| Case Presentation | No. 22-2018 | A 17-month-old girl presented with jaundice, hepatosplenomegaly, ascites and scaly erythematous skin lesion Sayma Rahman Munmun, A. S. M. Bazlul Karim, Rezina Parveen, Md. Benazamin, Md. Shafikul Alam Tanim and Manmohan Bir Shrestha results, the case was provisionally diagnosed. Article Info Presentation of Case Department of Pediatric Gastroenterol- Dr. Rezina Parveen: A 17-month-old girl, only ogy and Nutrition, Faculty of Medicine, issue of non-consanguineous parents, hailing Bangabandhu Sheikh Mujib Medical Provisional Diagnosis University, Shahbag, Dhaka, Bangladesh from Sirajgonj, Bangladesh immunized as per (SRM, ASMBK, RP, MB); Department of EPI schedule got admitted to the inpatient Chronic liver disease with portal hypertension Pathology, Faculty of Basic Science and department with the history of abdominal with pneumonia Paraclinical Science, Bangabandhu distension, jaundice, not growing well along Sheikh Mujib Medical University, Chronic liver disease Shahbag, Dhaka, Bangladesh (MSAT); with scaly skin lesion over scalp, chest, palm Department of Radiology and Imaging, and sole for 9 months. She was reasonably well The chronic liver disease is a disease process of Faculty of Medicine, Bangabandhu 9 months back. Then she developed abdominal the liver that involves a process of progressive Sheikh Mujib Medical University, distention which was increasing day-by-day Shahbag, Dhaka, Bangladesh (MBS) destruction and regeneration of liver paren- associated with gradually deepening jaundice. chyma leading to fibrosis and cirrhosis and in For Correspondence: There was also a history of irregular fever and some cases hepatocellular carcinoma. Unlike Sayma Rahman Munmun cough for 1 month. Fever was high-grade irre- [email protected] adults, there is no time duration of illness that gular in nature, subsided by taking antipyretics defines the chronic liver disease. All the inheri- Received: 21 September 2018 in conjunction with non-productive cough. ted metabolic and genetic conditions even Accepted: 13 October 2018 There was no history of contact with the when diagnosed at birth could have the Available Online: 25 December 2018 tuberculosis patient, any surgery or dental advance liver disease. The common causes of procedure, family history of liver disease, but chronic liver diseases in children are infection ISSN: 2224-7750 (Online) the history of one unit blood transfusion. 2074-2908 (Print) (chronic hepatitis B or C), metabolic liver On physical examinations, she was dyspnic, disease (Wilson disease, α1 antitrypsin deficien- DOI: 10.3329/bsmmuj.v11i4.38382 febrile, mildly pale and icteric (Figure 1). The cy, glycogen storage disease, lipid abnormali- ties, cystic fibrosis), developmental/genetic respiratory rate was 48 breaths/min, SPO2 95% (biliary atresia, congenital hepatic fibrosis, Keywords: Ascites; Hepatosplenomeg- without O2. The blood pressure was within aly; Jaundice; Langerhans’cell histiocyto- normal limit. The digital clubbing, multiple red Alagille syndrome), immune-related (auto- sis; Liver; Scaly erythematous; Spleen papular non-itchy skin lesion on both palm and immune hepatitis, sclerasing cholangitis), vas- sole and diffuse scaly lesion on the scalp, and cular (Budd chiari syndrome, hepatic veno- Cite this article: BCG mark were present. There was no occlusive disease, portal vein thrombosis), etc. Munmun SR, Karim ASMB, Parveen R, Benazamin M, Tanim MSA, Shrestha lymphadinopathy. She was severely wasted, Patients with chronic liver disease clinically MB. A 17-month-old girl presented with stunted and underweight. On systemic may present with anemia, jaundice, bleeding jaundice, hepatosplenomegaly, ascites examination, the abdominal distention with manifestations and other stigmata of chronic and scaly erythematous skin lesion. huge hepatosplenomegaly and ascites were liver disease (palmar erythema, nail clubbing, Bangabandhu Sheikh Mujib Med Univ J. found. The wheeze and crackles were present 2018; 11: 304-309. spider nevi), ascites, features of hypersplenism on both lung fields. with splenomegaly.1 In this case, anemia, jaun- Copyright: dice, clubbing, hepatoslenomegaly and ascites The laboratory data (Table I) showed anemia The copyright of this article is retained are in favor of chronic liver disease but the by the author(s) [Atribution CC-By 4.0] (hemoglobin: 8.6 g/dL), markedly raised characteristic skin lesion and lung findings are erythrocyte sedimentation rate (120 mm in 1st Available at: against the diagnosis of chronic liver disease. hour), grossly impaired liver function (serum www.banglajol.info alanine aminotransferase: 238 U/L, total bilirubin: 9.2 mg/dL, serum albumin 27 g/L). The X-ray chest showed consolidation (Figure Differential Diagnosis 2). The Mantoux test was 2 mm. Dr. Md. Benzamin: The patient presented with The abdominal ultrasonography showed heter- jaundice, gradual abdominal distension and ogenous echotexture of the liver with multiple failure to thrive. There was a history of cough nodular lesion. After evaluating the clinical and irregular fever. The child was dyspneic, data, physical findings and investigation febrile, mildly pale and icteric. Digital clubbing BSMMU J 2018; 11: 304-309 305 lymphadenopathy and the Mantoux test was negative. These go against the diagnosis of tuber- culosis. Tuberculosis Tuberculosis is an important global health problem and Bangladesh is one of the high tuberculosis burden countries in the world. It includes the pulmonary and extrapulmonary form. Studies from Bangladesh showed that extrapulmonary form is commoner in children.2, 3 Disseminated tuberculosis is defined as tuberculous infection involving the bloodstream, bone marrow, liver, or involvement of two or more non-contiguous sites, or miliary tuberculosis.4 It is a potentially life-threatening form 4, 5 A B of tuberculosis. The incidence of disseminated tuberculosis ranges from 1-3% of all tuberculosis cases.6, 7 It is common among the infants and children.8 Dr. Sayma Rahman Munmun: After evaluating the patient’s presenting features, physical findings and the laboratory test results she was considered for further investigation because the skin lesions remained unexplained. For this, the skin biopsy was done and the tissue was sent for histopathology. C D Dr. Md. Shafikul Alam Tanim: The histopathological examination of the biopsy specimen taken from the Figure 1: The patient with jaundice (A), abdominal distension (B), erythematous scalp following anesthesia revealed mixed infiltrate rash over scalp and face (C), and hand (D) of Langerhans cells, lymphocytes and occasional eosinophils in the upper dermis. However, a was present. She was severely wasted and under- variable number of neutrophils and plasma cells weight. On systemic examination, wheeze and were also found in many other cases. Immuno- crackles were present in both lung fields. Huge histochemical analysis was performed with anti- hepatosplenomegaly with ascites was also present. CD1a, S100 and CD68 primary antibodies. Langer- Investigations showed high erythrocyte sedimenta- hans cells organized in groups and singly showed tion rate and consolidation on the chest X-ray. After brown immunohistochemical stain for CD1a and evaluating the clinical data, physical findings and S100 protein, but not for CD68 protein. In immuno- investigations report we differentially considered it histochemical examination, CD1a and S100 posi- as a case of disseminated tuberculosis. But there tivity confirmed the diagnosis of Langerhans cell was no history of contact with the tuberculosis histiocytosis (Figure 3). patient, BCG mark was present. There was no Dr. Munmun: After getting the histopathology report, we did the skeletal survey to see the bone involvement. We also did the bone marrow puncture which was normal and planned for the liver biopsy but could not do that as because the parents did not give consent. Dr. Manmohan Bir Shrestha: Patient’s X-ray of the right leg revealed well-defined round intramedu- llary non-expansile lucent lesion with marginal sclerosis in the metadiaphyseal region of the proximal shaft of tibia. Radiological differentials for this type of lesion includes benign bone neoplasms (i.e. simple bone cyst, enchondroma, chondro- blastoma), Brodie’s abscess, Langerhans cell histio- cytosis, brown tumor of hyperparathyroidism and A B metastasis. In Langerhans cell histiocytosis, the Figure 2: Consolidation of lungs (A) and lytic lesion over tibia (B) as shown by common locations include the skull and long bones. arrows In the case of long bones, Langerhans cell 306 BSMMU J 2018; 11: 304-309 histiocytosis. After considering the history, physical Table I examination, biopsy and immunohistochemistry findings we reached a diagnosis of Langerhans cell Laboratory investigations of the patient histiocytosis. Parameter Findings References Langerhans cell histiocytosis Hemoglobin (g/dL) 8.6 12.2-14.8 This is a rare disease of Langerhans cell where 9 WBC count (×10 /L) 10 4-10 multiple systems like skin, bones, lymph nodes, Differentials pituitary gland, bone marrow, liver, spleen, lungs Neutrophil (%) 65 40-80 etc. can be affected. Langerhans cell histiocytosis was previously called histiocytosis X, which inclu- Lymphocyte (%) 28 20-40 ded three entities named eosinophilic granuloma, Eosinophil (%) 3 1-6 Hand-Schuller-Christian disease, Letterer-Siwe Monocyte (%) 8 2-10 disease.9-13 These are, now, replaced by single- Platelet count (109/L) 150 150-400 system single site, single-system