E-ISSN 1858-8360 | ISSN 0256-4408
 

Case Report 


SUDANESE JOURNAL OF PAEDIATRICS

2021; Vol 21, Issue No. 2

CASE REPORT

Twenty nail dystrophy in a child with Langerhans cell histiocytosis and tuberculosis

Vinson James (1), Anand Prakash (1), Chikkanayakanahalli Indumathi (1)

(1) Pediatrics Department, St. John’s Medical College Hospital, Bangalore, India

Correspondence to:

Dr. Vinson James

Pediatrics Department, St. John’s Medial College Hospital, Bangalore, India

Email: dr.vinsonjames [at] gmail.com

Received: 08 October 2020 | Accepted: 08 April 2021

How to cite this article:

James V, Prakash A, Indumathi CK. Twenty nail dystrophy in a child with Langerhans cell histiocytosis and tuberculosis. Sudan J Paediatr. 2021;21(2):200–204.

https://doi.org/10.24911/SJP.106-1602105103

ABSTRACT

Langerhans cell histiocytosis (LCH) is a rare disorder of proliferation of myeloid derived dendritic cells. Nail involvement in LCH is extremely uncommon. We report a child who presented with dystrophic changes in all 20 nails along with skin, dental, respiratory and abdominal findings who was diagnosed as pulmonary tuberculosis (PTB) and multisystem LCH. This is the fourth case being reported having nail changes with multisystem involvement, including high risk organs (liver and spleen), and the first case worldwide to be reported as microbiologically confirmed PTB with multisystem LCH and dystrophy of 20 nails. In the setting of a multisystem presentation in an endemic country for tuberculosis, where diagnosis is challenging, the characteristic nail changes of LCH help in the diagnosis.


KEYWORDS

Langerhans cell histiocytosis; Nail changes; Pulmonary tuberculosis; Multisystem Langerhans cell histiocytosis.


INTRODUCTION

Langerhans cell histiocytosis (LCH) is a rare disorder characterized by abnormal accumulation of cells belonging to the mononuclear phagocytic system. It may involve only a single system LCH wherein only one system is involved, or may involve multiple systems LCH, wherein two or more systems are involved [1]. LCH is a clinico-pathological diagnosis where, in the appropriate clinical setting, histological finding of Langerhans cells and positivity for CD1a and/or CD207 (Langerin) is required. Nail changes in LCH are extremely uncommon [2]. Rarely, LCH has been associated with tuberculosis (TB) with almost all cases being reported in adults [3-6].

We present here a young boy who came with chronic respiratory concerns, where the characteristic nail changes helped in confirmation of diagnosis.


CASE REPORT

A 2 ½-year-old boy from rural southern India presented with complaints of recurrent episodes of fever, cough and coryza since 9 months. He had been started on anti-tuberculosis treatment (ATT) empirically due to unresolving symptoms, cavitatory chest radiograph which was continued in view of broncho-alveolar lavage (BAL) showing acid fast bacilli (AFB). The child was referred to our center as a case of drug resistant TB. Besides the fever and chronic cough, parents complained of significant weight loss, progressive nail dystrophy and a recent onset scalp swelling which started after a month of ATT initiation. At the end of history, the possibilities considered were resistant TB/ with superadded infection, immunodeficiency, fungal infection and paronychia due to fungal/ immuno-deficient states. Physical examination revealed pallor, non-tender cystic swelling of the scalp associated with scaling, hyperkeratotic plaques on palms and soles, loose teeth (molars) and onycho-dystrophy of all twenty nails (Figures 1-3).

Figure 1. LCH with multiple nail involvement. The arrows show nail destruction.

Head circumference and weight were between -2 and -3 on the z scores for age, and height was within 0-2 z scores for age. The child had respiratory distress (chest retractions, oxygen requirement as room air saturation was 88%, and tachypnea with respiratory rate of 40/minutes) and was hemodynamically stable. On systemic examination of respiratory system, increased work of breathing with suprasternal and subcostal retractions were noted along with reduced air entry in the right supra-clavicular, mammary and axillary areas and bronchial breath sounds and crepitations on auscultation. Hepatosplenomegaly was noted on abdominal examination with liver span being 16 cm extending 10 cm below right costal margin, whereas the spleen extended 9 cm below left costal margin. At the end of examination, the possibilities considered remained the same.

Laboratory findings showed haemoglobin of 11.5 g/l, total white blood cell count of 23.74 × 109/l, differential count of 12.58 × 109/l neutrophils, 10.2 × 109/l lymphocyte, 0.2 × 109/l eosinophil, 0.5 × 109/l basophil, 0.2 × 109/l monocyte and platelet count of 840 × 109/l. Peripheral smear showed normocytic hypochromic blood picture. Liver function tests showed hypoalbuminemia (24 g/l), conjugated hyperbilirubinemia (24 μmol/l with total bilirubin being 27.4 μmol/l) and elevated liver enzymes (aspartate aminotransferase – 59.8 units/l, alanine aminotransferase – 53.89 units/l, alkaline phosphatase – 994 units/l and gamma glutamyltransferase – 469 units/l). Human immunodeficiency virus 1 and 2 antibodies were negative. Immuno deficiency and cystic fibrosis were ruled out by performing flow cytometry, immunoglobulin assay and sweat chloride studies. Nitroblue tetrazolium reduction test was negative. Sputum/ gastric aspirate/ bronchoalveolar lavage (BAL)/nail scrapings were negative for bacterial, mycobacterial and fungal infections, respectively. Potassium hydroxide staining of oral mucosa showed normal study. Repeat AFB and Mantoux test done at our centre on admission (after 2 months of ATT initiation) were negative. Chest radiograph was suggestive of consolidation (Figure 4) and skull radiograph showed a single lytic lesion (Figure 5).

Figure 2. Nail plate destruction. The arrows show complete nail plate destruction.

Figure 3. LCH with multiple toe nail involvement. The arrows show dystrophic foot nails.

Ultrasound examination of abdomen demonstrated hepatosplenomegaly with no lymphadenopathy or ascites. Chest computed tomography (CT) scan revealed right upper lobe consolidation, cavitation with extensive bilateral nodular opacities and central necrosis, mild bronchiectasis and peribronchial interstitial thickening (Figure 6).

Figure 4. Chest X-ray. The arrow shows consolidation.

Figure 5. Skull X-ray. The arrow shows lytic skull bone lesion.

In view of persistent respiratory distress, hilar lymphadenopathy, persistent right upper lobe pneumonia, AFB positive BAL and good adherence and adequate dosage of ATT, disseminated/ resistant TB was considered. In view of scaly scalp lesions, dystrophic nail changes, loosening of teeth with gum hypertrophy, persistent lung symptoms, hepatosplenomegaly and poor response to ATT, LCH was considered. Biopsy of scalp, nail and skin (of palms and soles) were performed and histopathology revealed Birbeck granules with immuno-histochemical staining showing positivity for CD1a and S100 (Figure 7). At this point, the child was diagnosed to have co-existing pulmonary tuberculosis (PTB) and multi-systemic LCH with nail changes.

Figure 6. Chest CT scan. The arrow shows a CT slice suggestive of LCH.

Figure 7. Skin histology slide suggestive of histiocytosis. The arrow shows highly cellular large, polygonal cells with ample cytoplasm. Nuclei are round, oval or bean shaped with fine and even chromatin and prominent longitudinal grooves.

Child was started on LCH III protocol (vinblastine 6 mg/m2 and prednisone 40 mg/m2) and ATT was continued. There was resolution of skin, nail and scalp lesions with persistence of consolidation on radiographs. After one and a half year of remission, he presented with cholestatic jaundice with liver biopsy confirming active LCH. He was treated with salvage LCH protocol (cytarabine and cladribine) but succumbed to septic shock after the second cycle of chemotherapy.


DISCUSSION

Nail findings are very rare in LCH with only 27 cases being reported worldwide till now [6]. The first cases of LCH with nail findings were described by Bender and Holtzman [7], and Kahn [8] in adults and pediatrics, respectively. First cases of LCH with nail findings in India were described by Jain et al. [9] and Chander et al. [2] in adults and pediatrics, respectively. We describe here the first case report in India with co-existent TB and multisystem LCH and nail involvement which showed a good response to standard chemotherapy with resolution of nail findings.

In most of the reported cases, nail findings were associated with only skin and lung findings. Historically, nail findings were thought to be associated with poorer prognosis; however, recent studies indicate otherwise [6]. Nail changes in LCH mostly occur in patients with multisystem disease and involvement of high-risk organs (liver, spleen, lungs and hematopoietic system), predisposes to higher recurrence risk and mortality.

Co- existing PTB and multi-system LCH is a very rare finding with only six case reports from world-over [3,4]. Clinical features like recurrent episodes of fever, cough with lymphadenopathy, hepatosplenomegaly, cavitatory chest lesions on chest radiographs in an undernourished child can occur in both TB and LCH. Nail findings, pulmonary radiology, lytic bone lesion and non-resolution of symptoms with ATT should prompt to look for LCH [10].

This clinical presentation was challenging for our team and we hope this case report helps other clinicians faced with similar clinical scenarios. Co-existing PTB and LCH must be considered in a patient with PTB in an endemic region, who is not responding to treatment. This case report also shows that dystrophy of all twenty nails is a very rare feature associated with LCH and is usually associated with an advanced disease.


ACKNOWLEDGEMENT

Thanks are due to members of the Department of Pediatrics at St. John’s Medical College, Bangalore, India for their contribution.


CONFLICT OF INTEREST

The authors declare that they have no conflicts of interest.


FUNDING

None.


ETHICAL APPROVAL

Informed consent for participation and publication of medical details was obtained from the parents of this child. Confidentiality of patient’s data was ensured at all stages. The authors declare that ethics committee approval was not required for this case report.


REFERENCES

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  2. Chander R, Jaykar K, Varghese B, Garg T, Seth A, Nagia A. Pulmonary disease with striking nail involvement in a child. Pediatr Dermatol. 2008;25(6):633–4. https://doi.org/10.1111/j.1525-1470.2008.00788.x
  3. Arai T, Inoue Y, Yamamoto S, Akira M, Uesugi H, Hayashi S, et al. Incipient stage of pulmonary Langerhans-cell histiocytosis complicated with pulmonary tuberculosis was examined by high-resolution computed tomography. Respir Med. 2005;99(9):1188–90. https://doi.org/10.1016/j.rmed.2005.02.017
  4. Okutan O, Kartaloglu Z, Ilvan A, Deniz O, Silit E, Gorur R. Active pulmonary tuberculosis in a patient with Langerhans cell histiocytosis. Saudi Med J. 2006;27(3):401–2.
  5. Kumar S, Batra C, Kataria SP, Arora B, Singh G, Nayanrattan K, et al. Langerhans’ cell histiocytosis with tuberculosis: a rare combination diagnosed on FNAC. Ann Diagn Paediatr Pathol. 2007;11:21–3.
  6. Timpatanapong P, Hathirat P, Isarangkura P. Nail involvement in histiocytosis X. A 12-year retrospective study. Arch Dermatol. 1984;120(8):1052–6. https://doi.org/10.1001/archderm.1984.01650440082024
  7. Bender B, Holtzman IN. Histiocytosis X (granulomatous reticuloendotheliosis): report of a case of chronic disseminated reticuloendotheliosis whose evolution may have some bearing on the classification. AMA Arch Derm. 1958;78(6):692–702. https://doi.org/10.1001/archderm.1958.01560120012003
  8. Kahn G. Nail involvement in histiocytosis-X. Arch Dermatol. 1969;100(6):699–701. https://doi.org/10.1001/archderm.1969.01610300049007
  9. Jain S, Sehgal VN, Bajaj P. Nail changes in Langerhans cell histiocytosis. J Eur Acad Dermatol Venereol. 2000;14(3):212–5. https://doi.org/10.1046/j.1468-3083.2000.00066.x
  10. Prakash A, Durugappa T, Ramachandran P, Lalitha AV. Pleurodesis in pulmonary langerhans cell histiocytosis in children: a life-saving measure. Indian J Med Paediatr Oncol. 2019;40(5 Suppl S1):160–2. https://doi.org/10.4103/ijmpo.ijmpo_250_17


How to Cite this Article
Pubmed Style

James V, Prakash A, Indumathi C. Twenty nail dystrophy in a child with Langerhans cell histiocytosis and tuberculosis. Sudan J Paed. 2021; 21(2): 200-204. doi:10.24911/SJP.106-1602105103


Web Style

James V, Prakash A, Indumathi C. Twenty nail dystrophy in a child with Langerhans cell histiocytosis and tuberculosis. https://www.sudanjp.com/?mno=9028 [Access: December 04, 2022]. doi:10.24911/SJP.106-1602105103


AMA (American Medical Association) Style

James V, Prakash A, Indumathi C. Twenty nail dystrophy in a child with Langerhans cell histiocytosis and tuberculosis. Sudan J Paed. 2021; 21(2): 200-204. doi:10.24911/SJP.106-1602105103



Vancouver/ICMJE Style

James V, Prakash A, Indumathi C. Twenty nail dystrophy in a child with Langerhans cell histiocytosis and tuberculosis. Sudan J Paed. (2021), [cited December 04, 2022]; 21(2): 200-204. doi:10.24911/SJP.106-1602105103



Harvard Style

James, V., Prakash, . A. & Indumathi, . C. (2021) Twenty nail dystrophy in a child with Langerhans cell histiocytosis and tuberculosis. Sudan J Paed, 21 (2), 200-204. doi:10.24911/SJP.106-1602105103



Turabian Style

James, Vinson, Anand Prakash, and Chikkanayakanahalli Indumathi. 2021. Twenty nail dystrophy in a child with Langerhans cell histiocytosis and tuberculosis. Sudanese Journal of Paediatrics, 21 (2), 200-204. doi:10.24911/SJP.106-1602105103



Chicago Style

James, Vinson, Anand Prakash, and Chikkanayakanahalli Indumathi. "Twenty nail dystrophy in a child with Langerhans cell histiocytosis and tuberculosis." Sudanese Journal of Paediatrics 21 (2021), 200-204. doi:10.24911/SJP.106-1602105103



MLA (The Modern Language Association) Style

James, Vinson, Anand Prakash, and Chikkanayakanahalli Indumathi. "Twenty nail dystrophy in a child with Langerhans cell histiocytosis and tuberculosis." Sudanese Journal of Paediatrics 21.2 (2021), 200-204. Print. doi:10.24911/SJP.106-1602105103



APA (American Psychological Association) Style

James, V., Prakash, . A. & Indumathi, . C. (2021) Twenty nail dystrophy in a child with Langerhans cell histiocytosis and tuberculosis. Sudanese Journal of Paediatrics, 21 (2), 200-204. doi:10.24911/SJP.106-1602105103





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