Symmetrical, papular, eruptive auricular collagenomas

Symmetrical, papular, eruptive auricular collagenomas Roberto Betti, MD,a Elena Inselvini, MD,a Claudia Pazzini, MD,a Laura Moneghini, MD,b and Carlo Crosti, MDa Milan, Italy Collagenomas are connective tissue nevi of the skin consisting of excessive deposition of collagen in the dermis. We describe a patient with acquired eruptive collagenomas located on both ears. Histologically, thickening of the dermis, caused by collagen deposition was present. (J Am Acad Dermatol 1998;39:363-4.)

Cutaneous hamartomas of the collagen type are divided into the inherited and acquired types. The acquired forms include eruptive and isolated collagenomas. Usually, the isolated form presents as a single plaque on the trunk and arm; the multiple familiar or eruptive collagenomas appear as asymptomatic papulonodules on the same areas of the body. We describe an unusual presentation of acquired eruptive collagenomas symmetrically distributed on the ears. CASE REPORT A 42-year-old man had asymptomatic papules on both ears that appeared 2 years before, and continued to develop. He had no previous skin eruptions or auricular trauma. No other family members were similarly affected. Examination revealed multiple, firm, elastic, fleshcolored papules, sometimes coalescing in plaques, on both ears (Fig. 1). The results of routine laboratory studies were normal. Electrocardiogram, echocardiogram, and radiologic examination of the bones and chest did not reveal any abnormalities. A biopsy specimen showed only minimal epidermal changes and thickening of the dermis due to increased collagen deposition. In the upper and mid-dermis, an accumulation of dense, coarse, thick collagen fibers was present. Collagen bundles were variably oriented with many of them running perpendicularly to the skin surface. The collagen was strongly stained with Masson This article is made possible through an educational grant from Ortho Dermatological. From the Università degli Studi di Milano, Clinica Dermatologica IV,a and the Università degli Studi di Milano, Istituto di Anatomia ed Istologia Patologica II.b Reprint requests: Roberto Betti, MD, Clinica Dermatologica IV, Osp.S.Paolo, Via di Rudinì 8, 20142 Milan, Italy. Copyright © 1998 by the American Academy of Dermatology, Inc. 0190-9622/98/$5.00 + 0 16/4/88471

trichrome stain, showing an intense blue-green color (Fig. 2). The orcein stain for elastic tissue showed sparse, fragmented, elastic fibers that seemed to be diminished in number in the upper and mid-dermis. The Alcian-blue stain at pH 2.5 and the periodic acid Schiff stain showed no accumulation of mucopolysaccharides. DISCUSSION

Collagen tissue nevi are hamartomas characterized by an excess or deficit in the number of the cells or their biosynthetic products: collagen, elastic fibers, and glycosaminoglycans. Various classifications have been proposed. Rocha and Winkelsmann1 proposed that connective tissue nevi are hamartomas of collagen resulting in an alteration in the smooth muscle, fat and elastic tissue balance. Pierard and Lapiere2 classified nevi of connective tissue into two main basic subgroups according to the portion of the dermis principally involved: nevi of reticular connective tissue and nevi of the adventitial connective tissue. Uitto et al.3 proposed a classification based on clinical, genetic, and histopathologic considerations. According to their classification, hamartomas of the collagen type or collagenomas can be inherited (familial cutaneous collagenoma, shagreen patches in tuberous sclerosis) or acquired (eruptive collagenomas, isolated collagenoma). Familial cutaneous collagenomas are generally composed of numerous symmetrical, asymptomatic papules, or nodules on the trunk and arms; most are located on the upper two thirds of the back.4,5 An autosomal dominant mode of inheritance has been reported. Individual lesions vary in diameter from a few millimeters to a few centimeters. They are discrete, firm, indurated, flesh-colored, round or oval, and slightly elevated. The lesions usually appear during adolescence. Cardiac abnormalities, in par363

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Fig. 1. Multiple papules on ear. Both ears are similarly affected.

ticular idiopathic progressive myocardiopathy with congestive heart failure and early R-wave transition are present in some patients.4,5 No osteopoikilosis is observed unlike the Bushke-Ollendorff syndrome. Histologically, the lesions show an accumulation of dense, coarse collagen fibers in the dermis with an apparent reduction in the number of elastic fibers. In contrast, eruptive collagenomas are acquired and nonfamilial, although they are clinically and histologically similar to familial cases. Our case fits into this category because of the negative family history and the accumulation of collagen fibers without other components, such as mucopolysaccharides and elastic fibers. The term eruptive collagenoma has been applied to these cases because of their abrupt development. Only a few cases have been described,6-8 but to our knowledge, this is the first report of symmetrical involvement of the ears. However, the lesions themselves are clinically similar to those of patients described by Woerdeman7 as having eruptive collagenomas on the extremities and lower trunk. This is thought to be a separate entity, but the

Journal of the American Academy of Dermatology August 1998

Fig. 2. Biopsy specimen, showing many dense, coarse collagen fibers. (Masson stain; original magnification ×100.)

papules are similar if not identical to lichen myxedematosus and contain mucin in the dermis.9 The lack of mucin in our case further supports the diagnosis of true eruptive collagenoma. REFERENCES 1. Rocha G, Winkelmann RK. Connective tissue nevus. Arch Dermatol 1962;85:722-9. 2. Pierard GE, Lapiere CM. Nevi of connective tissue: a reappraisal of their classification. Am J Dermatopathol 1985;7:325-33. 3. Uitto J, Santa Cruz DJ, Eisen AZ. Connective tissue nevi of the skin. J Am Acad Dermatol 1980;3:441-61. 4. Henderson RR, Wheeler CE, Abele DC. Familial cutaneous collagenoma. Arch Dermatol 1968;98:23-7. 5. Uitto J, Santa Cruz DJ, Eisen AZ. Familial cutaneous collagenoma: genetic studies on a family. Br J Dermatol 1979;101:185-95. 6. Lowenthal LJA. Connective tissue nevi and collagenome eruptif. Dermatologica 1957;114:81-90. 7. Woerdeman MJ. Is collagenoma eruptive a separate entity? Br J Dermatol 1960;72:217-20. 8. Berberian BB, Wood C. Asymptomatic nodules on the back and abdomen: connective tissue nevi, eruptive collagenoma type. Arch Derm 1987;123:811-2. 9. Metz J, Schubert E. Das sog.”eruptive Kollagenom”- ein Lichen myxedematosus? Arch Dermatol Forsch 1971; 240:148-59.