Nail Involvement as Presenting Feature of Langerhans Cell Histiocytosis

Soheila Khazaei

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Soheila Khazaei.

Pediatric infectious diseases Department, MPH-Iran..

ADDRESS FOR CORRESPONDENCE
Soheila Khalilzadeh, Masih Daneshvari Hospital, Darabad Ave, Tehran, IR Iran.
Email: soheilak@yahoo.com

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Abstract

Langerhans Cell Histiocytosis is characterized by fever, hepatosplenomegaly, skin lesions, lymphadenopathy and bone marrow involvement. Nail involvement is unusual and rarely reported. Our patient with LCH had unusual findings such as nail lesions, clavicle involvement and occult lung involvement. We report such a case.

Introduction

Langerhans cell histiocytosis (LCH) summarizes a spectrum of diseases on the basis of histogenetic criteria. These are characterized by an accumulation of cells with Langerhans cell phenotype in one or multiple organs. The natural history varies from a benign disorder that resolves spontaneously to a progressive fatal disease (1). It has an extremely variable presentation; the disease may involve one or more body organ systems or tissues, such as bone, skin/mucous membranes, lung, lymph nodes, hypothalamus/posterior pituitary gland, liver and various soft tissues including the testes (2). The most common presenting features include fever, hepatosplenomegaly, skin lesions, lymphadenopathy and bone marrow involvement (3).

Case Report

A 27-month-old girl was admitted with the chief complaints of skin ulceration over the left clavicle and crusted ulcers on the scalp from 10 months ago. Her general health and development had been normal; her mother gave a previous history of trauma to her left clavicle. She also complained that the child's nails kept separating from nail beds and regrowing with normal shape from one year ago. She was treated with oral antibiotics without improvement. During the last months she had developed recurrent thrush. Physical examination at that time revealed crusted, impetigo-like lesions on her scalp. There were post auricular swellings with purplish discoloration. Multiple tiny papules measuring about 1-2mm were present around her nostrils. An ulcer with purulent discharge was present on her left clavicle. Paronychial erythema and swelling with subungual collections underneath of some fingernails and toenails were seen .She had no respiratory distress. There were no skin rashes, no lymphadenopathy or hepatosplenomegaly. Complete blood count showed a leukocyte count of 4600/ml, neutrophils: 37%, lymphocytes: 58%, eosinophils: 5%, a hemoglobin 8.5g/l and a platelet count of 266000/ml; ESR: 93mm/h and CRP: 3+. Liver function test, immunoglobulin level, neutrophilic chemotaxy, NBT (nitro blue tetrazolium test), sweat test and urinalysis were normal. The patient's ppd test was negative. Blood and urine culture were negative were normal. Swabs taken from nail lesions were negative for bacteria and fungi culture. The chest-x ray showed cystic changes and interstitial opacities in the lung fields and sclerotic changes in left clavicle with no evidence of bony lytic lesions. Considering history of trauma, presence of purulent discharge from the wound on her clavicle and sclerotic changes detected on her X-rays, she was treated with systemic antibiotics as a case of chronic osteomyelitis. Local antibiotic (mupirocin) was administered for scalp lesions. After six weeks of treatment, scalp lesions got noticeably better, the wound on her clavicle healed almost completely, the nails had regrown in normal shape and her ESR returned to normal.

Two months after her discharge, her nail and scalp lesions recurred and ESR rose up again, she was admitted for the second time for further evaluation. At that time chest-x-ray showed honeycomb pattern mostly in the upper lobes, sparing the costophrenic angles. The lung CT scan with contrast was done, which showed multiple cystic changes with micro nodular pattern in lungs field, involvement of upper lobe with relative sparing of bases. The child didn't show any respiratory symptoms. Skeletal survey showed an osteolytic lesion in the left femoral epiphyses. Abdominal imaging was normal. Biopsies of skin, lung and bone marrow were obtained which confirmed the diagnosis of Langerhans cell histiocytosis (LCH). Microscopic examination of lung tissue revealed a diffuse infiltration of histiocytes with abundant eosinophilic cytoplasm, some with reniform nuclei, which were positive for S100 and CD1a antigen. Unfortunately at this time, the parents demanded the child's discharge from the hospital. After 3 weeks she returned with severe respiratory distress. She was admitted in ICU and treated with oral steroid and combined chemotherapy but there was no response. She had consequent episodes of pneumothorax; each time managed with insertion of chest tubes. She died after 4 months of definitive diagnosis because of respiratory failure.

Discussion

LCH has an incidence of 1 per 1,000,000 in children younger than 15 years (4). Bone lesions may be single or numerous, asymptomatic or associated with pain and local swelling. Isolated bone lesions are reported in 36%, with the skull and proximal femur being the most common sites (2). The first sign in this patient was clavicle involvement with fistula formation, so she was treated as a case of chronic osteomyelitis with complete responded to antibiotic therapy. In literature review we didn't find this type of presentation. Subsequently we found an osteolytic lesion in the left femoral epiphyses when we have done bone survey.

Skin involvement occurs in about 50% of patients. They can be recurrent pyoderma-like lesions with crusting and scaling, vesicopustular and purpuric eruption occurs in crops over the face, scalp and trunk, resembling seborrheic dermatitis (2). Cutaneous lesions may be the sole presenting feature in LCH; the diagnosis is based on the presence of characteristic S-100 positive Langerhans cells (4). Our patient had these types of lesions but at that time it was assumed as impetigo.

In 10%-15% of patients, pulmonary infiltrates are found in radiography. The lesions may vary from diffuse fibrosis and disseminated nodular infiltrates to diffuse cystic changes. Rarely pneumothorax may be a complication (5). In our patient radiologic lung involvement was present without clinical evidence though terminally she had severe respiratory distress.

Nail changes in LCH are distinctly uncommon and it seems to be a prognostic factor. Paronychial erythema, swelling and subungual pustules of the fingernails and toenails are cardinal features (6,7,8). A review of 15 cases of histiocytosis X in Thailand showed that three of the seven patients with Letterer-Siwe disease had nail involvement and all three died rapidly, this paper conclude that nail involvement in histiocytosis X is an unfavorable prognostic sign (9) as was seen in our patient.

In survey of 70 cases, all patients who died with LCH (11% overall mortality) were under 2 years of age at diagnosis. Involvement of lung, liver, and bone marrow were confirmed as poor prognostic features. The presence of bone disease and absence of skin rash were identified as favorable features (8). Our patient had unusual findings such as nail lesions, clavicle involvement and occult lung involvement. These findings should alert the clinician to the possibility of Langerhans cell histiocytosis and avoid delayed diagnosis.

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Funding None

Conflict of Interest None

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