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 Table of Contents  
LETTER TO EDITOR
Year : 2020  |  Volume : 6  |  Issue : 2  |  Page : 91-93

Successful treatment of an uncommon disorder: Letterer-siwe disease


Department of Dermatology, Venereology and Leprology, Government Medical College, Nagpur, Maharashtra, India

Date of Submission24-Aug-2019
Date of Decision07-Aug-2020
Date of Acceptance19-Nov-2020
Date of Web Publication28-Dec-2020

Correspondence Address:
Vaishali H Wankhade
Department of Dermatology, Venereology, Leprosy, Government Medical College and Hospital, Nagpur-440 003, Maharashtra
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijdd.ijdd_49_19

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How to cite this article:
Supekar BB, Ghanate TD, Wankhade VH, Singh RP. Successful treatment of an uncommon disorder: Letterer-siwe disease. Indian J Drugs Dermatol 2020;6:91-3

How to cite this URL:
Supekar BB, Ghanate TD, Wankhade VH, Singh RP. Successful treatment of an uncommon disorder: Letterer-siwe disease. Indian J Drugs Dermatol [serial online] 2020 [cited 2021 Jan 16];6:91-3. Available from: https://www.ijdd.in/text.asp?2020/6/2/91/305127



Sir,

Langerhans cell histiocytosis (LCH) is a group of rare diseases, characterized by idiopathic monoclonal infiltration of Langerhans cells in different organs such as the skeleton, skin, hematopoietic system, lymph nodes, liver, spleen, lungs, and central nervous system.[1] Here, we report a case of LCH diagnosed clinically and histopathology, who showed an excellent response to treatment as per Histiocyte Society LCH Trial III after 1 year.

An 11-month-old female child born out of a nonconsanguineous marriage referred from the pediatric department with complaints of progressive reddish raised lesions over the scalp and trunk for 5 months. Her antenatal, postnatal history, and developmental milestones were normal. The child had a history of poor feeding, failure to thrive for 7 months with abdominal distention for the past 1 month, for which she was admitted to the pediatric department. At the time of admission, her weight was 6.07 kg ( less than 3 standard deviation[SD]), height was 67 cm (-3SD and −2SD), head circumference was 41 cm (−3SD and −2SD), and BMI was13.52 kg/m2, and was diagnosed with a case of severe malnutrition. On systemic examination, there was pallor and shifting dullness suggestive of moderate ascites with hepatosplenomegaly. Bilateral basal crepitations were heard. On cutaneous examination, there were multiple erythematous papules, plaques with greasy scales, and hypopigmented macules distributed over the scalp, chest, abdomen, and back. Multiple petechiae were present over palms and soles [Figure 1]a, [Figure 1]b, [Figure 1]c, [Figure 1]d. The rest of the cutaneous examination was normal. Routine investigations revealed anemia thrombocytopenia, raised prothrombin time (PT), and activated partial thromboplastin time, hypoproteinemia, and raised alkaline phosphatase. Peripheral blood smear revealed moderate hypochromia, target cells, and teardrop cells, and suggestive dimorphic anemia. Ultrasonography of the abdomen revealed moderate hepatosplenomegaly with mild-to-moderate ascites. X-ray of the skull and chest revealed single punched out lesion and pulmonary infiltrates, respectively [Figure 2]a and [Figure 2]b. Abdominal X-ray revealed moderate ascites with increased hepatic and splenic shadow [Figure 2]c. Histopathological examination from the lesion over trunk revealed infiltration of histiocytes, eosinophils, lymphocytes and neutrophils, and large mononuclear histiocytes seen with reniform cleaved nuclei and abundant eosinophilic cytoplasm in the dermis, suggestive of LCH [Figure 3]a and [Figure 3]b. Based on history, clinical histopathological findings, hematological, and radiological investigations a diagnosis of LettererSiwe disease was reached. Immunohistochemistry (IHC) and electron microscopy were not done due to financial constraints. Our patient belonged to Group I (multisystem risk patients) as per risk group according to Histiocyte Society LCH Trial III and was treated according to guidelines [Table 1]. At the end of 6 weeks, there were complete resolution of skin lesions over the scalp, trunk, palms, and soles [Figure 4]a, [Figure 4]b, [Figure 4]c, [Figure 4]d. There was also improvement in hematological parameters [Table 2] and a decrease in the size of the liver and spleen and ascitic fluid on ultrasonography. The child was on regular follow-up in the pediatric and dermatology outpatient department.
Figure 1: Multiple erythematous papules, plaques with greasy scales, and hypopigmented macules distributed over the scalp (a), chest and back (b). Multiple petechiae were present over palms and soles (c and d)

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Figure 2: X-ray skull and chest revealed punch out lesion (a) and pulmonary infiltrates (b) respectively. Abdominal X-ray revealed moderate ascites with increased hepatic and splenic shadow (c)

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Figure 3: (a and b) Histopathological examination from the lesion over trunk revealed, infiltration of histiocytes, eosinophils, lymphocytes, and neutrophils, and large mononuclear histiocytes seen with reniform cleaved nuclei and abundant eosinophilic cytoplasm in the dermis, suggestive of Langerhans cell histiocytosis (a: H and E, ×4, b: ×10x)

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Figure 4: At the end of 6 weeks, there were complete resolution of skin lesions over the scalp (a), trunk (b), palms (c), and soles (d)

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Table 1: Treatment of child as per risk group according to Histiocyte Society LCH Trial III

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Table 2: Improvement in hematological parameters at the end of 6 weeks of treatment

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LCH affects young children aged 1–4 years but can occur at any age. The incidence is approximately 2–5 per million per year in infants and children and is even rarer in adults.[2] It is categorized into three variants as LettererSiwe disease, Hand-Schuller-Christian disease, and eosinophilic granuloma.[3] The most characteristic dermatological presentation is seborrheic dermatitis-like and purpuric and/or purpuric rash with the involvement of the scalp, trunk, and flexural involvement. Skin lesions are usually the first manifestation of LCH in 80% of patients.[2] In our case, systemic symptoms preceded the typical skin manifestations.

The diagnosis of LCH is based on the presence of Langerhans cells, along with histiocytes and eosinophils, on histopathological examination. IHC withS-100, CD1a, CD4, and HLA-DR is helpful in confirmation.[2] Drugs such as oral prednisolone, vinblastine, 6-MP, methotrexate, etoposide, and cladribine have been used according to standard protocols in various studies with a favorable outcome.[4],[5] Our patient was treated with Group I Histiocyte LCH trial regimen consisting of prednisolone, vinblastine, and methotrexate along with leucovorin for 6 weeks which was followed by an additional 6 mercaptopurine for the next 10 months. The child had shown significant response in the clinical, hematological, and radiological parameters, without any side effects of chemotherapeutic agents. The current standard of care for patients with high-risk LCH is 1 year of therapy with vinblastine/prednisone/mercaptopurine, based on the LCH-III study. The addition of methotrexate to vinblastine/prednisone/mercaptopurine did not impact the response or relapse-free survival of high-risk patients. However, increasing treatment duration from 6 months in LCH-II to 12 months in LCH-III was beneficial.[6],[7] If patients have refractory or recurrent disease with vinblastine/prednisone, subsequent treatment with cytarabine or clofarabine may be effective.[8] A close follow-up for signs of treatment failure or reactivation IN these patients has been recommended. Generalized disease with skeletal involvement of sphenoid, mastoid, orbit bones, and recurrent disease are important risk factors for the development of residual sequelae. The mortality decreases significantly if there is no disease progression over 6–12 months. There are fewer chances of mortality. Very few cases of management of LCH according to guidelines have been reported from India.[9] Thus, we report a rare case of LettererSiwe disease in an 11-month female child treated as per Group I (multisystem risk patients), according to Histiocyte Society LCH Trial III.

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 images and other clinical information to be reported in the journal. The patient's parents understand that name and initials will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Iraji F, Poostiyan N, Rajabi Dehnavi P, Soghrati M. Langerhans cell histiocytosis: A case report with unusual cutaneous manifestations. Adv Biomed Res 2018;7:102.  Back to cited text no. 1
    
2.
Garg A, Kumar P. Multisystem Langerhans cell histiocytosis in adult. Indian J Dermatol 2012;57:58-60.  Back to cited text no. 2
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3.
Kumar YP, Agrawal J, Mohanlakshmi J, Kumar PS. Langerhans cell histiocytosis revisited: Case report with review. Contemp Clin Dent 2015;6:432-6.  Back to cited text no. 3
[PUBMED]  [Full text]  
4.
Narula G, Pradhan ND, Arora B, Banavali SD. Treatment of Langerhans cell histiocytosis with a modified risk-adapted protocol-experience from a tertiary cancer institute in India. Pediatr Blood Cancer 2018;65:e27028.  Back to cited text no. 4
    
5.
Minkov M. Multisystemic Langerhans cell histiocytosis in children: Current treatment and future directions. Paediatr Drugs 2011;13:75-86.  Back to cited text no. 5
    
6.
Gadner H, Grois N, Arico M, Broadbent V, Ceci A, Jakobson A, et al. A randomized trial of treatment for multisystem Langerhans' cell histiocytosis. J Pediatr 2001;138:728-34.  Back to cited text no. 6
    
7.
Gadner H, Grois N, Pötschger U, Minkov M, Aricò M, Braier J, et al. Improved outcome in multisystem Langerhans cell histiocytosis is associated with therapy intensification. Blood 2008;111:2556-62.  Back to cited text no. 7
    
8.
Simko SJ, Tran HD, Jones J, Bilgi M, Beaupin LK, Coulter D, et al. Clofarabine salvage therapy in refractory multifocal histiocytic disorders, including Langerhans cell histiocytosis, juvenile xanthogranuloma and Rosai-Dorfman disease. Pediatr Blood Cancer 2014;61:479-87.  Back to cited text no. 8
    
9.
Jha VK, Kumar R, Kunwar A, et al. Efficacy of Vinblastine and Prednisone in Multicentric Reticulohistiocytosis With Onset in Infancy. Pediatrics. 2016;137:e20152118.  Back to cited text no. 9
    


    Figures

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