|Year : 2018 | Volume
| Issue : 2 | Page : 139-142
Spiegler–Fendt sarcoid/lymphocytoma cutis: A focus on nomenclature and diagnosis
Virendra N Sehgal1, Deepa Sehgal1, Jangid B Lal2, Sonal Sharma3
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 Publication||26-Mar-2018|
Virendra N Sehgal
Dermato-Venereology (Skin/VD) Center, Sehgal Nursing Home, Panchwati, New Delhi
Source of Support: None, Conflict of Interest: None
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 Oct 29];19:139-42. Available from: https://www.ijpd.in/text.asp?2018/19/2/139/211813
| Introduction|| |
Spiegler–Fendt sarcoid  is a fascinating caption, recognized as one of the extraordinary cutaneous, inflammatory clinical variants of sarcoidosis, characterized by a moderate-to-severe infiltration of lymphocytes in the dermis, simulating cutaneous lymphomas. Lymphocytoma cutis  (LC) is its other nomenclature. The entity has thus far been sparingly reported. 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|| |
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.
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)|
Click here to view
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.
A complete regression of lesion was experienced, following administration of hydroxychloroquine sulfate 100 mg twice a day in 10 days.
| Discussion|| |
Spiegler–Fendt sarcoid, also known as LC, is an inflammatory disorder of sarcoidosis, characterized by a moderate-to-severe infiltration of lymphocytes in the dermis, simulating cutaneous lymphomas. LC , 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, borrelial lymphocytoma, lymphadenosis benigna cutis,,, LC/pseudolymphoma,, pseudolymphoma of Spiegler–Fendt/sarcoidosis of Spiegler and Fendt, Spiegler–Fendt lymphoid hyperplasia, and Spiegler–Fendt sarcoid. Their clinical connotation, related features, and IHC are portrayed in [Table 1] and [Table 2].
|Table 2: Spiegler–Fendt sarcoid/lymphocytoma cutis: Differential diagnosis|
Click here to view
Several factors  such as insect bites, scabies, stings and spider bites, vaccinations, desensitization injections, trauma, acupuncture, gold earring piercing, infections with Borrelia burgdorferi (lyme disease),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), 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,, lupus érythémateux tumidus,,, and plaque-type polymorphous light eruption , are required to be considered in differential diagnosis, the salient clinical connotation, related features, and speculated IHC ,,,, 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 , 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
Conflicts of interest
There are no conflicts of interest.
| References|| |
Furmaniuk J, Owczarek L. Spiegler-Fendt sarcoid. Pol Tyg Lek 1971;26:1990-1.
Sehgal VN, Riyaz N, Chatterjee K, Venkatash P, Sharma S. Sarcoidosis as a systemic disease. Clin Dermatol 2014;32:351-63.
Shalom G, Gurion R, Benharroch D. Primary cutaneous γ/δ T-cell lymphoma. An atypical case with bone marrow granulomas. J Dermatol Case Rep 2015;9:15-8.
Orfuss AJ. Lymphocytic infiltration of the skin. AMA Arch Derm Syphilol 1953;68:447-9.
Oliveira EV, Badiale GB, Moraes MM. Lymphocytoma cutis – Case report. An Bras Dermatol 2013;88 6 Suppl 1:128-31.
van Vloten WA, Willemze R. The many faces of lymphocytoma cutis. J Eur Acad Dermatol Venereol 2003;17:3-6.
James WD, Berger TG, Elston DM, Odom RB. Andrews' Diseases of the Skin: Clinical Dermatology. 10th
ed. Philadelphia: Saunders Elsevier; 2006.
Sehgal VN, Khurana A. Lyme disease/borreliosis as a systemic disease. Clin Dermatol 2015;33:542-50.
Epstein E. Spiegler-Fendt (Lymphoblastomatous) Sarcoid. Dermatologica1940;81:236-44.
Vignali C. Spiegler-Fendt sarcoid (lymphadenosis benigna cutis). Rass Dermatol Sifilogr 1961;14:176-88.
Albrecht S, Hofstadter S, Artsob H, Chaban O, From L. Lymphadenosis benigna cutis resulting from Borrelia infection (Borrelia lymphocytoma). J Am Acad Dermatol 1991;24:621-5.
Brodell RT, Santa Cruz DJ. Cutaneous pseudolymphomas. Dermatol Clin 1985;3:719-34.
Bergman R. Pseudolymphoma and cutaneous lymphoma: Facts and controversies. Clin Dermatol 2010;28:568-74.
Arai E, Shimizu M, Tsuchida T, Izaki S, Ogawa F, Hirose T. Lymphomatoid keratosis: An epidermotropic type of cutaneous lymphoid hyperplasia: Clinicopathological, immunohistochemical, and molecular biological study of 6 cases. Arch Dermatol 2007;143:53-9.
Gougerot H, Burnier R. Lupus érythémateux “tumidus”. Bull Soc Fr Dermatol Syphiligr 1930;37:1291-2.
Choonhakarn C, Poonsriaram A, Chaivoramukul J. Lupus erythematosus tumidus. Int J Dermatol 2004;43:815-8.
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.
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.
Jansén CT. The natural history of polymorphous light eruptions. Arch Dermatol 1979;115:165-9.
Isedeh P, Lim HW. Polymorphous light eruption presenting as pinhead papular eruption on the face. J Drugs Dermatol 2013;12:1285-6.
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.
Nasser N, Nasser Filho N, Rosa TSC. Linfocitoma cutis no nariz. PE-338; 67. Congresso Brasileiro de Dermatologia; 2012 set 1-4; Rio de Janeiro, Brasil. Rio de Janeiro: Sociedade Brasileira de Dermatologia; 2012.
Duray PH. Histopathology of clinical phases of human Lyme disease. Rheum Dis Clin North Am 1989;15:691-710.
Wolf M. Lymphocytic infiltration of the face. Arch Dermatol 1957;75:136.
Jessner M, Kanof NB. Lymphocytic infiltration of the skin. Arch Dermatol
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.
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.
[Figure 1], [Figure 2]
[Table 1], [Table 2]