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 Table of Contents  
CASE REPORT
Year : 2018  |  Volume : 19  |  Issue : 2  |  Page : 139-142

Spiegler–Fendt sarcoid/lymphocytoma cutis: A focus on nomenclature and diagnosis


1 Dermato-Venereology (Skin/VD) Center, Sehgal Nursing Home, Panchwati, India
2 Department of Dermatology and Venereology, All Institute of Medical Sciences and Research, New Delhi, India
3 Department of Pathology, University College of Medical Science and Associated with Guru Teg Bahadur Hospital, New Delhi, India

Date of Web Publication26-Mar-2018

Correspondence Address:
Virendra N Sehgal
Dermato-Venereology (Skin/VD) Center, Sehgal Nursing Home, Panchwati, New Delhi
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijpd.IJPD_36_17

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  Abstract 


Spiegler–Fendt sarcoid/lymphocytoma cutis, presenting as an asymptomatic, slowly progressive, erythematous macule of the size of a guinea over the left cheek, is presented in a 7-year-old girl, the diagnosis of which was complimented by lymphocytic exocytosis with spongiosis of the epidermis. Immunohistochemistry revealed wide-ranging T-cell variations, which was more positive for T-cell markers, CD3, of which predominance of CD4 subset was seen, emphasizing its role as a diagnostic tool.

Keywords: Cutaneous lymphoid hyperplasia, Lymphocytoma cutis, Spiegler–Fendt sarcoid


How to cite this article:
Sehgal VN, Sehgal D, Lal JB, Sharma S. Spiegler–Fendt sarcoid/lymphocytoma cutis: A focus on nomenclature and diagnosis. Indian J Paediatr Dermatol 2018;19:139-42

How to cite this URL:
Sehgal VN, Sehgal D, Lal JB, Sharma S. Spiegler–Fendt sarcoid/lymphocytoma cutis: A focus on nomenclature and diagnosis. Indian J Paediatr Dermatol [serial online] 2018 [cited 2020 Jan 29];19:139-42. Available from: http://www.ijpd.in/text.asp?2018/19/2/139/211813




  Introduction Top


Spiegler–Fendt sarcoid [1] is a fascinating caption, recognized as one of the extraordinary cutaneous, inflammatory clinical variants of sarcoidosis,[2] characterized by a moderate-to-severe infiltration of lymphocytes in the dermis, simulating cutaneous lymphomas.[3] Lymphocytoma cutis [4] (LC) is its other nomenclature. The entity has thus far been sparingly reported.[5] Hence, it was thought worthwhile to report the same, focusing attention, in particular, to its diagnostic clinical criteria, supported by histopathology, immunohistochemistry (IHC)-prevalent nomenclature, and its differential diagnosis.


  Case Report Top


A 7-year-old girl was brought to the “outpatient clinic” with slowly progressive bizarre red eruption affecting the left cheek for 3 years. It had a spontaneous onset, without any apparent history of trauma, insect bites, or scabies. Examination of the skin surface revealed an erythematous macule of the size of a guinea, the British coin measuring 16.0 mm. The border of the lesion was serrated, with ill-defined edges [Figure 1]. Apple-jelly nodules could not be elicited on diascopy. Radiograph of the chest (anteroposterior) was normal and did not reveal any hilar lymphadenopathy. Mantoux tuberculin skin test was negative. Hemogram was within normal limits.
Figure 1: An erythematous macules with irregular borders

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Hematoxylin and eosin (H and E)-stained section(s) prepared from skin biopsy from the left cheek depicted lymphocytic exocytosis with spongiosis of the epidermis. The dermis showed dense infiltration, perifollicular lymphoplasmacytic infiltrate with histiocytes. Folliculotropism was also seen. No granuloma or parasite was identified in the sections examined. Aggregation of lymphocytes at the peripheral as well as interspersed lymphocytes was a peculiar feature [Figure 2]a,[Figure 2]b,[Figure 2]c,[Figure 2]d.
Figure 2: (a-d) Lymphocytic exocytosis with spongiosis of the epidermis, dense infiltration, perifollicular lymphoplasmacytic infiltrate with histiocytes folliculotropism (H and E, ×10, ×20, ×40)

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Physiologic factor analysis (PFA) and IHC undertaken on a fresh (exclusive) skin biopsy were found to be more positive for T-cell markers CD3, more of CD4 subset were seen. CD marker - CD20 was significant, but less in number. CD marker - CD3 dominance was another feature in the case.

Treatment

A complete regression of lesion was experienced, following administration of hydroxychloroquine sulfate 100 mg twice a day in 10 days.


  Discussion Top


Spiegler–Fendt sarcoid,[1] also known as LC,[2] is an inflammatory disorder of sarcoidosis,[3] characterized by a moderate-to-severe infiltration of lymphocytes in the dermis, simulating cutaneous lymphomas.[4] LC [1],[6] has a peculiar cutaneous presentation(s) identified by soft and doughy or firm nodules, of skin color, reddish-brown, or reddish-purple. Occasionally, the nodule has a scale or crust on top. They are neither itchy nor sore and are likely to occur on an exposed area, such as the face.

Spiegler–Fendt sarcoid is an intriguing entity; the evolving nomenclature of which is fascinating, for it may explain its clinicopathological spectrum. The condition has been identified by interchangeable names; cutaneous lymphoid hyperplasia,[7] borrelial lymphocytoma,[8] lymphadenosis benigna cutis,[9],[10],[11] LC/pseudolymphoma,[12],[13] pseudolymphoma of Spiegler–Fendt/sarcoidosis of Spiegler and Fendt, Spiegler–Fendt lymphoid hyperplasia, and Spiegler–Fendt sarcoid.[2] Their clinical connotation, related features, and IHC are portrayed in [Table 1] and [Table 2].
Table 1: Spiegler–Fendt sarcoid/lymphocytoma cutis nomenclature

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Table 2: Spiegler–Fendt sarcoid/lymphocytoma cutis: Differential diagnosis

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Several factors [22] such as insect bites, scabies, stings and spider bites, vaccinations, desensitization injections, trauma, acupuncture, gold earring piercing, infections with Borrelia burgdorferi (lyme disease),[23]Varicella zoster (chickenpox), and human immunodeficiency virus have been incriminated, warranting elucidation of history to establish its triggering or precipitating factor(s).

Its diagnosis is suggested by the clinical behavior of the lesion, characterized by small solitary lesion.

H and E-stained section(s) prepared from the skin biopsy is mandatory, wherein moderate-to-severe infiltration of lymphocytes in the dermis is a feature, which might be a pointer to its diagnosis. Furthermore, it is imperative to undertake an IHC (panel) to define and identify a mixture of T-cells (thymus cells) and B-cells (bone marrow or bursa-derived cells),[24] the major cellular components of the adaptive immune response. The demonstration of aforementioned cells is paramount to arrive at and supplement its tangible diagnosis and to determine the benign nature of Spiegler–Fendt sarcoid.

Furthermore, a few conditions such as Jessner's lymphocytic infiltrate of the skin,[25],[26] lupus érythémateux tumidus,[15],[16],[17] and plaque-type polymorphous light eruption [19],[20] are required to be considered in differential diagnosis, the salient clinical connotation, related features, and speculated IHC [14],[18],[21],[27],[28] are depicted in [Table 1].

PFA and IHC were intriguing in the current case, because of the wide-ranging pattern of T-cells, which are at variance with those reported [14],[28] earlier, warranting perspective studies on the subject recognized by interchangeable names/nomenclature.

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

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Kuhn A, Richter-Hintz D, Oslislo C, Ruzicka T, Megahed M, Lehmann P. Lupus erythematosus tumidus – A neglected subset of cutaneous Lupus erythematosus: Report of 40 cases. Arch Dermatol 2000;136:1033-41.  Back to cited text no. 17
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Alexiades-Armenakas MR, Baldassano M, Bince B, Werth V, Bystryn JC, Kamino H, et al. Tumid lupus erythematosus: Criteria for classification with immunohistochemical analysis. Arthritis Rheum 2003;49:494-500.  Back to cited text no. 18
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Kölgen W, van Meurs M, Jongsma M, van Weelden H, Bruijnzeel-Koomen CA, Knol EF, et al. Differential expression of cytokines in UV-B-exposed skin of patients with polymorphous light eruption: Correlation with Langerhans cell migration and immunosuppression. Arch Dermatol 2004;140:295-302.  Back to cited text no. 21
    
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Duray PH. Histopathology of clinical phases of human Lyme disease. Rheum Dis Clin North Am 1989;15:691-710.  Back to cited text no. 23
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B Cell”. Merriam-Webster Dictionary. Encyclopaedia Britannica. Available from: https://en.wikipedia.org/wiki/Lymphocyte#cite_ref-. [Last retrieved on 2011 Oct 28].  Back to cited text no. 24
    
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Wolf M. Lymphocytic infiltration of the face. Arch Dermatol 1957;75:136.  Back to cited text no. 25
    
26.
Jessner M, Kanof NB. Lymphocytic infiltration of the skin. Arch Dermatol 1953;68:447-9.  Back to cited text no. 26
    
27.
Dippel E, Poenitz N, Klemke CD, Orfanos CE, Goerdt S. Familial lymphocytic infiltration of the skin: Histochemical and molecular analysis in three brothers. Dermatology 2002;204:12-6.  Back to cited text no. 27
    
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Jaworsky C. Connective tissue diseases. In: Elder DE, Elenistas R, Johnson BL, Murphy GF, editors. Lever's Histopathology of the Skin. 9th ed. Philadelphia: Lippincott Williams & Wilkins; 2005. p. 304-5.  Back to cited text no. 28
    


    Figures

  [Figure 1], [Figure 2]
 
 
    Tables

  [Table 1], [Table 2]



 

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