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Papular variant of annular elastolytic giant-cell granuloma

  • Author(s): Marmon, Shoshana
  • O'Reilly, Kathryn E
  • Fischer, Max
  • Meehan, Shane
  • Machler, Brian
  • et al.
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Papular variant of annular elastolytic giant-cell granuloma
Shoshana Marmon MD PhD, Kathryn E O’Reilly MD PhD, Max Fischer MD, Shane Meehan MD, Brian Machler MD
Dermatology Online Journal 18 (12): 23

The Ronald O. Perelman Department of Dermatology, New York University School of Medicine, New York, New York

Abstract

A 53-year-old woman presented with a six-month history of non-pruritic, erythematous papules and papular-plaques that were localized to the anterior and lateral aspects of the neck. A biopsy specimen showed elastolysis and granuloma formation, which were consistent with a diagnosis of annular elastolytic giant-cell granuloma. This is one of the few reported cases of this entity that consists predominantly of papular lesions rather than annular plaques.



History

A 53-year-old woman presented with a six-month history of a non-pruritic eruption of the neck. The patient was initially referred for patch tests because the cutaneous eruption was thought to be the result of an allergic contact dermatitis. Prior treatments included high-potency topical glucocorticoids with negligible effect. Past medical history included breast cancer that resulted in a mastectomy. She reported allergies to penicillin and sulfonamides. Her medications included valsartan, amlodipine, and eszopiclone. She stopped taking valsartan and amlodipine after development of the skin condition without any improvement. She was subsequently started on a course of antibiotics to treat the eruption. After having failed multiple courses of topical glucocorticoids, a combination of rifampin, floxacin, and minocycline was initiated.


Physical examination


Figure 1Figure 2

Pink-to-red papules with some coalescence into poorly demarcated plaques were present on the anterior and lateral aspects of the neck.


Laboratory data

None.


Histopathology


Figure 3Figure 4

Within the reticular dermis, there is a nodular infiltrate of mono- and multinucleated histiocytes demonstrating elastophagocytosis. An elastic Van Gieson stain highlights solar elastosis within the papillary dermis and a loss of elastic fibers within the histiocytic infiltrate.


Discussion

Annular elastolytic giant-cell granuloma (AEGCG) is an uncommon dermatologic condition that is characterized by granuloma formation and phagocytosis of elastic fibers in the dermis [1, 2]. Although AEGCG shares histopathologic similarities with generalized granuloma annulare and necrobiosis lipoidica, the absence of mucin and necrobiosis and the presence of giant cells in the dermis distinguish it as a unique entity [3]. The majority of AEGCG cases have been identified in middle-aged, Caucasian women [4]. The most common clinical presentation consists of annular plaques or patches often with elevated borders and central atrophy [4], although rare reports of a papular variant of AEGCG exist [3, 5, 6]. AEGCG is alternatively termed actinic granuloma when present on sun-exposed skin where it most often is noted [2, 7]. Nonetheless, lesions have been demonstrated in covered areas of the body, which suggests that sun exposure is not necessary for pathogenesis [2, 5]. In one study, the lesions of actinic granuloma could not be induced [8].

Although the etiology of AEGCG remains unclear, it has been theorized that there may be an association with systemic diseases [7]. Speculation exists that the presence of diabetes mellitus may contribute to the expression of AEGCG by inducing damage to elastic fibers in the skin [9]. The concomitant presence of the two conditions has been described in a number of case reports [9, 10, 11, 12]. AEGCG has also been identified with solid organ tumors and hematologic malignant conditions [7]. In two cases of AEGCG that were associated with hematologic malignant conditions, the cutaneous presentation improved upon treatment of the underlying illness [13, 14]. At the molecular level, the macrophage metalloproteinase MMP-12 has been demonstrated in biopsy specimens from a patient with AEGCG, which suggests that this enzyme may be involved in the degradation of elastic fibers [7].

Treatment options, which included high-potency topical glucocorticoids, hydroxychloroquine, isotretinoin, and PUVA photochemotherapy, have had various degrees of success [12, 15]. Rifampin, ofloxacin, and minocycline have been reported to be effective in the treatment of granuloma annulare that is refractory to standard therapeutic modalities [16].

References

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16. Marcus DV, et al. Granuloma annulare treated with rifampin, ofloxacin, and minocycline combination therapy. Arch Dermatol 2009;145:787 [PubMed]

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