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Anais Brasileiros de Dermatologia

Print version ISSN 0365-0596On-line version ISSN 1806-4841

An. Bras. Dermatol. vol.93 no.5 Rio de Janeiro Sept./Oct. 2018

http://dx.doi.org/10.1590/abd1806-4841.20187796 

Letters

Multifocal cutaneous Rosai-Dorfman disease masquerading as lupus vulgaris in a child*

1Department of Dermatology, Dr Ram Manohar Lohia Hospital, New Delhi, India.

2Dermpath Laboratory, New Delhi, India.

Dear Editor,

A 13-year-old girl presented to us with red plaques and nodules on her chest and ears for the past two years. The lesions gradually increased in size and were asymptomatic. The patient was otherwise healthy and had no significant past medical history. General physical examination was normal, with no lymph node enlargement. Cutaneous examination revealed multiple infiltrative erythematous plaques, nodules and scars on the right breast surrounding an area of central atrophy and hypopigmentation, which occurred due to the intralesional triamcinolone injections received by the patient from a local practitioner (Figure 1). There was a reddish-yellow nodule of 1cm in size on the pinna of the left ear (Figure 2). There was no mucosal involvement.

Figure 1 Mul tiple erythema tous infiltrated plaques and no dules present on the right breast surrounding an area with atro phy, hypopig mentation and erythema 

Figure 2 Red dish-yellow no dule with telan giectasia present on the pinna of the left ear 

Figure 3 Dermal infiltrate composed of histiocytes, giant cells and numerous plasma cells. Emperipolesis of lymphocytes and plasma cells within histiocytes (Hematoxylin & eosin, x400) 

Routine biochemical tests were normal. Histopathological examination revealed a dense pandermal infiltrate composed of histiocytes and admixed plasma cells, neutrophils, lymphocytes and multinucleate giant cells. Histiocytes had abundant eosinophilic to pale cytoplasm and occasionally an inflammatory cell was present within the cytoplasm suggestive of emperipolesis (Figure 3). Mild fibrosis was present intervening the infiltrate. Stain for acid fast bacilli (AFB) was negative. Immunohistochemical staining revealed that the histiocytes were positive for S-100, CD68 and CD163. CD1a staining was negative. Fungal and AFB cultures were negative. Based on the above findings a diagnosis of cutaneous Rosai-Dorfman disease (RDD) was made. Patient was advised excision of the lesions.

RDD, originally known as sinus histiocytosis with massive lymphadenopathy, is a benign histiocytic proliferative disorder characterized clinically by a massive, painless, often bilateral cervical lymphadenopathy accompanied by fever, neutrophilia, anemia, raised erythrocyte sedimentation rate and polyclonal hypergammaglobulinemia.1 Extranodal involvement is seen in 43% of cases with skin being the most commonly affected site. The term cutaneous Rosai-Dorfman disease (CRDD) is used to describe the rare form of RDD which involves the skin exclusively with no involvement of lymph nodes or other sites.

The etiology of RDD remains unknown. It is believed that RDD represents an exaggerated immune response to an infectious agent due to the polyclonal nature of infiltrating cells and the clinical course of the disease. Epstein-Barr virus, human herpesvirus 6, Brucella and Klebsiella have been found to be associated with RDD, but no definitive link has been established as yet.

The histologic features in cutaneous RDD include a dermal infiltrate of large polygonal histiocytes with abundant pale to eosinophilic cytoplasm admixed with an infiltrate of lymphocytes and plasma cells. Emperipolesis, which is the presence of intact inflammatory cells like lymphocytes within the cytoplasm of histiocytes, is highly suggestive of the disease. The CRDD histiocytes stain positively for S100, CD 163 and CD68, but are generally negative for CD1a. This microscopic and immunohistochemical constellation confirms the diagnosis of CRDD. However, increased amounts of fibrosis, less prominent emperipolesis and fewer histiocytes in cutaneous lesions may make the diagnosis difficult.

Contrary to systemic RDD, which primarily involves children and young adults and has a male predominance (1.4:1), cutaneous RDD tends to occur at an older age (median age, 43.5 years) with a reversed male to female ratio (1:2) and has been rarely reported in children. Majority of RDD patients are of African American descent while CRDD most commonly affects Caucasian and Asian individuals. Clinically, it has a variable presentation with isolated or disseminated slow-growing asymptomatic brown or yellow-red papules, nodules, plaques or tumors. The most common sites involved are the trunk followed by the head and neck region, lower and upper extremities. Our patient was a child who presented with multifocal disease involving the right breast and pinna of left ear. As per our knowledge there are only 5 cases of pediatric CRDD without lymph node involvement reported previously and none of them have shown involvement of the pinna as seen in our patient (Table 1).1-5

Table 1. Cases of cutaneous Rosai-Dorfman disease reported in children  

Author Age, gender Ethnicity/ country Site Type of lesion Duration Laboratory findings Treatment Follow up
1 Al-Khateeb1 4, M Saudi Arabia Right paro­tid area Nodule 3 months Within normal limits Surgical excision No recur­rence
2 Kala et al2 10, M India Both eye­lids Diffuse swelling Few mon­ths Within normal limits - -
3 Mebazaa et al3 12, F Tunisia Cheeks, perioral region, upper trunk, lo­wer limbs Papules and nodules 3 months Complete blood count was normal, elevated ESR, polyclonal hypergammaglo­bulinemia Acitretin (20 mg daily for 4 months) and surgi­cal excision of the large lesions Improve­ment in lesions
4 Jat et al4 6, F India Eyelids Diffuse swelling 3 years Anemia, increased ESR, hypergammaglo­bulinemia Surgical excision No recur­rence
5 Castillo et al5 3, M Spain Upper lip, trunk, and limbs Papules 2 months Hypochromic microcytic anemia, high immunoglobulin A titers, slightly high ESR Wait-an- d-watch approach Resolu­tion of all lesions within 6 months
6 Gupta et al 13, F India Chest, ear Nodules, plaques 2 years Elevated ESR Plan for surgical excision No change in lesions since 6 months

CRDD can mimic histiocytoses, juvenile xanthogranuloma, sarcoidosis, lymphoproliferative disorders, tuberculosis, leishmaniasis and other infiltrative and infectious etiologies. In our patient there was a strong suspicion of lupus vulgaris. However, involvement of the ear aroused suspicion of infiltrative disorders like CRDD at the first visit. Other diseases involving the ear such as pseudolymphoma, leprosy, perichondritis and relapsing polychondritis were clinically ruled out in our case.

Most patients with CRDD have a self-limiting and benign clinical course and spontaneous resolution is frequent. Treatment is required only in symptomatic cases or those with disseminated disease. Corticosteroids, thalidomide, alkylating agents, retinoids, radiotherapy, and surgical excision have been used previously. Although progression to systemic disease has not been reported, nevertheless, follow-up of CRDD patients is recommended to exclude any possible recurrence or subsequent development of systemic disease.

*Work conducted at the Department of Dermatology, Dr Ram Manohar Lohia Hospital, New Delhi, India.

Financial support: None.

REFERENCES

1 Al-Khateeb TH. Cutaneous Rosai-Dorfman Disease of the Face: A Comprehensive Literature Review and Case Report. J Oral Maxillofac Surg. 2016;74:528-40. [ Links ]

2 Kala C, Agarwal A, Kala S. Extranodal manifestation of Rosai-Dorfman disease with bilateral ocular involvement. J Cytol. 2011;28:131-3. [ Links ]

3 Mebazaa A, Trabelsi S, Denguezli M, Sriha B, Belajouza C, Nouira R. Extensive purely cutaneous Rosai-Dorfman disease responsive to acitretin. Int J Dermatol. 2007;46:1208-10. [ Links ]

4 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. [ Links ]

5 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;32:249-50. [ Links ]

Received: October 29, 2017; Accepted: February 16, 2018

Mailing Address: Pooja Arora. E-mail: drpoojamrig@gmail.com

Conflict of interest: None.

AUTHORS'CONTRIBUTIONS

Aastha Gupta

0000-0002-4057-5365

Preparation and writing of the manuscript

Pooja Arora

0000-0002-0718-6990

Approval of the final version of the manuscript, Critical review of the literature

Meenakshi Batrani

0000-0002-8539-084X

Collecting, analysis and interpretation of data

Prafulla Kumar Sharma

0000-0001-9064-7032

Approval of the final version of the manuscript

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