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On-line version ISSN 1806-4841
An. Bras. Dermatol. vol.81 no.6 Rio de Janeiro Nov./Dec. 2006
Langerhans cell histiocytosis with nail involvement in childhood a case report*
Wellington Luiz MendesI; Claudia Zavaloni MelottiII; Alanna Mara BezerraIII; Fernando Augusto SoaresIV; Beatriz de CamargoV
IPhysician, Department of Oncological
Pediatrics, Cancer Hospital Treatment and Research Center São Paulo, Brazil
IIPhysician, Department of Dermatology / Cutaneous Oncology, Cancer Hospital Treatment and Research Center São Paulo, Brazil
III3rd. Year Resident Physician, Department of Pathology, Cancer Hospital Treatment and Research Center São Paulo, Brazil
IVChair, Department of Pathology, Cancer Hospital Treatment and Research Center São Paulo, Brazil
VChair, Department of Oncological Pediatrics, Cancer Hospital Treatment and Research Center São Paulo, Brazil
Langerhans cell histiocytosis may appear in a variety of ways. The authors
present the case report of a 3-year-old white boy with a main complaint about
chronic media otitis and a tumor lesion in right mastoid bone. Pathology revealed
histiocytosis. The patient had severe dermatitis on the scalp and dystrophic
changes with onycholysis, pustules, and nail plate deformity underneath all
fingernails and toenails. These lesions responded to antineoplastic therapy.
Development of nail dystrophics in Langerhans cell histiocytosis is unusual
in children. This case suggests that treatment with antineoplastic therapy might
Langerhans cell histiocytosis may appear in a variety of ways. The authors present the case report of a 3-year-old white boy with a main complaint about chronic media otitis and a tumor lesion in right mastoid bone. Pathology revealed histiocytosis. The patient had severe dermatitis on the scalp and dystrophic changes with onycholysis, pustules, and nail plate deformity underneath all fingernails and toenails. These lesions responded to antineoplastic therapy. Development of nail dystrophics in Langerhans cell histiocytosis is unusual in children. This case suggests that treatment with antineoplastic therapy might be effective.
Keywords: Child; Langerhans cell histiocytosis; Langerhans cell histiocytosis/diagnosis; Langerhans cell histiocytosis/therapy; Nails; Nails/lesions
The disease formally known as histiocythosis X is currently referred to as Langerhans Cell Histiocytosis (LCH).1 LCH may present itself in a variety of clinical forms ranging from the spontaneous regression of the single bone lesion to a severe multisystemic disorder. In every case, the single histiological finding is the proliferation of Langerhans cells.2
LCH diagnosis is made in a presumptive manner when the typical morphological features of Langerhans cells are observed through optical microscopy, as well as when immunohistochemical assays for S100 protein are positive. The confirmation of the diagnosis is obtained if the reaction with CD1a is positive or when cytoplasm organelles (Birbeck granules) are seen in electronic microscopy.1
Although LCH is a specific disease, its clinical findings vary from single bone lesions to multiple skeletal lesions, which are frequently associated with a variety of manifestations in visceral sites, such as the skin, lymphnodes, bone marrow, liver, spleen and central nervous system.
Nail alterations in LCH are unusual and they are characterized by longitudinal grooves, purpureal striations, hyperkeratosis, dense deformities under nails, nail plate loss, and paronychia. Some reports suggest that nail involvement indicates an unfavorable prognosis for the disease.3
The purpose of this case report is to emphasize the nail manifestations in LCH in a three-year-old boy diagnosed with LCH. The therapy addressed to histiocytosis treatment resulted in an improvement of nail lesions, as well as other cutanous changes and involvement of systemic sites.
Three-year-old boy whose main complaint was chronic otitis media. He was submitted to mastoidectomy in another service due to a tumoral lesion in the region. The anatomopathological examination revealed histiocytosis. The patient was then referred to the Department of Pediatrics of the Cancer Hospital Treatment and Research Center.
A general physical examination was performed upon admission. Otocoscpy revealed purulent drainage of the right external auditory channel. The exam of the oral cavity showed ulcerated lesion of the mucosa close to the right upper molar, and the cutaneous tissue revealed scales and crusts on the scalp, suggesting the appearance of seborrheic dermatitis. Nail alterations were also seen, including paronychia, onycholysis, nail hyperkeratosis, pustules, and purpureal striations visible under all fiver and toenails (Figure 1).
It is important to stress that all such changes were limited to the nail bed, not reaching the matrix. Lymphnodes and liver appeared to be normal, as well as the rest of the physical examination.
Blood test, hemosedimentation speed, liver and kidney functions, urine I and bone marrow aspirate were normal. Cultures for bacteria and fungi from the nail lesions were negative, as well as the potassium hydroxide (KOH) preparation. The CT scan of head and neck revealed an erosive lesion in the left mastoid bone. Chest and long bone X-rays were normal.
The mastoid bone lesions were reexamined, but the finger- and toenail lesions were not submitted to biopsy. Histopathological findings were characterized by a dense histiocytic infiltrate, made up mainly of Langerhans cells with a homogeneous rose cytoplasm and lobed nucleus. Some cells showed nuclear longitudinal grroves. No mitosis was identified and phagocytosis was absent. Numerous eosinophils were also present. This inflammatory infiltrate was not accompanied by necrosis. The mastoid bone involvement had an infiltrative pattern in trabecular bone.
Immunohistochemistry revealed a strong positivity for proteins S100, CD 68, and CD 1a in the histiocytic Langerhans cells (Figure 2).
Before the beginning of treatment, the patient presented symptoms of diabetes insipidus, with polyuria and polydipsia, which were easily controled by means of desmopressine (1-desamino-8-D arginine vasopressine [DDAVP]) after the water deprivation text confirmed the diagnosis.
The LCH diagnosis was confirmed and treatment began according to Chart 1. The first assessment was carried out after the induction phase, with a significant improvement of dystrophic alterations, desappearance of the dermatitis and improvement of the purulent otitis in the right ear, with an adequate visualization of the eardrum membrane. The maintenance phase continued for six months, with complete disease remission. The dystrophic lesions and pustules under the nails disappeared totally after six months therapy (Figure 3), as well as the dermatitis lesions. Two years after the end of systemic therapy, the patient was disease-free.
Histiocytosis includes a spectrum of diseases with many similarities. The development of nail dystrophy is unusual among children, even though its occurrence in adults has been reported by several authors.4-6 Bender and Holtzman were the first to publicize a case of nail involvement in LCH.4 After this paper, Kahn reported another case of LCH which presented nail dystrophy and good response to therapy with cytotoxic agents7. Other authors reported treatment success with antimetabolite agents in patients with nail involvement without the description of the pathology,8 even though there are some reports which do not show the same experience.9 All such reports associated the nail involvement with Letterer-Siwe Disease, a term no longer used for a disease that carried a poor prognosis, probably due to factors such as young age at the onset of the disease, malignant histological pattern and multisystemic involvement.10 Timpatanapong and cols. recorded nail involvement in three patients with Letterer-Siwe-type LCH and lesions similar to those of seborrheic dermatitis, suggesting an unfavorable prognosis even with the use of aggressive therapy. Other published experiences, however, did not agree with the author. 3-5,7,11
Holzbert and cols. reported the first LCH case in which a longitudinal biopsy of the nail showed that the dystrophy was due to the same atypical histiocytes that caused the cutaneous lesions.12
There is little evidence that the nail alternations constitute a prognostic factor, compared to the major organic dysfunctions which are considered useful outcome markers,10 but several authors reported a poor prognosis for patients who had the nail matrix compromised. 3, 9
In previous reports, the clinical findings seen in the nails included longitudinal grooves, purpureal striations, hyperkeratosis, pustules under the nails, dense deformities or cracks in the nail plates. The patient in question had fingernail involvement that illustrated all such changes. In accordance with other reports, the authors have been able to detect a correlation between the efficacy of chemotherapy and the disappearance of the nail lesions. The complete recovery of the nail after therapy probably occurred because the nail matrix was not affected.
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3. Timpatanapong P, Hathirat P, Isarangkura P. Nail involve ment in histiocytosis X. A 12-year retrospective study. Arch Dermatol. 1984;120:1052-6. [ Links ]
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8. Diestelmeier MR, Soden CE, Rodman OG. Histiocytosis X: a case with nail involvement. Cutis. 1982;30:483-6. [ Links ]
9. Harper JI, Staughton R. Letterer-Siwe disease with nail involvement. Cutis. 1983;31:493-8. [ Links ]
10. Lahey ME. Histiocytosis-X: na analysis of prognostic factors. J Pediatr. 1975;87:184-9. [ Links ]
11. Ellis JP. Histiocytosis X--unusual presentation with nail involvement. J R Soc Med. 1985;78( Suppl 11):S3-5. [ Links ]
12. Holzberg M, Wade TR, Buchanan ID, Spraker MK. Nail pathology in histiocytosis X. J Am Acad Dermatol. 1985;13:522-4. [ Links ]
Wellington Luiz Mendes
Alameda Barão de Limeira, 1503.
Campos Elíseos São Paulo SP
Tel: 55 (11) 3828-1059
Fax: 55 (11) 3828-1059
Conflict of interests: None
Received on July. 27 of 2004
Approved by the Consultive Council and accepted for publication on Oct. 30 of 2006
* Work carried out at the Cancer Hospital Treatment and Research Center São Paulo. Brasil.