A case of papular elastolytic giant cell granuloma: A mini-review of reported cases Papular elastolytic giant cell granuloma (PEGCG) is a rare variant of annular elastolytic giant cell granuloma (AEGCG). Since Kato et al. [1] reported the first case of PEGCG, only eight additional cases have been reported. Herein, we report a case of PEGCG and review the literature of reported cases. A 75-year-old Japanese man presented with multiple pruritic eruptions on his back that recurred for 2 months. Past medical history included hypertension and hyperlipidemia. Physical examination disclosed reddish papules on the trunk and all the extremities (figure 1A). Some of the papules had aggregated to form irregular reddish plaques. No annular lesions were observed. Histopathologically, lymphocytes, histiocytes and multinuclear giant cells infiltrated among the collagen fibers in the dermis (figure 1B). Elastica van Gieson stain revealed a loss of elastic fibers in the reticular dermis and phagocytosis of the fragmented elastic fibers by multinucleated giant cells (figure 1C). Dermal mucin deposition was not identified in Alcian blue staining. The hemoglobin A1c value was normal. Lymphocyte transformation tests for his medications of amlodipine, fenofibrate and bisoprolol were all negative. We diagnosed the skin lesions as PEGCG. The patient was treated with topical corticosteroids for 4 months. Although the papules themselves remained, their color faded with treatment. AEGCG is a rare granulomatous skin disease, of which PEGCG is a variant. To the best of our knowledge, nine cases have been reported since the first report by Kato et al. in 1989 [1]. Asymptomatic red to flesh-colored papules scatter on the trunk and arms. A histopathological feature of these entities of elastolytic giant cell granuloma (EGCG) is the presence of granulomas in the upper to middle dermis, accompanied by elastophagocytosis and a decrease of elastic fibers in that part. We surveyed 10 cases of PEGCG after 1989, including our case (table 1) [1-9]. The female-to-male ratio was found to be 3:7. The age of onset ranged from the fifth to the eighth decade, with a mean of age 62. In comparison with cases in a review of elastolytic granuloma, which mainly reviewed AEGCG and actinic granuloma [10], the ages of onset for PEGCG are similar to those for AEGCG and actinic granuloma. Complications of PEGCG were identified in seven cases, including hypertension, hyperlipidemia, monoclonal gammopathy, diabetes mellitus, osteoarthritis and anxiety disorder. As for the morphology of the ten cases, papules and annular lesions were simultaneously seen in two cases [1, 3], and papules were seen without descriptions of annular lesions in eight cases [2, 4-9]. Morita et al. [6] supposed that the papules in their PEGCG case were very early lesions of AEGCG that failed to form an annular pattern. However, eight cases of PEGCG without descriptions of annular lesions during their course and more AEGCG cases without papules [10] are inconsistent with a hypothesis that papules are early lesions of annular lesions in EGCG. From the above, papules and annular lesions could be observed independently of each other when the histopathology was EGCG. If papules and annular lesions coexist, the diagnoEJD, vol. 25, n◦ 2, March-April 2015

A

B

C

Figure 1. A) Reddish papules measuring 2 to 3 mm in diameter on the back. In some places, there is coalescence among neighbouring papules. B) Interstitial infiltration of lymphocytes, histiocytes and multinuclear giant cells is observed in the dermis (hematoxylin and eosin stain, original magnification ×200). C) A fragmented elastic fiber is seen in a multinuclear giant cell (Elastica van Gieson stain, original magnification ×200).

sis of PEGCG or AEGCG should be given according to the prevalent type of eruption. Regarding the location, papules were observed on the trunk in nine cases, the arms in seven cases, the lower limbs in three cases and the neck in two cases; there was no case with papules on the scalp or face. Thus, papular lesions of EGCG mainly involve the sun-protected areas, unlike the lesions of AEGCG. Therefore, sun-exposure might be a determining factor in the morphology of EGCG. Conversely, some case reports [1, 3] in which annular lesions appeared on sunprotected skin suggest that factors other than sun exposure are involved in the morphology of EGCG. There is no generally accepted treatment regimen for PEGCG, but most cases are started with topical corticosteroids (table 1). Other treatments such as pimecrolimus or cyclosporine might be therapeutic candidates for PEGCG. Sun protection might be also useful for preventing exacerbation. In conclusion, we present an additional case of PEGCG and reviewed 9 reported cases in the literature. In cases with numerous papules on the sun-protected skin of patients in middle to old age, PEGCG should be considered as a rare differential diagnosis.  Disclosure. Financial support: none. Conflict of interest: none. 1

Division of Dermatology, Department of Dermatology, Hokkaido University Graduate School of Medicine, Sapporo, Japan 3 Division of Pathology, Hokkaido P.W.F.A.C. Obihiro-Kosei General Hospital, West 6 South 8, Obihiro 080-0016, Japan 2

Shinichi NAKAZATO1 Yasuyuki FUJITA2 Ken MURAMATSU1 Keisuke KIKUCHI3 Hidetsugu SATO1 Hiroshi SHIMIZU2

205

Table 1. Cases of papular elastolytic giant cell granuloma reported in the literature. “N.D.” indicates “not described”. Reference Sex Age Sites of discrete papules or coalscent papules

Coexistence ofannular lesions

Complications

Effect of topical corticosteroid

Effect of oral corticosteroid

Other effective therapies

1

M

55

Trunk, arms

N.D.

N.D.

N.D.

2

M

75

Trunk, extremities

+ (on the trunk) None

Intralesional corticosteroid Hydroxychloroquine, quinacrine

3

F

63

Trunk, arms

4

M

52

5

F

53

Trunk, extremities Neck

6

M

71

Trunk, arms

None

7

M

71

Trunk, hand

N.D.

8

F

43

Neck, trunk

N.D.

9

M

62

Trunk

N.D.

Our case

M

75

Trunk, extremities

None

+ (on the abdomen) N.D. N.D.

Non-insulinNo improvement dependent diabetes mellitus, Hypertension, Osteoarthritis, Anxiety disorder Impaired glucose N.D. tolerance N.D.

Improvement

A history of mastectomy due to breast cancer Impaired glucose tolerance N.D.

No improvement

Improvement (prednisolone 20 mg) No improvement N.D.

No improvement

N.D.

Tranilast

No improvement

N.D.

Monoclonal gammopathy of unknown significance A history of gastrectomy due to a gastric ulcer Hypertension, hyperlipidemia

N.D.

N.D.

Narrow-band ultraviolet B Topical tacrolimus

N.D.

N.D.

Spontaneous regression

No improvement

N.D.

None

1. Kato H, Uyeki Y, Yaoita H. Papular lesions associated with annular elastolytic giant cell granuloma. J Am Acad Dermatol 1989; 21: 398400. 2. Kelly BJ, Mrstik ME, Ramos-Caro FA, Iczkowski KA. Papular elastolytic giant cell granuloma responding to hydroxychloroquine and quinacrine. Int J Dermatol 2004; 43: 964-6. 3. Kato H, Kitajima Y, Yaoita H. Annular elastolytic giant cell granuloma: an unusual case with papular lesions. J Dermatol 1991; 18: 667-70. 4. Fujimura T, Terui T, Tagami H. Disseminated papular interstitial elastolytic giant cell granuloma. Acta Dermato Venereol 2003; 83: 234-5. 5. Marmon S, O’Reilly KE, Fischer M, Meehan S, Machler B. Papular variant of annular elastolytic giant-cell granuloma. Dermatol Online J 2012; 18: 23. 6. Morita K, Okamoto H, Miyachi Y. Papular elastolytic giant cell granuloma: a clinical variant of annular elastolytic giant cell granuloma or generalized granuloma annulare? Eur J Dermatol 1999; 9: 647-9. 7. Takata T, Ikeda M, Kodama H, Ohkuma S. Regression of papular elastolytic giant cell granuloma using narrow-band UVB irradiation. Dermatology 2006; 212: 77-9. 8. Rongioletti F, Baldari M, Burlando M, Parodi A. Papular elastolytic giant cell granuloma: report of a case associated with monoclonal gammopathy and responsive to topical tacrolimus. Clin Exp Dermatol 2009; 35: 145-8. 9. Misago N, Ohtsuka Y, Ishii K, Narisawa Y. Papular and reticular elastolytic giant cell granuloma: rapid spontaneous regression. Acta Dermato Venereol 2007; 87: 89-90.

206

No improvement

N.D.

N.D. N.D.

10. Limas C. The spectrum of primary cutaneous elastolytic granulomas and their distinction from granuloma annulare: a clinicopathological analysis. Histopathology 2004; 44: 277-82. doi:10.1684/ejd.2015.2521

Calcipotriol/betamethasone dipropionate ointment compared with tacrolimus ointment for the treatment of erosive pustular dermatosis of the scalp: a split-lesion comparison Erosive pustular dermatosis of the scalp (EPDS) is a rare disorder of uncertain aetiology that mainly occurs on the sun-damaged scalps of elderly patients after trauma. Topical corticosteroids (TCS) have been widely used in the treatment of EPDS; anecdotal reports have described successful results with topical tacrolimus [1], calcipotriol [2] and dapsone [3, 4]. Nevertheless, due to the rarity of the disease, a comparison of topical treatments in terms EJD, vol. 25, n◦ 2, March-April 2015