|Year : 2018 | Volume
| Issue : 1 | Page : 51-53
Annular elastolytic giant cell granuloma: An atypical presentation
Vinitha Gopalakrishnan1, Thonduparambil Pappan Thankappan2, Gopalan Sulochana3
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 Publication||22-May-2018|
Dr. Vinitha Gopalakrishnan
3/96, Rajeevam, Puthukulangara, Edassery PO, Via Talikkulam, Thrissur - 680 569, Kerala
Source of Support: None, Conflict of Interest: None
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 2019 Feb 17];5:51-3. Available from: http://www.ijdpdd.com/text.asp?2018/5/1/51/232959
| Introduction|| |
Annular elastolytic giant cell granuloma (AEGCG) is a rare granulomatous disease with unknown pathogenesis., It is characterized by well-defined erythematous annular plaques in polycyclic or serpiginous pattern usually occurring in sun-exposed areas., There are very few reports of nonannular forms like papular variant of the disease. 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|| |
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 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|| |
AEGCG is a rare clinical entity, first proposed by Hanke et al. in 1979. They clinicopathologically correlated their 5 cases with atypical necrobiosis lipoidica of face and scalp, Meischer's granuloma of the face and actinic granuloma,, 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. 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. 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. 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. 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, various malignancies including acute myeloid leukemia, T-cell leukemia, sarcoidosis, and monoclonal gammopathy. 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. 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|| |
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.
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Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]