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CASE REPORT
Year : 2021  |  Volume : 22  |  Issue : 3  |  Page : 241-243

An unusual presentation of cutaneous Rosai Dorfman Disease in a child


1 Department of Dermatology, Gauhati Medical College and Hospital, Guwahati, Assam, India
2 Unipath Specialty Laboratory, Ahmedabad, Gujarat, India

Date of Submission06-May-2020
Date of Decision27-May-2020
Date of Acceptance30-Mar-2021
Date of Web Publication30-Jun-2021

Correspondence Address:
Seujee Das
Department of Dermatology, Gauhati Medical College and Hospital, Guwahati - 781 032, Assam
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijpd.IJPD_78_20

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  Abstract 


Introduction: Rosai-dorfman disease (RDD) is a self-limiting, non-Langerhans cell histiocytosis of unknown etiology. It was originally thought to involve only the lymph nodes, however recent literature suggests extranodal involvement as wel. Case Report: Herein we report a case of cutaneous RDD in a six year-old girl, presenting with multiple subcutaneous nodules. Discussion: Cutaneous RDD is an extremely rare entity and unlike classic RDD, it has a predilection for the age group above 40 years and is more common in females. It is extremely rare in children.

Keywords: Child; Histiocytosis, Sinus; Dermatology; Emperipolesis; Histiocytes


How to cite this article:
Das S, Adhicari P, Dutta B, Jajodia E. An unusual presentation of cutaneous Rosai Dorfman Disease in a child. Indian J Paediatr Dermatol 2021;22:241-3

How to cite this URL:
Das S, Adhicari P, Dutta B, Jajodia E. An unusual presentation of cutaneous Rosai Dorfman Disease in a child. Indian J Paediatr Dermatol [serial online] 2021 [cited 2021 Jul 24];22:241-3. Available from: https://www.ijpd.in/text.asp?2021/22/3/241/319953




  Introduction Top


 Rosai-Dorfman Disease More Details (RDD) is a rare, idiopathic, benign, polyclonal proliferation of the histiocytes originally known to involve only the lymph nodes. It is also known as sinus histiocytosis with massive lymphadenopathy. However, the reports of extranodal involvement in the literature warrant it to be termed as “Rosai-Dorfman disease” more appropriately.

RDD involves lymph nodes as well as extranodal organs among which skin is the most commonly involved, but exclusive involvement of the skin is an extremely rare occurrence. Around 43% of the cases with RDD have extranodal involvement, of which 11% cases have skin involvement, whereas 3% of the cases are reported to have solely cutaneous involvement.[1]

Cutaneous RDD is now considered to be a distinct entity with isolated skin involvement without any lymphadenopathy or systemic symptoms. We report a case of cutaneous RDD in a child presenting with multiple subcutaneous nodules.


  Case Report Top


A 6-year-old girl presented to us with multiple asymptomatic subcutaneous nodules over bilateral lower eyelids, left cheek, upper abdomen, bilateral buttocks, and left anterior thigh [Figure 1]. The nodules developed insidiously, were progressive in nature and were gradually increasing in size for the past eight months. On cutaneous examination, the nodules were nontender, varied in size from 1 to 3 cm in diameter, were discrete, well defined and immobile, firm in consistency, and not fixed to the overlying skin. The overlying skin appeared normal without any change in color or texture. No lymphadenopathy was found. History of fever or constitutional symptoms was absent.
Figure 1: Subcutaneous nodules on bilateral lower eyelids

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Routine blood, urine, and biochemical investigations were normal, except for microcytic, hypochromic anemia, and a raised erythrocte sedimentation rate (ESR) (65 mm). Chest X-ray and abdominal ultrasonography were normal. Mantoux test was negative. Fine-needle aspiration cytology from the subcutaneous nodule in the gluteal area from the lesion in the buttock predominantly showed histiocytes with plenty of plasma cells and lymphocytes. Few of the histiocytes showed emperipolesis.

Histopathological examination was done after the excision of the subcutaneous nodule over the left anterior thigh which showed an unencapsulated mass consisting of numerous lymphoid follicles. Surrounding areas showed sheets of histiocytes having bland nuclei and abundant foamy cytoplasm along with fibrocollagenous tissue and vascular fragments. Occasional histiocytes showed the presence of intact lymphocytes and plasma cells in its cytoplasm suggesting emperipolesis [Figure 2]. The presence of numerous foci of plasma cell aggregates was also noted. Periodic acid-Schiff stain, Gram stain, and Fite stain were all negative. Immunohistochemistry studies could not be done due to affordability issues. Thus, taking into consideration, the cutaneous, systemic, laboratory, and histopathological findings, a diagnosis of cutaneous RDD was made.
Figure 2: (a) Section from the subcutaneous nodule showing lymphoid follicles surrounded by sheets of histiocytes with fibrocollagenous tissue and vascular fragments (H and E, ×100); (b) Sheets of histiocytes with one histiocyte showing features suggesting emperipolesis (arrow) (H and E, ×200); (c) Higher magnification shows a histiocyte with eccentrically placed vesicular nucleus and abundant pale eosinophilic cytoplasm containing intact lymphocytes and plasma cell confirming emperipolesis (H and E, ×400)

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The patient was started on oral prednisolone (0.5 mg/kg) with a significant decrease in the size of the lesions after one month. Thereafter, the patient was lost to follow-up.


  Discussion Top


RDD was first described by Destombes in 1965 but was first recognized as a distinct clinicopathological entity by Juan Rosai and Ronald F. Dorfman in 1969.[2] It is a self-limiting, non-Langerhans cell histiocytosis of unknown etiology occurring in any age group with a predilection for young adult males, presenting with painless massive cervical lymphadenopathy that is mostly bilateral. Although constitutional symptoms and laboratory abnormalities such as leucocytosis, raised ESR, and polyclonal hypergammaglobulinemia are common in systemic RDD, they may not be present in purely cutaneous RDD.

Cutaneous RDD is an extremely rare entity and unlike classic RDD, it has a predilection for the age group above 40 years and is more common in females. It is extremely rare in children with only a few cases being reported[3],[4] previously as per our literature search.

Cutaneous RDD may present as asymptomatic yellowish red or erythematous or brownish papules, plaques, nodules, or a combination of these. The most common site involved is the face, while trunk, upper, and lower extremities may also be involved. Lesions may be solitary or multiple; may occasionally occur in clusters or with satellite lesions. Kong et al. described three main types of skin lesions, i.e., papulonodular type (79.5%), indurated plaque type (12.8%), and tumor type (7.7%).[5] Cutaneous RDD resembling acne, psoriasis, and eruptive xanthoma has been reported.[6],[7] Few cases are known to have presented with subcutaneous nodules.[1],[8]

Histopathologically, there is a dense histiocytic infiltrate with plasma cells and lymphocytes. “Rosai-Dorfman cells” are characteristic, which are large foamy polygonal histiocytes with pale eosinophilic cytoplasm and having vesicular nuclei with feathery or indistinct cell borders. They exhibit the phenomenon of emperipolesis which represents the presence of intact lymphocytes, plasma cells or rarely neutrophils, and red blood cells engulfed within the histiocytes. These histiocytes are strongly reactive for S-100 protein and variably positive for CD68 but are negative for CD1a. Lymphoid follicules with germinal centers may occur in cutaneous RDD as in our case.[8]

Although the exact etiology of this disease remains unknown, literature suggests an immune dysfunction to an antigen or infectious agents such as human herpesvirus 6, Epstein–Barr virus, parvovirus B19, herpes simplex virus,  Brucella More Details, Klebsiella rhinoscleromatis, and Nocardia; on the basis of temporal association and reactive serology in some affected patients.[1] Association with other diseases such as bilateral uveitis, antinuclear antibody positive lupus erythematosus, rheumatoid arthritis, hypothyroidism, lymphoma, and HIV infection has been noted.[9]

The prognosis of cutaneous RDD is good and often follows a benign course with a strong possibility of spontaneous remission occurring over months to years. Single and isolated skin lesions can be excised and generalized lesions may require systemic treatment. Cryotherapy, local radiation, topical and intralesional corticosteroids can be used for limited number of lesions. Systemic corticosteroids, thalidomide, dapsone, isotretinoin, and imatinib are other treatment modalities with varying response.[9]

The fact that our case presented solely with multifocal subcutaneous nodules and that too in a 6-year-old child complicated the diagnosis of cutaneous RDD. A rare clinical presentation at an age much earlier than previously reported is what makes our case worth reporting. Thus, it is important to consider cutaneous RDD as a differential diagnosis in a child presenting with subcutaneous nodules. A high index of suspicion along with histopathology and immunohistochemistry is thereby very essential for the early diagnosis of this disease as it may present with nonspecific and a wide array of cutaneous lesions and in any age group.

Declaration of consent

The authors certify that they have obtained all appropriate consent forms, duly signed by the parent(s) of the patient. In the form the parent(s) has/have given his/her/their consent for the images and other clinical information of their child to be reported in the journal. The parents understand that the names and initials of their child 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.
Lu CI, Kuo TT, Wong WR, Hong HS. Clinical and histopathologic spectrum of cutaneous Rosai-Dorfman disease in Taiwan. J Am Acad Dermatol 2004;51:931-9.  Back to cited text no. 1
    
2.
Wang KH, Chen WY, Liu HN, Huang CC, Lee WR, Hu CH. Cutaneous Rosai-Dorfman disease: Clinicopathological profiles, spectrum and evolution of 21 lesions in six patients. Br J Dermatol 2006;154:277-86.  Back to cited text no. 2
    
3.
Jat KR, Panigrahi I, Srinivasan R, Singh U, Vasishta RK, Sharma N, et al. Cutaneous Rosai-Dorfman disease: Presenting as massive bilateral eyelid swelling. Pediatr Dermatol 2009;26:633-5.  Back to cited text no. 3
    
4.
Toledo del Castillo B, Mata-Fernández C, Rodríguez Soria VJ, Parra Blanco V, Loughlin G, Campos-Domínguez M. Self-healing extranodal cutaneous Rosai-Dorfman in a child. Pediatr Dermatol 2015;32:e249-50.  Back to cited text no. 4
    
5.
Kong YY, Kong JC, Shi DR, Lu HF, Zhu XZ, Wang J, et al. Cutaneous rosai-dorfman disease: A clinical and histopathologic study of 25 cases in China. Am J Surg Pathol 2007;31:341-50.  Back to cited text no. 5
    
6.
Uniyal SK, Beena KR, Ramesh V, Mukherjee A. Cutaneous Rosai-Dorfman disease preceding inguinal lymphadenopathy. Int J Dermatol 2002;41:404-6.  Back to cited text no. 6
    
7.
El-Kamel MF, Selim MK, Gawad MMA. A new presentation of isolated cutaneous Rosai-Dorfman disease: Eruptive xanthoma-like lesions. Indian J Dermatol Venereol Leprol 2020;86:158-61.  Back to cited text no. 7
[PUBMED]  [Full text]  
8.
Pitamber HV, Grayson W. Five cases of cutaneous Rosai-Dorfman disease. Clin Exp Dermatol 2003;28:17-21.  Back to cited text no. 8
    
9.
Fang S, Chen AJ. Facial cutaneous Rosai-Dorfman disease: A case report and literature review. Exp Ther Med 2015;9:1389-92.  Back to cited text no. 9
    


    Figures

  [Figure 1], [Figure 2]



 

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