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 Table of Contents  
CASE REPORT
Year : 2018  |  Volume : 5  |  Issue : 1  |  Page : 51-53

Annular elastolytic giant cell granuloma: An atypical presentation


1 Department of Dermatology, Sree Narayana Institute of Medical Sciences, Ernakulam, Kerala, India
2 Department of Dermatology, Pushpagiri Institute of Medical Sciences, Tiruvalla, Kerala, India
3 Department of Pathology, Pushpagiri Institute of Medical Sciences, Tiruvalla, Kerala, India

Date of Web Publication22-May-2018

Correspondence Address:
Dr. Vinitha Gopalakrishnan
3/96, Rajeevam, Puthukulangara, Edassery PO, Via Talikkulam, Thrissur - 680 569, Kerala
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijdpdd.ijdpdd_42_17

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  Abstract 


Annular elastolytic giant cell granuloma is a rare granulomatous disease with unknown pathogenesis. It clinically presents as single or multiple well-defined erythematous annular arciform serpiginous polycyclic plaque with indurated elevated borders and central atrophy in sun-exposed areas. It is found to have a causal association with conditions such as diabetes mellitus, T-cell leukemia, CD4 lymphoma, cutaneous amyloidosis, squamous cell carcinoma lung, sarcoidosis, and monoclonal gammopathy. On histopathology, characteristic “elastophagocytosis” of dermal elastic fibers by the multinucleated giant cells is seen. The presence of horizontally oriented fragmented elastic fibers is characteristic. There is no necrobiosis or mucin deposition, thus differentiating from granuloma annulare. We herein report a case of elderly male with type 2 diabetes mellitus who presented with asymptomatic multiple discrete erythematous shiny dome-shaped papules over anterior chest, abdomen, and back of 2-month duration. The diagnosis was confirmed by biopsy and thorough workup for associations was done. This case is reported due to the rarity of the disease, the atypical presentation, and the characteristic histopathology which lead to the diagnosis.

Keywords: Actinic granuloma, elastophagocytosis, papular annular elastolytic giant cell granuloma


How to cite this article:
Gopalakrishnan V, Thankappan TP, Sulochana G. Annular elastolytic giant cell granuloma: An atypical presentation. Indian J Dermatopathol Diagn Dermatol 2018;5:51-3

How to cite this URL:
Gopalakrishnan V, Thankappan TP, Sulochana G. Annular elastolytic giant cell granuloma: An atypical presentation. Indian J Dermatopathol Diagn Dermatol [serial online] 2018 [cited 2018 Dec 12];5:51-3. Available from: http://www.ijdpdd.com/text.asp?2018/5/1/51/232959




  Introduction Top


Annular elastolytic giant cell granuloma (AEGCG) is a rare granulomatous disease with unknown pathogenesis.[1],[2] It is characterized by well-defined erythematous annular plaques in polycyclic or serpiginous pattern usually occurring in sun-exposed areas.[3],[4] There are very few reports of nonannular forms like papular variant of the disease.[5] On histopathology, characteristic “elastophagocytosis” of dermal elastic fibers by the multinucleated giant cells is seen which confirms the diagnosis. Papular variant of AEGCG can be diagnosed only by histopathology.


  Case Report Top


A 64-year-old male, known diabetic for the past 10 years on oral hypoglycemic agents, presented with asymptomatic red lesions over the trunk of 45 days' duration. The lesions started as redness followed by small raised lesions over chest gradually involving abdomen and back. There was no systemic symptoms neither there was a history of any drug intake before the onset of skin lesion. Dermatological examination [Figure 1] and [Figure 2] revealed multiple discrete erythematous shiny nonfollicular dome-shaped papules of varying sizes distributed over anterior chest, abdomen and back, and few scattered papules in both the arms. Examination of other systems was noncontributory. Routine hematological and biochemical tests were normal except for raised fasting and postprandial blood sugars (165 mg% and 280 mg%, respectively). Syphilis serology (Venereal Disease Research Laboratory and  Treponema pallidum Scientific Name Search magglutination) was negative. Chest X-ray was noncontributory. A differential diagnosis of generalized granuloma annulare and papular pityriasis rosea were considered. Biopsy was taken and sent for histopathological examination. Histopathology showed normal epidermis; dermis showing multiple granulomas composed of nonpalisading histiocytes, epithelioid cells, and multinucleated giant cells [Figure 3] and [Figure 4]. No solar elastosis, necrobiosis, or any mucin deposits were found. Staining with Verhoeff-van Gieson [Figure 5] showed completely fragmented and lysed elastic fibers suggestive of elastophagocytosis. Special stains for acid–fast bacilli and fungi were negative. These findings were suggestive of AEGCG.
Figure 1: Multiple discrete erythematous shiny nonfollicular dome-shaped papules of varying sizes distributed over anterior chest, abdomen, and shoulders

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Figure 2: Multiple discrete erythematous shiny nonfollicular dome-shaped papules of varying sizes distributed over shoulders

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Figure 3: Histopathology (×10) showing normal epidermis; dermis showing multiple granulomas composed of nonpalisading histiocytes, epithelioid cells, and multinucleated giant cells

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Figure 4: High-power view (×40) showing multinucleated giant cells and inflammatory infiltrate

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Figure 5: Staining with Verhoeff-van Gieson (×40) showing completely fragmented and lysed elastic fibers suggestive of elastophagocytosis

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  Discussion Top


AEGCG is a rare clinical entity, first proposed by Hanke et al. in 1979.[1] They clinicopathologically correlated their 5 cases with atypical necrobiosis lipoidica of face and scalp,[2] Meischer's granuloma of the face and actinic granuloma,[3],[4] and put forward this as separate entity both clinically and histopathologically. Histopathology is characterized by elastophagocytosis of dermal elastic fibers by the multinucleated giant cells. The presence of horizontally oriented fragmented elastic fibers is a very characteristic feature. There is no necrobiosis or mucin deposition distinguishing thus from necrobiosis lipoidica and granuloma annulare.[2] The histopathological differential diagnosis and the differentiating salient features of AEGCG from others are discussed in brief [Table 1]. Clinically, it is more common among middle-aged Caucasian women, though men may be affected.[2] Mostly favors sun-exposed areas, but reports involving covered areas also exist indicating that other factors besides sunlight may be a part in their initiation. Ultraviolet light induces damage to dermal elastic fibers through matrix metalloproteinase enzyme 12. It is also proposed that damaged elastic fibers thus become antigenic eliciting an inflammatory response; the epitheloid cells and giant cells in the granuloma express 67 kDa elastin receptors.[6] Classical AEGCG presents as well-defined erythematous annular arciform serpiginous polycyclic plaque with indurated elevated borders and central atrophy. Single or multiple lesions may be seen. In addition to the classical annular form, AEGCG can present in nonannular forms like papules.[5] The earliest lesions are supposed to be papules which expand centrifugally and assume annular patterns as a result of photodamage. AEGCG is found to have causal association with conditions like diabetes mellitus,[7] various malignancies including acute myeloid leukemia,[8] T-cell leukemia,[9] sarcoidosis,[10] and monoclonal gammopathy.[11] Spontaneous resolution of lesions can occur. No specific treatment modalities are described. The main line of management is by topical and intralesional steroids, topical immunomodulators, and cryotherapy. Systemic therapy with antimalarials, oral retinoid, systemic steroids, and cyclosporine are considered in resistant cases.[12] Our patient was successfully treated with topical tacrolimus and systemic hydroxychloroquine.
Table 1: The histopathological differential diagnosis and the differentiating salient features of AEGCG from others

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  Conclusion Top


AEGCG is a rare disease with varied clinical morphology. The lesions are not limited to sun-exposed areas. Papular variant of AEGCG pose a great diagnostic challenge and can be confirmed only by the histopathological examination.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Hanke CW, Bailin PL, Roenigk HH Jr. Annular elastolytic giant cell granuloma. A clinicopathologic study of five cases and a review of similar entities. J Am Acad Dermatol 1979;1:413-21.  Back to cited text no. 1
[PUBMED]    
2.
Ko CJ, Glusac EJ, Shapiro PE. Noninfectious granulomas. In: Elder DE, Elenitsas R, Johnson BL, Murphy GF, Xu X, editors. Histopathology of the Skin. 10th ed. Philadelphia: Lippincott Williams and Wilkins; 2005. p. 361-87.  Back to cited text no. 2
    
3.
Dowling GB, Jones EW. Atypical (annular) necrobiosis lipoidica of the face and scalp. A report of the clinical and histological features of 7 cases. Dermatologica 1967;135:11-26.  Back to cited text no. 3
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4.
O'Brien JP. Actinic granuloma. An annular connective tissue disorder affecting sun- and heat-damaged (elastotic) skin. Arch Dermatol 1975;111:460-6.  Back to cited text no. 4
    
5.
Nakazato S, Fujita Y, Muramatsu K, Kikuchi K, Sato H, Shimizu H, et al. Acase of papular elastolytic giant cell granuloma: A mini-review of reported cases. Eur J Dermatol 2015;25:205-6.  Back to cited text no. 5
    
6.
Asahina A, Shirai A, Horita A, Saito I. Annular elastolytic giant cell granuloma associated with prostate carcinoma: Demonstration of human metalloelastase (MMP-12) expression. Clin Exp Dermatol 2012;37:70-2.  Back to cited text no. 6
[PUBMED]    
7.
Aso Y, Izaki S, Teraki Y. Annular elastolytic giant cell granuloma associated with diabetes mellitus: A case report and review of the Japanese literature. Clin Exp Dermatol 2011;36:917-9.  Back to cited text no. 7
[PUBMED]    
8.
Garg A, Kundu RV, Plotkin O, Aronson IK. Annular elastolytic giant cell granuloma heralding onset and recurrence of acute myelogenous leukemia. Arch Dermatol 2006;142:532-3.  Back to cited text no. 8
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9.
Kuramoto Y, Watanabe M, Tagami H. Adult T cell leukemia accompanied by annular elastolytic giant cell granuloma. Acta Derm Venereol 1990;70:164-7.  Back to cited text no. 9
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10.
Kurose N, Nakagawa H, Iozumi K, Nogita T, Furue M, Ishibashi Y, et al. Systemic elastolytic granulomatosis with cutaneous, ocular, lymph nodal, and intestinal involvement. Spectrum of annular elastolytic giant cell granuloma and sarcoidosis. J Am Acad Dermatol 1992;26:359-63.  Back to cited text no. 10
    
11.
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 2010;35:145-8.  Back to cited text no. 11
[PUBMED]    
12.
Wagenseller A, Larocca C, Vashi NA. Treatment of annular elastolytic giant cell granuloma with topical tretinoin. J Drugs Dermatol 2017;16:699-700.  Back to cited text no. 12
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