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CASE REPORT
Year : 2018  |  Volume : 19  |  Issue : 4  |  Page : 366-368

Idiopathic localized involutional lipoatrophy in a child with spontaneous resolution


1 Department of Dermatology, Indira Gandhi Medical College, Shimla, Himachal Pradesh, India
2 Department of Dermatology, Regional Hospital, Bilaspur, Himachal Pradesh, India

Date of Web Publication28-Sep-2018

Correspondence Address:
Dr. Mudita Gupta
Department of Dermatology, Venereology and Leprosy, Indira Gandhi Medical College, Shimla, Himachal Pradesh
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijpd.IJPD_81_17

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  Abstract 


Idiopathic localized involutional lipoatrophy (ILIL) is focal loss of subcutaneous tissue without any clinical or histopathological inflammation. Histopathology of ILIL characteristically shows diminutive fat lobules composed of small adipocyte resembling embryonic fat tissue. Immunohistochemical examination reveals a number of CD-68 positive macrophages in the fat lobules. Most reported cases of ILIL were unilateral in young women; we are reporting a case of 4-year-old child with bilateral involvement showing quick spontaneous resolution.

Keywords: Idiopathic, involutional, lipoatrophy


How to cite this article:
Sharma RK, Gupta M, Sharma A. Idiopathic localized involutional lipoatrophy in a child with spontaneous resolution. Indian J Paediatr Dermatol 2018;19:366-8

How to cite this URL:
Sharma RK, Gupta M, Sharma A. Idiopathic localized involutional lipoatrophy in a child with spontaneous resolution. Indian J Paediatr Dermatol [serial online] 2018 [cited 2020 Oct 29];19:366-8. Available from: https://www.ijpd.in/text.asp?2018/19/4/366/220992




  Introduction Top


Idiopathic localized involutional lipoatrophy (ILIL) is a rare distinctive idiopathic form of localized lipoatrophy. It is characterized by specific focal loss of adipose tissue without antecedent inflammation. We are reporting a 4-year-old male child with bilateral involvement of buttocks with spontaneous resolution within 4 weeks. We are presenting this case because of the rarity of the disease, young age of onset, and quick spontaneous recovery.


  Case Report Top


A 4 year-old male child was brought to us with asymptomatic bilateral depression over buttocks, which they noticed 2 weeks before presentation. There was no history of any injury, injection at the site, drug intake, viral exanthem, and infection before the development of the condition. There was no history suggestive of any autoimmune connective tissue or endocrinal disease. Physical examination revealed nontender, unindurated, well-demarcated, depigmented, symmetrical, depressed plaque of size approximately 45 mm × 25 mm over the left side and 25 × 25 over the right gluteal region [Figure 1]a. Similar lesions were not seen elsewhere on the body or in any family member. The child developed itching after application of some indigenous medication followed by erosions. There was no personal or family history suggestive of diabetes. He was born at term by normal vaginal delivery and had normal milstones. Clinical diagnosis of idiopathic form of localized lipoatrophy with a differential diagnosis of lichen sclerosus et atrophicus, morphea, or lupus profundus was kept. Routine hematological and biochemical investigations were within normal limits. Fasting blood sugar, glycated hemoglobin, thyroid function tests, and antinuclear antibodies were normal. Test for human immunodeficiency virus was nonreactive in the child as well as in parents. Skin biopsy was sent for histopathological examination which showed unremarkable epidermis, sparse perivascular lymphohistiocytic infiltrate with hyalinization of dermis, and diminished fat lobules in subcutaneous tissue without inflammation or increased vascularity [Figure 2]a. Immunohistochemical staining showed CD-68-positive macrophages [Figure 2]b. Diagnosis of ILIL was made, and emollients were prescribed; the child was followed after 4 weeks. There was drastic regression of atrophic lesions bilaterally with repigmentation with residual postinflammatory marks (scratch marks and biopsy site) present [Figure 1]b.
Figure 1: (a) Depressed, depigmented plaques over bilateral gluteal region at the time of presentation. (b) Spontaneous resolution of bilateral depressed plaques with repigmentation after 4 weeks

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Figure 2: (a) Histopathological examination showing diminutive fat without evident inflammatory changes in subcutaneous tissue and hyalinization of the dermis (H and E, ×10). (b) Immunoperoxidase staining showing scattered CD-68 positive macrophages among the affected adipose tissue

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  Discussion Top


Lipodystrophy is heterogeneous disorder characterized by atrophy and infrequently hypertrophy of adipose tissue. Lipoatrophy refers specifically to selective loss of subcutaneous fat without exudative reaction or fibrosis. Both terms lipoatrophy and lipodystrophy are often used interchangeably.[1] Lipoatrophy may be acquired or inherited. Depending on the extent of involvement, acquired lipoatrophy may be generalized (Lawrence syndrome), partial (Barraquer–Simons syndrome), or localized. Acquired localized lipoatrophy can be caused due to various types of insult to subcutis, for example, repetitive trauma, pressure, abscess, localized connective tissue disease (lupus profundus, panniculitic lymphoma, morphea), and systemic autoimmune diseases (systemic lupus erythematosus, Sjogren's syndrome, dermatomyositis, nephritis, thyroiditis).[2] It may be iatrogenic due to injectables such as insulin (especially nonhuman form), corticosteroids, antibiotics, methotrexate, iron, heparin, vaccines, and growth factors.[1] There is no precise proven pathogenetic mechanism for localized lipoatrophy; however, literature suggests impairment of adipocyte differentiation, adipocyte apoptosis, and mitochondrial dysfunction, as the heterogeneous pathogenesis, reflecting the different subtypes.[3] Localized lipoatrophies with no particular etiology are labeled as idiopathic type. Clinically various types of idiopathic lipoatrophy (ILA) are annular type, abdominal, and semicircular lipoatrophy.[1]

Peters and Winkelmann first reported ILA in 1986. It was Dahl et al. in 1996 who observed the distinct histological pattern of adipocytes and classified the variant as ILIL.[4] About 60% of ILA are involutional where single sharply defined, depressed asymptomatic, annular, noninflammatory, nonindurated skin-colored, or depigmented plaque is seen.[1] Rarely, multiple lesions may be seen as in our case. Histologically, these lesions are marked by involution (simulating embryonic adipose tissue) which is characterized by small adipocytes embedded in hyaline connective tissue with many capillaries and non- or pauci-inflammatory cells in the subcutis.[5] Immunoreactants in ILIL are positive for macrophages CD-68 only and negative for T-cells and B-cells.[6] Rest 40% ILA can be classed as inflammatory where multiple lesions of localized lipoatrophy are seen. Histopathologically, the lipocytes are normal associated with diffuse infiltrate of lymphocytes, histiocytes, and plasma cells in the fat lobules; all show immunoreactants to macrophages, T-cell, and B-cell.

ILIL has more commonly been reported in young females. Only few reports are there in males, the cause of which is not clear. Childhood cases reported are rare. Sites most commonly involved are arms, buttocks, thighs, and abdomen. Most of the cases are sporadic except there are two reports of ILIL in siblings.[7] It has been reported with a duration of 2 weeks to an year before patient presents for medical examination, this can be due to asymptomatic nature of the entity. Macrophages are supposed to be involved in the process of localized involutional lipoatrophy. Electron microscopic analysis revealed macrophages becoming lipophages.[8] They secrete various cytokines such as transforming growth factor β, platelet-derived growth factor, interleukin-1, and tumor necrosis factor α, which lead to involution of subcutis.

Histopathologically, ILA has to be differentiated from morphea which shows much more pronounced inflammatory infiltrate in subcutaneous fat than in dermis. Furthermore, trabeculae subdividing the subcutaneous fat are thickened because of inflammation and deposition of new collagen.[9] In lichen sclerosus atrophicus epidermal atrophy, interface dermatitis and collagenization and marked edema in upper and mid dermis are seen and the subcutis is spared.

ILIL may be self-resolving as seen in our case. Anti-inflammatory agents such as steroids, calcineurin inhibitors, or antimalarials can be used in initial phase when lesion is expanding.[10] In long-standing lesions which persist, fillers can be used to improve cosmetic outcome.


  Conclusion Top


Idiopathic localized involution lipoatrophy is a rare entity. There are multiple dermatological diseases which may mimic ILIL. Diagnosis is usually made after excluding other causes of localized atrophy. Histopathology showing localized diminutive adipose tissue which gives clue to diagnosis, and immunohistochemistry showing only CD-68 positivity confirm the diagnosis. ILIL may resolve spontaneously.

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.
Gibbon DH. Sucbcutaneous fat. In: Burns T, Breathnach S, Cox N, Griffiths C, editors. Rook's Textbook of Dermatology. 8th ed. Oxford: Wiley-Blackwell; 2004. p. 46.34-46.42.  Back to cited text no. 1
    
2.
Soós N, Shakery K, Mrowietz U. Localized panniculitis and subsequent lipoatrophy with subcutaneous glatiramer acetate (Copaxone) injection for the treatment of multiple sclerosis. Am J Clin Dermatol 2004;5:357-9.  Back to cited text no. 2
    
3.
Giralt M, Domingo P, Villarroya F. Adipose tissue biology and HIV-infection. Best Pract Res Clin Endocrinol Metab 2011;25:487-99.  Back to cited text no. 3
    
4.
Dahl PR, Zalla MJ, Winkelmann RK. Localized involutional ipoatrophy: A clinicopathologic study of 16 patients. J Am Acad Dermatol 1996;35:523-8.  Back to cited text no. 4
    
5.
McNutt NS, Moreno A, Contreras F. Inflammatory diseases of subcutaneous fat. In: Elder DE, Elenitsas R, Johnson BL. Lever's Histopathology of the Skin. 10th ed. New Delhi: Wolter Kluwer; 2009. p. 509-38.  Back to cited text no. 5
    
6.
Yamamoto T, Yokozeki H, Nishioka K. Localized involutional lipoatrophy: Report of six cases. J Dermatol 2002;29:638-43.  Back to cited text no. 6
    
7.
Cheng-Huang C, Kan-Tang F, Song-Jen H. Morphea-like localized involutional lipoatrophy - A case report associated with family history. Dermatol Sin 2010;28:113-6.  Back to cited text no. 7
    
8.
Zalla MJ, Winkelmann RK, Gluck OS. Involutional lipoatrophy: Macrophage-related involution of fat lobules. Dermatology 1995;191:149-53.  Back to cited text no. 8
    
9.
Shen LY, Edmonson MB, Williams GP, Gottam CC, Hinshaw MA, Teng JM, et al. Lipoatrophic panniculitis: Case report and review of the literature. Arch Dermatol 2010;146:877-81.  Back to cited text no. 9
    
10.
Pai K, Pai S. Localised involutional lipoatrophy: A case report. Our Dermatol Online 2013;4:356-7.  Back to cited text no. 10
    


    Figures

  [Figure 1], [Figure 2]



 

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