Volume 27 Number 2, Part 2 August 1992
20. 21. 22. 23. 24.
nosis:demonstration of normallipid metabolismin cultured cells. Arch Dermatol 1974;110:591-4. HashimotoK, KlingmiillerG, Rodermund OE. Hyalinosis cutis et mucosae. An electron microscopic study. Aeta Derm Venereol (Stockh) 1972;52:179-95. Fabrizi G, Porfiri B, BorgioliM, et al. Urbach-Wiethedisease. Light and 91ectronmicroscopicstudy. J Cutan Pathol 1980;7:8-20. lshibashi A. Hyalinosis cutis et mucosae: defectivedigestion and storage ofbasal lamina glycoproteinsynthesizedby smooth muscle cells. Dermatologica 1982;165:7-15. Dupre A, Viraben R, Bonafe JL. Etude ultrastructurale d'un cas de hyalinose eutaneo-muquense. Ann Dermatol Venereol 1981;108:1003-10. Bauer EA, Santa-Cruz DJ, Aisen AZ. Lipoidproteinosis: in vivoand in vitro evidencefor a lysos..omalstoragedisease. J Invest Dermatol 1981;76:119-25.
25. Harper JI, Duance VC, Sims T J, et al. Lipoid proteinosis: an inheriteddisorder of collagenmetabolism? Br J Dermatol 1985;113:145-51. 26. FleischmajerR, Krieg T, Dziadek M, et al. Ultrastructure and compositionof connective tissue in hyalinosis cutis et mucosae skin. J Invest Dermatol 1984;82:252-8, 27. Olsen DR, Chu M-L, Uitto J. Expression of basement membrane zone genes coding for type IV proeollagen and laminin by human skin fibroblastsin vitro: elevated ~I(IV) collagen mRNA levels in lipoid proteinosis. J Invest Dermatol 1988;90:734-8. 28. Moy LS, Moy RL, Matsuoka LY, et al. Lipoid proteinosis: ultrastructural and biochemical studies. J AM ACADDERMATOL1987;16:1193-201. 29. Wong CK, Lin CS. Remarkable response of lipoid proteinosis to oral dimethyl sulphoxide. Br J Dermatol 1988; 119:541-4,
Lobular capillary hemangioma (pyogenic granuloma) with satellitosis James W. Taira, MD, Tamara L.
MD, and M a r k A. Everett, M D
Oklahoma City, Oklahoma We describe a 43-year-old white man who rapidly developed multiple, extensive angiomatous lesions on the temple and scalp after excision of a solitary lobular capillary hemangioma. This is a well-recognized but rare event. Our case differs from previously reported examples in terms of the age of the patient, the location and extent of the lesions, the histologic features in the form of small foci of angiosarcoma-like infiltration, and possibly with respect to the response to therapeutic intervention. Because of the alarming clinical picture produced by multiple lobular capillary hemangiomas, in addition to the occurrence of disturbing histologic features, the benign and self-limited nature of this disease must be emphasized. (J AM ACAD DERMATOL 1992;27:297-300.) The term lobular capillary hemangioma ( L C H ) was introduced in 19801 to provide a descriptive designation for vascular proliferations historically referred to as pyogenic granuloma. This common, benign, usually solitary lesion has on rare occasions appeared with striking multiplicity. W e report a case of L C H with multiple satellites that differs from previously published cases in several clinical, histologic, and therapeutic aspects.
Fromthe DepartmentofDermatology,UniversityofOklahomaHealth SciencesCenter. Reprint requests: James W. Taira, MD, Universityof Oklahoma HealthSciencesCenter,DepartmentofDermatology,619N.E. 13th St., OklahomaCity, OK 73104.
CASE REPORT A 43-year-old white man noticed the appearance of a small, red papule on his left temple on August 20, 1990. The lesion was excised on Sept. 8, 1990, and a diagnosis of capillary hemangioma was made at another institution. During the next several weeks multiple lesions that were similar in appearance developed around the excision site. On Oct. 31, 1990, a biopsy specimen of one of the recurrent lesions was again interpreted as capillary hemangioma. Numerous additional lesions appeared after this second procedure. We first saw the patient on Nov. 13, 1990. In the region of his left forehead he had multiple, firm, dusky red papules and nodules ranging from 2 to 12 mm in diameter that varied from being fiat to being elevated approximately 5 mm above the skin surface (Fig. 1). The lesions were located in a superior and lateral position to the pre297
Journal of the American Academy of Dermatology
Taira et al.
',s, , ? 'i '/,,
. . . . '1
Fig. 1. Lesions as they appeared approximately 9 weeks after original excision. Only a few of the smaller lesions developed from within the surgical scar. Fig. 2. Low-power view of a larger satellite lesion that is composed of vascular spaces arranged in lobules in the dermis.The epidermis is effaced and the lobule is surrounded by an epiderma! collarette. (Hematoxylin-eosin stain; • 10.) Fig. 3. Resolution of lesions; only erythema and hyperpigmentation remain. Induration is found in areas formerly occupied by some larger lesions.
vious excision site and occupied an area that measured approximately 8 • 5 cm. Only a few smaller lesions appeared to develop from within the scar itself. A biopsy of several additional lesions was performed. Histologic examination showed features of a typical LCH. Vascular proliferations arranged in discrete lobules of varying size appeared throughout the papillary and reticular dermis (Fig. 2). The epidermis overlying the lobules was effaced and formed epidermal collarettes in some areas. Some lobules closest to the skin surface were superficially ulcerated. Individual lobules were composed predominantly of small, uniform, well-formed capillary channels clustered around central vascular spaces that were sUghtly more angulated and dilated. The endothelial cells were focally enlarged, but significant nuclear atypicality was not observed. Mitotic figures were in-
frequent. The surrounding stroma varied from loose and edematous to slightly fibrotic. Inflammatory cells in the nonulcerated areas were sparse. In several small loci vascular proliferations appeared to lose their lobular organization and dissected between collagen fibers as illdefined, irregularly branching spaces (Fig. 3). The endothelial cells in these loci were, however, small and uniform. We reviewed sections from the two previous biopsy specimens. Both showed features that, in our opinion, warranted a ctiagnosis of LCH. During the next 2 months most of the lesions persisted unchanged, whereas a few appeared to become slightly more protuberant. In addition, many new lesions appeared on the upper forehead and extended into the scalp. Few if any of the lesions underwent regression, although
Volume 27 Number 2, Part 2 August 1992
one of the larger lesions became necrotic and spontaneously sloughed. A course of oral erythromycinresulted in no discernable change. Three days after a regular visit on January 19, 199 l, at which time no significant change was noted, the patient began daily applications of an over-the-counter topical salve (Melaleuca*). This treatment was associated with a rapid decrease in the size of the lesions and eventual, gradual resolution. On March 11, 1991, the lesions had virtually disappeared, leaving only mild erythema, hyperpigmentation, and induration. One month later the lesions had not recurred. DISCUSSION Although L C H is very common, multiple lesions are rarely reported. The first 21 cases with multiple satellite lesions (occasionally referred to in the literature as the Warner-Wilson Jones syndrome) were summarized in 1968. 2 Since then, only eight additional cases have been described in the English language literature. 3-7 Most cases have developed after extirpation of a single solitary tumor. However, at least six cases of patients with widely disseminated lesions that were unassociated with a history of previous manipulation have been reported. 812 Our patient differs in two clinical aspects from the usual patient with multiple satellite lesions. First, most of the patients in the 29 cases referenced above were children or adolescents. Only four patients were more than 20 years of age and only two patients were older than ours. 2, 7 Second, lesions in most reported cases occur on the trunk, particularly in the region of the scapula. In only one other case did satellite lesions involve the face. a Disturbing histologic features are a well-recognized finding in otherwise benign vascular tumors. These include areas of angioendothelioma-like proliferations with deep extension into the subcutaneous tissue, 2 prominent capillary endothelial proliferation, 3 areas mimicking intravascular papillary endothelial hyperplasia (Masson's pseudoangiosarcoma), 4 endothelial cell atypicality with shedding into the vascular lumina,13~" and intravascular growth. 14 *Melaleuca is an over-the-counterpreparation marketed as a topical salve(Melaleuca Inc., Idaho Falls, Idaho). It is extractedas a 100% pure natural oil from the plant Melaleuca alterntfolia. Terpinen4-ol is listedas the most important of 48 compoundscontainedwithin the product. The manufacturer claims antisepticand antifungalactivity as the major therapeutic properties. ]'This case has subsequently been widely accepted in literature as an exampleof papular angioplasia,
Lobular capillary hemangioma with satellitosis 299 In our case small foci of endothelial-lined vascular spaces dissecting collagen fibers reminiscent of angiosarcoma were a source of concern. Although we tend to agree with previously published statements that the histologic criteria for distinguishing between benign and malignant neoplasms are occasionally unsatisfactory, 15 the dominant microscopic features in our case fulfill the criteria for L C H as originally described. 1 Another interesting aspect of this case is the potential role of therapeutic intervention. Even in cases of disseminated lesions, the usual clinical course has consistently been spontaneous resolution, which usually occurs within 6 months, l~ For this reason no specific therapy was attempted with this patient, with the exception of a short course of oral antibiotics. However, the patient's self-treatment correlated with a dramatic clinical response. Within 7 weeks an essentially complete regression of his lesions was achieved. Because of the temporal relationship of treatment to a rapid response, an effect of this topical preparation on the course of the disease cannot be ruled out. It is important to recognize that the often alarming clinicopathologic features of multiple satellite lesions do not imply malignancy. Patients should be advised that it is a potential complication of excisional biopsy of a solitary LCH, and they should be reassured that it is usually self-limited in nature when it does occur. Although spontaneous resolution is the usual expected outcome, lesions can persist unchanged for many months and can be a source of cosmetic concern. Therefore the role of nonsurgical (i.e. topical) therapeutic intervention to hasten the course of the disease deserves further study. REFERENCES 1. Mills SE, Cooper PH, Feehner RE. Lobular capiUary hemangioma: the underlying lesion of pyogenic granuloma: a study of 73 cases from the oral and nasat mucous membranes. Am J Surg Pathol 1980;4:471-9. 2. Warner J, Wilson Jones E. Pyogenic granuloma recurring with multiple satellites: a report of 11 eases. Br J Dermatol 1968;80:218-27. 3. Zaynoun ST, Juljulian HH, Kurban AK. Pyogenic granuloma with multiple satellites. Arch Dermatol 1974;109: 689-91. 4. Fisher I. Recurrent multiple pyogenie granuloma with multiple satellites: features of Masson's hemangioma. Cutis 1977;20:201-5. 5. Allen RK, Rodman OG. Pyogenic granuloma recurrent with satellite lesions. J Dermatol Surg Onco11979;5:490-3. 6. Amerigo J, Gonzalez-Campora R, Galera H, et al. Recurrent pyogenic granuloma with multiple satellites: elinico-
Journal of the American Academy of Dermatology
Taira et aL pathological and ultrastructural study. Dermatologica 1983;166:117-21. 7. Bliekenstaff RD, Roenigk RK, Peters MS, et al. Recurrent pyogenie granuloma with satetlitosis. J AM ACADDF_~MATOL 1989;21:1241-4. 8. Juhlin L, Hjertquist SO, Ponten J, et al. Disseminated granuloma pyogenieum. Acta Dermatol (Stockholm) 1970;50:134-6. 9. DeKamiasky AR, Otero AC, Kaminsky CA, et al. Multiple disseminated pyogenie granuloma. Br J Dermatol 1978;98:461-4. 10. Nappi O, Wick MR. Disseminated lobular capillary hemangioma (pyogenicgranutoma): a clinicopathologicstudy of two cases. Am J Dermatopathol i986;8:379-85.
11. Wilson BB, Greer KE, Cooper PH. Eruptive disseminated lobular capillaryhemangioma(pyogenicgranutoma). J AM ACADDERMATOL1989;21:391-4. 12. Strohal R, Gillitzer R, Zonzitis E, et al. Localized vs generalized pyogenic granuloma. A clinicopathologicstudy. Arch Dermatol 1991;127:856-61. 13. Peterson WC, Fusaro RM, Goltz RW. Atypical pyogenic granuloma. A case of benign hemangioendotheliosis.Arch Dermatol 1964;90:197-201. 14. Cooper PH, McAllisterHA, Helwig EB. Intravenous pyogenic graauloma. A study of 18 cases. Am J Surg Pathol 1979;3:221-8. 15. Johnson WC. Pathologyof cutaneous vascular tumors. Int J Dermatol 1976;15:239-70.
Eruptive xanthomas during pregnancyPeter W. Jaber, MD, a Barbara B. Wilson, M D , a Dearing W. Johns, M D , c Philip H. Cooper, MD, a and James E. Ferguson, II, M D b Charlottesville, Virginia A case of eruptive xanthomas during two successive pregnancies is reported. These xanthomas developed in association with marked hypertriglyceridemia; complications included severe pancreatitis and acute respiratory distress syndrome. This patient most likely had combined familial hyperlipidemia which usually causes only a modest elevation in plasma lipid levels. However, with the added stimulus of estrogens during pregnancy, hypertriglyceridemia and secondary complications developed. (J AM ACAD DERMATOL 1992;27:300-2.) W e describe a patient with eruptive xanthomas that occurred during pregnancy; to the best of our knowledge this association has not been previously reported. CASE R E P O R T A 33-year-old woman (gravida 2, para 1) had a pruritic, papular eruption on the elbows, arms, buttocks and thighs (Fig. 1) at 20 weeks' gestation. The papules were pale yellow and measured 2 to 4 mm in diameter. The patient recalled having identical lesions during her first pregnancy which was complicated by hypertriglyceridemia, fulminant pancreatitis and acute respiratory distress syndrome. Results of a biopsy specimen showed findings characteristic of eruptive xanthoma (Fig. 2). The patient's triglyceride revel was 7530 mg/dl (normal, 40 to 195 mg/dl), and total cholesterol level was 1515 mg/dl (normal,