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CASE REPORT
Year : 2022  |  Volume : 23  |  Issue : 1  |  Page : 55-57

Cutaneous sarcoidosis masquerading as granuloma annulare


1 Department of Paediatric Dermatology, Cutis Academy of Cutaneous Sciences, Bengaluru, Karnataka, India
2 Department of Pediatric Dermatology, Institute of Child Health, Kolkata, West Bengal, India
3 Department of Pathology, Wizdermpath Lab, Kolkata, West Bengal, India

Date of Submission23-Jun-2021
Date of Acceptance14-Sep-2021
Date of Web Publication31-Dec-2021

Correspondence Address:
Rashmi Agarwal
Cutis Academy of Cutaneous Sciences, Bengaluru, Karnataka
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijpd.ijpd_97_21

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  Abstract 


Sarcoidosis always remains a diagnostic challenge due to its great imitating potential. Propensity to involve important organ systems such as lung points toward the importance of detailed evaluation and follow-up at regular intervals. It can present with a myriad of morphological forms. Isolated cutaneous involvement of sarcoidosis presenting as granuloma annulare (GA) like lesions have rarely been reported in children. Herein, we report a child with cutaneous sarcoidosis presenting as asymptomatic annular plaque on the face resembling GA without any systemic involvement. Our case has novelty from several aspects: presentation during childhood, isolated facial involvement, and morphological resemblance with GA.

Keywords: Children, granuloma annulare, sarcoidosis


How to cite this article:
Agarwal R, Saha A, Dhar S. Cutaneous sarcoidosis masquerading as granuloma annulare. Indian J Paediatr Dermatol 2022;23:55-7

How to cite this URL:
Agarwal R, Saha A, Dhar S. Cutaneous sarcoidosis masquerading as granuloma annulare. Indian J Paediatr Dermatol [serial online] 2022 [cited 2022 Jan 16];23:55-7. Available from: https://www.ijpd.in/text.asp?2022/23/1/55/334683




  Introduction Top


Sarcoidosis, a granulomatous multisystem disorder of unknown etiology, is relatively uncommon in the pediatric population. The exact prevalence of childhood sarcoidosis in India is not exactly known because of the paucity of literature. The most involved organs are skin, lung, lymph node, eye, and bone. Skin involvement is seen in 20%–35% cases of systemic sarcoidosis.[1] Being a great mimic, a vast number of morphological forms have been described in sarcoidosis. One of the authors had reported a case of cutaneous sarcoidosis which masqueraded as relapsed borderline tuberculosis (TB) more than two and half decades ago.[2] Isolated cutaneous involvement is rare and sarcoidosis presenting as granuloma annulare (GA)-like lesions have rarely been reported in children.[3],[4] Herein, we report a child with cutaneous sarcoidosis presenting as asymptomatic annular plaque on the face resembling GA without any systemic involvement.


  Case Report Top


An 8-year-old girl presented with an asymptomatic annular lesion over her right jaw for the past 7 months. There was no history of pain, fever, cough, weight loss, night sweat, and respiratory or visual disturbances. There was no history of recent or chronic drug intake. Family history was unremarkable.

On examination, an erythematous, firm, smooth, nontender erythematous annular plaque measuring approximately 2 cm × 3 cm was observed over the right jaw with raised borders and clear central area [Figure 1]. It started as a small papule, and then subsequently attained the present size. A provisional diagnosis of GA was made. Other differentials contemplated were lupus vulgaris, subacute cutaneous lupus erythematosus. The child's developmental milestones and vital signs were within the normal limits. There was no lymphadenopathy. Systemic examinations were normal. Immunization status was up to date.
Figure 1: An 8-year-old girl with firm smooth erythematous annular plaque having raised borders and clear central area on the right jaw

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A biopsy taken from the site was suggestive of cutaneous sarcoidosis. Skin histopathology (H and E stain) showed unremarkable epidermis, aggregates of epithelioid histiocytes in mid to deep dermis, few large, irregular giant cells with nuclei arranged in the periphery along with asteroid bodies, and a few lymphocytes around the aggregate suggestive of sarcoidosis [Figure 2] and [Figure 3]: H and E ×40, H and E ×400, respectively). Special stains such as Ziehl–Neelsen stain and periodic acid-fast stain failed to show any organism. TB polymerase chain reaction of tissue samples was negative.
Figure 2: Histopathological examination (H and E stain; ×40 magnification) from the annular plaque showing multiple epitheloid cell granulomas present extensively in the dermis

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Figure 3: Histopathological examination (H and E stain; ×400 magnification) of Langhans giant cell lying amid epitheloid cells. Asteroid body seen in the cytoplasm of the giant cell

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Complete blood count, erythrocyte sedimentation rate, routine urine examination, serum calcium, C reactive protein and angiotensin converting enzyme (ACE) levels were within normal limits. Chest X-ray and pulmonary function test did not reveal any abnormality. The ophthalmological evaluation was normal. Serology for syphilis, hepatitis, and HIV was negative.

The patient was treated with topical mid-potent steroid cream (mometasone furoate 0.01%) for 3 weeks, followed by topical pimecrolimus 1% cream for 6 weeks following which there was complete resolution of the lesion with mild hypopigmentation and atrophy [Figure 4]. The patient has been coming for follow-up every 3 months for the past 12 months without any evidence of recurrence.
Figure 4: Complete resolution of the plaque with mild hypopigmentation and atrophy after 2 months of topical therapy

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  Discussion Top


Sarcoidosis is known as a great imitator due to its extreme variability in the cutaneous manifestations. Infiltration of tissues with noncaseating granulomas is the hallmark feature of this disease. It is mainly a disease of adults with bimodal age distribution.[5] Childhood sarcoidosis has two distinct forms: early-onset sarcoidosis (occurs in children <4-year-old characterized by arthritis, uveitis, and cutaneous involvement) and late-onset sarcoidosis (more common form occurring mostly in patients aged 13–15 years and presenting a picture similar to that of adults, with frequent hilar lymphadenopathy and pulmonary infiltration). Cutaneous involvement may be more common in children than in adults. The skin is involved in approximately 80% of children younger than 5 years of age[6] and between 24% and 40% of older children with sarcoidosis.[7] Red to yellow–brown or violaceous flat-topped papules on the face with a waxing and waning course are the most frequently encountered specific cutaneous lesions and similar larger plaques may develop on the trunk, extremities, and buttocks. Other specific cutaneous lesions reported include nodules, hyperpigmented or hypopigmented lesions, ulcers, subcutaneous tumors, scar sarcoidosis, lupus pernio, erythrodermic, annular, ichthyosiform and lichenoid forms, subcutaneous tumors, alopecia, and livedo reticularis-like eruption.[8],[9],[10] Nonspecific lesions such as erythema nodosum, Sweet's disease, pyoderma gangrenosum, erythema multiforme, prurigo, calcifications, and nail clubbing are less frequent and may occur alone or coexist with specific lesions of sarcoidosis. Plaques and annular lesions are more likely to be associated with systemic manifestations.[11] Isolated cutaneous sarcoidosis is itself an uncommon phenomenon with an annular or serpiginous lesion on the face being a rarer observation and therefore can easily be confused with other entities such as GA, Hansen's disease, porokeratosis, subacute cutaneous lupus erythematosus, herald patch of pityriasis rosea, lupus vulgaris, and annular lichen planus, in the absence of systemic features.[3] Kwon et al. reported the occurrence of childhood sarcoidosis with atypical skin lesions resembling GA and histologic findings predominantly of a palisading granulomatous process with features of GA, palisading neutrophilic and granulomatous dermatitis and interstitial granulomatous dermatitis, and rarely, sarcoid-like naked epithelioid cell granulomas. However, the associated characteristic findings of uveitis and increased ACE levels confirmed the diagnosis of sarcoidosis.[4] Kumar et al. described isolated facial cutaneous lesion presenting as atypical annular plaque in sarcoidosis in a child.[3] Our patient presented with a single asymptomatic annular plaque on the face resembling GA. The diagnosis of sarcoidosis was made based on specific histopathological features, i.e. the presence of noncaseating granulomas with sparse, peripheral lymphocytic infiltrates. Although these features are considered characteristic of sarcoidosis, similar histopathologic features may be seen in other disorders. Further evaluation did not reveal any systemic involvement, but considering the future risk of organ involvement, the child was asked for regular follow-ups. In children, sarcoidosis may be self-limited, resolving over 2–3 years. Treatment of cutaneous sarcoidosis depends on the severity and extent of treatment, as well as on the site of involvement. Topical and or intralesional steroids are the first choice of treatment for localized skin involvement along with the incorporation of topical calcineurin inhibitors to maintain remission. Medium potency steroids should be considered for use on facial, genital, and intertriginous lesions, as there is increased risk for corticosteroid-induced skin atrophy in these areas.[12]

Actually, sarcoidosis is a diagnosis of exclusion. Compatible clinical and pathological pictures and exclusion of other close differentials clinche the diagnosis. In conclusion, sarcoidosis remains a diagnostic challenge to treating physicians and could present in any form we can think of. Although rare, pediatric patients should not be exempted from suspicion. GA-like lesions could be considered one of the specific manifestations. Finally, the necessity of long-term follow-up in a case of cutaneous sarcoidosis is not to be overemphasized.

Declaration of patient consent

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

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Samtsov AV. Cutaneous sarcoidosis. Int J Dermatol 1992;31:385-91.  Back to cited text no. 1
    
2.
Kaur S, Dhar S, Bambery P, Kanwar AJ, Khajuria A. Cutaneous sarcoidosis masquerading as relapsed borderline tuberculoid leprosy? Int J Lepr Other Mycobact Dis 1993;61:455-8.  Back to cited text no. 2
    
3.
Kumar S, Garg R, Aggarwal S, Kaur J. Isolated facial cutaneous sarcoidosis. J Nat Sci Biol Med 2012;3:87-9.  Back to cited text no. 3
    
4.
Kwon EJ, Hivnor CM, Yan AC, Forbes B, Elenitsas R, Albert D, et al. Interstitial granulomatous lesions as part of the spectrum of presenting cutaneous signs in pediatric sarcoidosis. Pediatr Dermatol 2007;24:517-24.  Back to cited text no. 4
    
5.
Henke CE, Henke LK, Eveheck CM. The epidemiology of sarcoidosi in Rochester MN: A population based study of incidence and survival. Am J Epidemiol 1986;123:840-5.  Back to cited text no. 5
    
6.
Wong L, Ho J, Yen Y. Early-onset childhood sarcoidosis: A case report. Dermatologicasinica 2011;29:125-28.  Back to cited text no. 6
    
7.
James DG, Kendig EL Jr. Childhood sarcoidosis. Sarcoidosis 1988;5:57-9.  Back to cited text no. 7
    
8.
Shetty AK, Gedalia A. Childhood sarcoidosis: A rare but fascinating disorder. Pediatr Rheumatol Online J 2008;6:16.  Back to cited text no. 8
    
9.
Alnami A, Alkhayal N, Alkhodair R. A rare manifestation of systemic sarcoidosis with livedo reticularis-like eruption in a pediatric patient: A case report. JAAD Case Rep 2019;5:392-4.  Back to cited text no. 9
    
10.
Seo SK, Yeum JS, Suh JC, Na GY. Lichenoid sarcoidosis in a 3-year-old girl. Pediatric Dermatol 2001;18:384-7.  Back to cited text no. 10
    
11.
Kang MJ, Kim HS, Kim HO, Park YM. Cutaneous sarcoidosis presenting as multiple erythematous macules and patches. Ann Dermatol 2009;21:168-70.  Back to cited text no. 11
    
12.
Prystowsky S, Sanchez M. Cutaneous Sarcoidosis: Management. Uptodate; 2021. Available from: https://www.uptodate.com/contents/cutaneous-sarcoidosis-management?search=cutaneous%20sarcoidosis&source=search_result&selectedTitle=2~47&usage_type=default&display_rank=2. [Last accessed on 2021 May 17].  Back to cited text no. 12
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4]



 

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