied toprevent further self-manipulation. Altogether, thesetreatments improved his skin condition.

Cutaneous botryomycosis is a chronic granulo-matous bacterial skin infection characterized bynodules, abscesses, and ulcers on the hands, feet,genitals, and head.2 It primarily results from theinoculation and persistent infection of injured skin3

by bacteria such as S aureus4 and Pseudomonasaeruginosa. T cellerelated immune deficiency anddiabetes can promote the disease.2

Norma Mechow, Daniela G€oppner, MD, IngolfFranke, MD, Malgorzata Kolesnik, BerndBonnekoh, MD, Harald P. M. Gollnick, MD,and Sven R. Quist, PhD, MD, MSc, MPharm, MBA

Department of Dermatology and Venereology, Otto-von-Guericke University, Magdeburg, Germany

Funding sources: None.

Conflicts of interest: None declared.

Correspondence to: Sven R. Quist, PhD, MD, MSc,MPharm, MBA, Department of Dermatology andVenereology, Otto-von-Guericke University, Leip-ziger Str. 44, D-39120, Magdeburg, Germany

E-mail: squist@gmx.de

REFERENCES

1. Hussein MR. Mucocutaneous Splendore-Hoeppli phenomenon.

J Cutan Pathol 2008;35:979-88.

Open access under CC BY-NC-ND license.

2. Mehregan DA, Su WP, Anhalt JP. Cutaneous botryomycosis.

J Am Acad Dermatol 1991;24:393-6.

3. Karthikeyan K, Thappa DM, Jeevankumar B. Cutaneous

botryomycosis in an agricultural worker. Clin Exp Dermatol

2001;26:456-7.

4. Akiyama H, Kanzaki H, Tada J, Arata J. Staphylococcus

aureus infection on cut wounds in the mouse skin:

experimental staphylococcal botryomycosis. J Dermatol Sci

1996;11:234-8.

http://dx.doi.org/10.1016/j.jaad.2014.06.017

Calcific elastosis in the setting of weight gain

To the Editor: A 51-year-old man presented forevaluation of stable, asymptomatic skin lesionsthat had been present for several years. Hishistory was notable for Graves disease treatedwith thyroidectomy, and significant ensuing weightgain that predated the onset of his cutaneousfindings. The patient was otherwise healthy,and his only prior medical treatment was levothy-roxine. He was adopted and his family history wasunknown.

Physical examination revealed an overweightman with asymptomatic hyperpigmented reticulatedplaques and longitudinal striae over his bilateralflanks and legs (Fig 1). No other concerningcutaneous lesions were present. A 4-mm punchbiopsy was performed of a right lower flankhyperpigmented plaque. Hematoxylin-eosin andelastic stains showed fragmented, mineralized elasticfibers in the reticular dermis. Elastic fibers withcalcium deposition were present on von Kossa stain(Fig 2).

The patient’s histologic examination, lesionalmorphology, and lack of systemic symptoms sug-gested a diagnosis of acquired calcific elastosis in thesetting of weight gain. The differential diagnosis alsoincluded pseudoxanthoma elasticum (PXE), a rareheritable disorder associated with defects of theABCC6 gene, causing progressive mineralization ofelastic fibers and complications of the skin, eye, andcardiovascular system.1,2 Cutaneous lesions may bethe presenting sign; however, 85% of patients withinherited PXE demonstrate angioid streaks, thecharacteristic retinal findings resulting from breaksin Bruch’s elastic membrane.3 Vascular involvementis also common, manifesting as hemorrhage, inter-mittent claudication, and/or hypertension.

PXE-like features have been reported in calci-phylaxis and inflammatory conditions, includinglipodermatosclerosis, granuloma annulare, lichensclerosis, morphea profunda, erythema nodosum,septal panniculitis, and nephrogenic systemic fibrosis.More broadly, 4 major types of cutaneous calcificationexist—dystrophic, metastatic, idiopathic, and

Fig 2. Localized calcific elastosis. Histology of lesionalskin, right lower flank. A, Hematoxylin-eosin stain withfragmented elastic fibers in the reticular dermis. B, vonKossa stain revealing calcium deposition on dermal elasticfibers. (Original magnifications: A, 340; B, 310.)

Fig 1. Localized calcific elastosis. Left flank with multiplehyperpigmented reticulated plaques and longitudinalstriae.

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iatrogenic—according to the original etiology of thesymptoms. Dystrophic calcinosis cutis, including thatof PXE and calcific elastosis, occurs in damaged orinflamed skin secondary to mechanical, chemical, orother factors; serum calcium and phosphate remainwithin normal limits in such cases.4 Localized calcificelastosis, although histologically indistinguishablefrom PXE, is a distinct, often skin-limited entity.2 It ismost frequently described as lax, reticulated, orcobble-stoned plaques often in the periumbilical

region of obese multiparous women. Mechanicalstress, such as that of pregnancy and/or surgery,with subsequent elastic fiber degeneration is thoughtto produce localized disease.2,3 Calcific elastosis hasbeen reported in the setting of ascites, anasarca,chronic renal failure, and tumefactive lipedema.2,5

Certain chemicals, such as Norwegian saltpeter(calcium-ammonium-nitrate salts) fertilizer and peni-cillamine have also been associated with calcificelastosis.

To definitively rule out PXE, the patientunderwent ophthalmologic evaluation, cardiovas-cular consultation, and routine health screening.Molecular analysis of ABCC6 was not performedin this case, in that these clinical evaluationswere all within normal limits.6 Localized calcificelastosis has not been previously describedsecondary to weight gain alone. As evidencedby our findings, when confronted with cutaneouselastic tissue abnormalities, it is important thatclinicians consider and pursue a comprehensivediagnostic workup to rule out underlying sys-temic involvement when appropriate for long-termclinical management and counseling of affectedpatients.

Monica Enamandram, MD,a Kathryn Schoch, NP,c

Daniel D. Miller, MD,b and Thomas D. Horn,MD, MBAa

Department of Dermatology, Massachusetts Gen-eral Hospital, Harvard Medical School,a andDermatopathology Sectionb and Department ofDermatology,c Boston University School ofMedicine, Boston, Massachusetts

Funding sources: None.

Conflicts of interest: None declared.

Correspondence to: Thomas D. Horn, MD, MBA,MGPO Dermatopathology Associates, 2 WellsAvenue, Newton, MA 02459

E-mail: THorn@partners.org

REFERENCES

1. Marchione R, Kim N, Kirsner RS. Pseudoxanthoma elasticum:

new insights. J Invest Dermatol 2009;129:258.

2. Lewis KG, Bercovitch L, Dill SW, Robinson-Bostom L. Acquired

disorders of elastic tissue: part I. Increased elastic tissue and

solar elastotic syndromes. J Am Acad Dermatol 2004;51:1-21,

quiz 22-4.

3. James WD, Berger TG, Elston DM. Abnormalities of the dermal

fibrous and elastic tissue. Andrews’ Disease of the Skin.

Philadelphia: WB Saunders; 2011.

4. Li Q, Jiang Q, Pfendner E, V�aradi A, Uitto J. Pseudoxanthoma

elasticum: clinical phenotypes, molecular genetics and

putative pathomechanisms. Exp Dermatol 2009;18:1-11.

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5. Taylor NE, Foster WC, Wick MR, Patterson JW. Tumefactive

lipedema with pseudoxanthoma elasticum-like microscopic

changes. J Cutan Pathol 2004;31:205-9.

6. Plomp AS, Toonstra J, Bergen AA, van Dijk MR, de Jong PT.

Proposal for updating the pseudoxanthoma elasticum

classification system and a review of the clinical findings.

Am J Med Genet A 2010;152A:1049-58.

http://dx.doi.org/10.1016/j.jaad.2014.06.034

Fig 1. Scleromyxedema. Skin-colored subcutaneous nod-ules on the forehead and eyelid edema.

Fig 2. Scleromyxedema. Photomicrograph of skin biopsyspecimen, showing pronounced deposit ofmucin associatedwith increase in fibroblasts and collagen. (Hematoxylin-eosin stain; original magnification:340.)

Atypical scleromyxedema with prominentnodular lesions associated with immunethrombocytopenia: An unusual presentation

To the Editor: Scleromyxedema (SM) is a rarecutaneous mucinosis that usually occurs with mono-clonal gammopathy ([83.2%), predominantly anIgG lambda subtype.1,2 SM may show a great varietyof extracutaneous manifestations ( gastrointestinal,musculoskeletal, neurologic, pulmonary, cardiac,and renal involvement) leading to significantmorbidity and mortality.1,2 We report a case of SMwith prominent scalp involvement, presence ofnodular lesions, and immune thrombocytopenia.

A 69-year-old woman presented with a 2-weekhistory of facial edema and progressive eruption.The patient also reported severe scalp pruritus andincreased hair loss.

On physical examination, skin-colored firm pap-ules, nodules, and edema of the face and both handswere observed. The papules were initially located onthe scalp, neck, and back of the auricular area, thengradually involved upper aspect of the trunk and thesurrounding skin showed scleroderma-like indura-tion in these areas. Several nodules, 4 to 10 mm insize, were present on the scalp, forehead (Fig 1), andside portions of the chin.

Pathological examination of biopsy specimensfrom a nodular scalp lesion and of a neck papule(Fig 2) revealed an increase in fibroblasts, collagen,and deposits of mucin in the papillary and mid-reticular dermis. Laboratory analysis showed a pro-gressive low platelet count (60-10 103/�L, normalrange 150-400 103/�L, platelet count within normallimits 6 months earlier), with a normal peripheralblood smear result and a normocellular bonemarrow with trilineage hematopoiesis. Serum pro-tein electrophoresis, immunofixation electropho-resis of serum and urine, and immunoglobulin freelight chain assays did not show paraproteinemia.Other laboratory examination findings includingthyroid function were within normal limits andautoantibody screening produced negative results.Computed tomographic scans of the chest,abdomen, and pelvis showed no relevant abnormal-ities. Electromyography detected signs consistent

with a right carpal tunnel syndrome and peripheralsensory and motor neuropathy. Based on clinicalmanifestations, and histopathological and laboratorydata, the diagnosis of SM with a secondary immunethrombocytopenia was made. The patient wastreated with 3 methylprednisolone pulses of 1 g,followed by 1 mg/kg/d of prednisone for 1 month,and intravenous immunoglobulins (2 g/kg percycle). Administration of a second course of intrave-nous immunoglobulins, delivered after 4 weeks,dramatically improved skin manifestations; howeverpersistence of low platelet count prompted theaddition of romiplostim, a thrombopoietin-receptoragonist. The prescribed corticosteroids were gradu-ally tapered off and romiplostim was withdrawn.After 12 months of follow-up, she remains asymp-tomatic and platelet count was within normal limits