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Year : 2017  |  Volume : 18  |  Issue : 3  |  Page : 241-244

Skin rash with respiratory distress: A report of two cases

Department of Pediatrics, Institute of Child Health, Kolkata, West Bengal, India

Date of Web Publication7-Jun-2017

Correspondence Address:
Department of Pediatrics, Institute of Child Health, Kolkata, West Bengal
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/2319-7250.206053

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Skin is the largest organ of the body. Skin rash is not an uncommon manifestation, and sometimes, can give clue to the diagnosis of a systemic disease. Here we present two cases which presented with a typical skin rash and respiratory distress with extensive lung involvement, and ultimately diagnosed to be a case of Langerhans Cell histiocytosis.

Keywords: Histiocytosis, Langerhans, Langerhans cell histiocytosis, pneumothorax, skin rash

How to cite this article:
Das S, Deepshikha, Giri PP. Skin rash with respiratory distress: A report of two cases. Indian J Paediatr Dermatol 2017;18:241-4

How to cite this URL:
Das S, Deepshikha, Giri PP. Skin rash with respiratory distress: A report of two cases. Indian J Paediatr Dermatol [serial online] 2017 [cited 2021 Jan 23];18:241-4. Available from: https://www.ijpd.in/text.asp?2017/18/3/241/206053

  Introduction Top

The term Langerhans cell histiocytosis (LCH) is part of a group of clinical syndromes called histiocytoses that previously encompassed eosinophilic granuloma and two clinical syndromes: Letterer–Siwe disease and Hand–Schüller–Christian disease. All these syndromes represent similar processes, in which the proliferating cells have the structural and functional features of Langerhans cells. LCH can involve several organ systems, including lungs, bones, skin, liver, lymph node, pituitary gland, and thyroid gland. Lung involvement may occur either in isolation or as a part of multisystem disease.[1] We came across two such cases of pulmonary LCH that presented as acute respiratory distress syndrome in a short span of 6 months.

  Case Reports Top

Case Report 1

A 6-month-old female infant was admitted with respiratory distress for 7 days. There was a history of intermittent fever with cough and cold for the last 1½ months. There was no history of contact of tuberculosis. She was admitted outside 5 days back and treated with nebulization and intravenous meropenem, with no response. On admission, the child had alert look with heart rate of 140/min and respiratory rate of 75/min. SpO2 was 87% with 10 L/m O2, axillary temperature 99.2 F. On examination, there were bilateral crepitations with normally audible heart sounds and hepatomegaly measuring 4 cm along the midclavicular line. There were maculopapular rashes over the palm and soles [Figure 1] and hypopigmented macular rash [Figure 2] all over the trunk.
Figure 1: Rash on the palm

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Figure 2: Hypopigmented macules on the trunk

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Chest skiagram revealed heterogenous patchy infiltrates diffusely involving both the lung fields [Figure 3]. Routine blood investigations revealed total leukocyte count - 13,000/mm 3 with 60% polymorphs and 28% lymphocytes, hemoglobin (Hb) - 14.8 g/dl, platelet - 300,500/mm 3, erythrocyte sedimentation rate (ESR) - 16 mm in 1st h, and C-reactive protein (CRP) - 2.8 mg/dl (cutoff - 5 mg/dl).
Figure 3: Chest X-ray showing bilateral patchy infiltrates

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The girl deteriorated further, was intubated, and put on mechanical ventilator. Bronchoalveolar lavage and skin biopsy from affected lesions were sent for histopathological examination. The lavage showed the presence of scanty histiocytes [Figure 4], and histopathology was suggestive of LCH with immunohistochemistry positive for CD-1a and S-100. She was started on prednisolone and vinblastine as per the histiocytic society recent guidelines and responded to the chemotherapy. She was weaned off from mechanical ventilator at day 6.
Figure 4: Histopathological slide of bronchoalveolar lavage fluid showing scanty histiocytes

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Case Report 2

A 1-year, 6-month-old male child presented with respiratory distress and cough for 7 days. He had a history of cough and intermittent respiratory distress for the last 7 months. Previous chest skiagram was suggestive of bilateral miliary shadows, for which he had received anti-tubercular drugs for 6 months but without much improvement.

On admission, the child had icterus, heart rate 130/min, and respiratory rate 48/min. SpO2 was 90 with 8 L/m O2. Systemic examination revealed bilateral chest crepitations, audible heart sounds with no murmur, and hepatomegaly. Further examination revealed the presence of seborrheic dermatitis on the scalp with brownish discoloration of the nails [Figure 5] and purple maculopapular rash over the palms and soles [Figure 6]. Chest skiagram revealed heterogenous patchy infiltrates diffusely involving both the lung fields [Figure 7]. Routine pathological and biochemical investigations of the blood revealed total leukocyte count - 16,800/mm 3 with 70% polymorphs and 20% lymphocytes, Hb - 10.9 g/dl, platelet - 567,000/mm 3, ESR - 14 mm in 1st h, total and direct bilirubin - 2.65 mg/dl and 2.24 mg/dl, respectively, serum glutamic pyruvic transaminase and serum glutamic oxaloacetic transaminase - 381 IU and 281 IU, respectively, and negative CRP - 4.7 mg/dl (cutoff - 5 mg/dl).
Figure 5: Brownish discoloration of nails

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Figure 6: Rash on sole

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Figure 7: Chest X-ray showing bilateral patchy infiltrates

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The child was intubated and put on mechanical ventilation due to increasing distress. There was development of left-sided pneumothorax with subcutaneous emphysema, for which intercostal drain was placed [Figure 8]. With strong suspicion of LCH, bronchoalveolar lavage along with skin biopsy from affected lesions was sent, and injection vinblastine and oral prednisolone were started. There was remarkable improvement after the first dose of vinblastine and the child was extubated after a couple of days. Later, lavage showed the presence of histiocytes and histopathological with immunohistochemistry findings of skin biopsy was suggestive of LCH.
Figure 8: Chest X-ray showing left-sided pneumothorax with intercostal drain in situ

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  Discussion Top

LCH is a specific type of histiocytic syndrome characterized by infiltration of tissues with a specific dendritic cell, the Langerhans cell.[2] LCH may affect an isolated organ (formerly referred to as eosinophilic granuloma) or may be a multisystemic illness involving several sites (formerly referred to as Hand–Schüller–Christian or Letterer–Siwe disease).[3] Pulmonary involvement in LCH is more common in adults and may be the sole organ involved or a part of multisystem disease.[4] The lungs in children are usually involved as a part of multisystem LCH, which has a high rate of mortality.[5] In our index cases, the lung involvement was a part of multisystem involvement, but as initially, there was only pulmonary symptoms, and diagnosis was delayed.

Pulmonary LCH usually presents with dyspnea, nonproductive cough, pleuritic chest pain, or spontaneous pneumothorax.[6] The skin rash that is pathognomonic for LCH may follow or proceed pulmonary symptoms. Pulmonary LCH has variable appearance depending on the stage of disease, ranging from small peribronchiolar nodules to ground-glass opacities, DIP-like change, emphysema, or mosaic attenuation.[7]

Pulmonary involvement as a miliary shadow or mimicking bronchopneumonia is the usual way of presentation in pediatrics and that misleads the clinician to treat is as infective etiology as in our index cases treated with antibiotics and anti-tubercular drugs. Chatkin et al. reported a case of sole pulmonary involvement by LCH in a 4-year, 7-month-old child.[8] Pulmonary involvement in LCH can rarely get complicated by pneumothorax as in our second case. Khanbabaee et al. reported a similar case from Iran.[9]

Treatment with vinblastine and etoposide in combination with one dose of corticosteroids seems to be an equally effective treatment for multisystem LCH.[10] However, age <2 years and organ involvement with dysfunction are important risk factors that are associated with poor outcome.[11] Both of our patients responded dramatically with steroids and vinblastine. The principle management of pneumothorax is water seal drainage though some patient may need pleurodesis or thoracotomy.[12]

Early diagnosis of LCH and follow-up of diagnosed patients are necessary to prevent further complications. The presence of pulmonary infiltrates on chest skiagram with skin lesions could alert pediatrician to suspect LCH. Appropriate management and specific chemotherapy could be started in suspected cases even before the definite diagnosis is achieved.


We wish to thank the Director Dr. Apurba Ghosh for giving us permission to publish this article.

Financial Support and Sponsorship


Conflicts of Interest

There are no conflicts of interest.

  References Top

Howarth DM, Gilchrist GS, Mullan BP, Wiseman GA, Edmonson JH, Schomberg PJ. Langerhans cell histiocytosis: Diagnosis, natural history, management, and outcome. Cancer 1999;85:2278-90.  Back to cited text no. 1
Weitzman S, Egeler RM. Langerhans cell histiocytosis: Update for the pediatrician. Curr Opin Pediatr 2008;20:23-9.  Back to cited text no. 2
Komp DM. Historical perspectives of Langerhans cell histiocytosis. Hematol Oncol Clin North Am 1987;1:9-21.  Back to cited text no. 3
Vassallo R, Ryu JH, Schroeder DR, Decker PA, Limper AH. Clinical outcomes of pulmonary Langerhans'-cell histiocytosis in adults. N Engl J Med 2002;346:484-90.  Back to cited text no. 4
Schönfeld N, Frank W, Wenig S, Uhrmeister P, Allica E, Preussler H, et al. Clinical and radiologic features, lung function and therapeutic results in pulmonary histiocytosis X. Respiration 1993;60:38-44.  Back to cited text no. 5
Abbott GF, Rosado-de-Christenson ML, Franks TJ, Frazier AA, Galvin JR. From the archives of the AFIP: Pulmonary Langerhans cell histiocytosis. Radiographics 2004;24:821-41.  Back to cited text no. 6
Brauner MW, Grenier P, Tijani K, Battesti JP, Valeyre D. Pulmonary Langerhans cell histiocytosis: Evolution of lesions on CT scans. Radiology 1997;204:497-502.  Back to cited text no. 7
Chatkin JM, Bastos JC, Stein RT, Gaiger AM. Sole pulmonary involvement by Langerhans' cell histiocytosis in a child. Eur Respir J 1993;6:1226-8.  Back to cited text no. 8
Khanbabaee G, Hassas Yeganeh M, Tabatabaei SA, Khatami A, Bazrafshan S, Rezaei N. Langerhans cell histiocytosis with pulmonary involvement and unilateral pneumothorax. Turk J Pediatr 2010;52:638-41.  Back to cited text no. 9
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. 10
Nezelof C, Frileux-Herbet F, Cronier-Sachot J. Disseminated histiocytosis X: Analysis of prognostic factors based on a retrospective study of 50 cases. Cancer 1979;44:1824-38.  Back to cited text no. 11
Gunes T, Koklu E, Ozturk MA, Patiroglu T, Patiroglu T, Karakukcu M. A case of Langerhans cell histiocytosis presented with pneumothorax. J Pediatr Hematol Oncol 2007;29:60-2.  Back to cited text no. 12


  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7], [Figure 8]


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