About the cover

Granuloma faciale is a diagnosis that is often overlooked clinically and by general pathologists. My experience is that clinicians often stumble into the diagnosis. There is usually only one lesion, so that their suspicion is of a neoplasm rather than of an inflammatory condition. General pathologists often do not know of the condition or remember it. This makes it a satisfying diagnosis for dermatopathologists to make. Granuloma faciale is also one of those wonderful misnomers in dermatology. The term “granuloma” reflects a clinical appearance suggesting granules, as is the case in granuloma pyogenicum and granuloma gluteale infantum, other diseases in which granulomatous inflammation does not normally occur. Lesions of granuloma faciale are seldom biopsied at an early stage. That is why the findings in an early lesion (Fig. 1, and on the cover) are worth looking at in detail. The condition is a small vessel leukocytoclastic vasculitis, but not a conventional one. There is fibrin in the walls of vessels, and neutrophils and their dust around them in both conditions. Unlike conventional leukocytoclastic vasculitis, the amount of neutrophilic nuclear dust is scant, and extravasated erythrocytes are few. The infiltrates evidently become much denser than in conventional leukocytoclastic vasculitis rather quickly. The changes that make granuloma faciale more distinctive develop with time. In addition to dense infiltrates of neutrophils, eosinophils, and plasma cells, fibrosis supervenes, often oriented concentrically around small vessels (Fig. 2). The lesions of granuloma faciale are typically solitary facial plaques (Fig. 3). They can be extraordinarily long lasting and resistant to treatment, although there have been recent reported successes with laser therapy (1). Ackerman and Mones recently briefly stated their position that granuloma faciale and erythema elevate diutinum are “different names for the same condition, namely, longstanding leukocytoclastic vasculitis on different sites, the face for the former and the extremities for the latter” (2). I believe that they are two distinct diseases. Patients with plaques of granuloma faciale on their faces may develop extrafacial granuloma faciale, but not in a symmetrical manner (3–5). Extrafacial granuloma faciale can occur without lesions on the face, although most occur with them. There is a peculiar condition, angiocentric eosinophilic fibrosis of the larynx, which can accompany granuloma faciale (6). The histopathology of extrafacial granuloma faciale (including its laryngeal variant) is similar to that of facial lesions. Erythema elevatum diutinum begins, like granuloma faciale, with neutrophils and neutrophilic nuclear dust around small vessels that have fibrin in their wall (7). The sites of predilection are different—the face is usually spared, and the skin on the dorsal aspects of joints is usually the target. The eruption is bilateral and symmetric in most cases, sometimes startlingly so (Fig. 4). The lesions can become much more protuberant than those of granuloma faciale. There are many cases of erythema elevatum diutinum associated with systemic diseases, but far fewer cases of granuloma faciale that are similarly associated (8). Histopathologically, eosinophils are few or absent, neutrophils dominate the picture, and plasma cells are only present episodically. The large nodules of erythema elevatum diutinum often have a distinctive pattern of fibrosis (long, parallel arrays of collagen bundles and interspersed neutrophils) that old lesions of granuloma faciale do not have (Fig. 5) (9). The conditions are members of the same family, to be sure, but not the same. From the Departments of Pathology and Dermatology, University of California, San Francisco, California. Address correspondence and reprint requests to Philip E. LeBoit, M.D., Dermatopathology Section, University of California, San Francisco, 1701 Divisadero Street, Room 350, San Francisco, CA 94115 U.S.A. E-mail: philipl@itsa.ucsf.edu The American Journal of Dermatopathology 24(5): 440–443, 2002 © 2002 Lippincott Williams & Wilkins, Inc., Philadelphia