|Year : 2018 | Volume
| Issue : 1 | Page : 74-76
A rare co-existence of langerhans cell histiocytosis and lichen planus
Aarti S Salunke, Anil P Gosavi, Ravindranath B Chavan, Pallavi Prataprao Patil
Department of Skin and VD, BJGMC, Pune, Maharashtra, India
|Date of Web Publication||28-Dec-2017|
Pallavi Prataprao Patil
Department of Skin and VD, BJGMC, Pune, Maharashtra
Source of Support: None, Conflict of Interest: None
Langerhans cell histiocytosis (LCH) is the commonest of the histiocytic disorders. Owing to the relative rarity of the condition, it remains a disease in which the diagnosis is often delayed or missed. Commonly seen in infancy or early childhood, the disorder is characterized by proliferation of abnormal and clonal Langerhans cells in skin, bone, lymph nodes, lungs, liver, spleen, and bone marrow. Lichen planus presents as purple, polygonal, pruritic, papular eruption of unknown etiology affecting skin, mucous membranes and nails. Despite of thourough literature search, we could not find any association of lichen planus with LCH. Hereby, we report a case of five year old child with acute onset of polymorphic eruptions all over the body which on histopathology and Immunohistochemistry showed features of Langerhans cell histiocytosis and lichen planus on bilateral lower limb.
Keywords: Immunohistochemistry, Langerhans cell histiocytosis, lichen planus
|How to cite this article:|
Salunke AS, Gosavi AP, Chavan RB, Patil PP. A rare co-existence of langerhans cell histiocytosis and lichen planus. Indian J Paediatr Dermatol 2018;19:74-6
|How to cite this URL:|
Salunke AS, Gosavi AP, Chavan RB, Patil PP. A rare co-existence of langerhans cell histiocytosis and lichen planus. Indian J Paediatr Dermatol [serial online] 2018 [cited 2019 May 23];19:74-6. Available from: http://www.ijpd.in/text.asp?2018/19/1/74/206075
| Introduction|| |
Langerhans cell histiocytosis (LCH) is a reactive disorder, in which cells having the phenotypic markers of epidermal Langerhans cells are found in skin and other organs where they cause damage by excessive production of cytokines and prostaglandins. Although this condition has been described using numerous different terms (histiocytosis X, Letterer-Siwe diseases, Hand–Schuller–Christian disease, eosinophilic granuloma), the Writing Group of the Histiocyte Society 3 divided histiocytosis into the following three classes: Class I: LCH, Class II: Non-LCH, and Class III: Malignant histiocytic diseases. Lichen planus in children is considered to be rare overall though it does not appear to be so in the Indian subcontinent.
| Case Report|| |
Five-year-old male child, second child born out of nonconsanguineous marriage was brought by parents with complaints of asymptomatic skin lesion over the body and scalp sparing face for 2 years. The patient developed whitish scaly lesions over the scalp and occasionally itchy lesions over lower limb which gradually spread to involve bilateral upper and lower limbs, trunk, sparing face, palms, and soles. He did not have a history of increased thirst, micturition, bone pain, breathlessness, lethargy, or diplopia.
On general examination, he had tender significant lymphadenopathy over the right submandibular, right cervical, and bilateral inguinal site. On systemic examination, hepatomegaly was present; other systemic examination was normal.
On dermatological examination, scalp showed greasy scales and crusts extending to bilateral ears [Figure 1]. There were multiple erythematous papules and crusted erosions over his trunk. Upper and lower limbs had few erythematous papules and large violaceous plaques over lower limbs [Figure 2]. Oral mucosa showed cobblestone appearance over hard palate while ocular examination was suggestive of squamous blepharitis.
On clinical examination, differential diagnosis of seborrheic dermatitis, LCH was formulated.
There was no any abnormality detected in his laboratory investigations, including complete blood counts, and renal and liver function tests. Serum osmolality and urine osmolality were normal. Radiological investigations did not reveal any lytic bony lesions. Magnetic resonance imaging brain was normal.
A histopathological study from scaly lesion at retroauricular area revealed epidermis containing dense polymorphs with necrotic layers in epidermis. Papillary dermis showed dense collection of histiocyte-like cells having moderate to abundant cytoplasm and round nuclei with occasional nuclei showing longitudinal grooves. There was moderate collection of polymorphs admixed with histiocytes [Figure 3]. Immunohistochemistry showed CD1a [Figure 4] and S-100 positivity with negative staining for CD68.
|Figure 3: Histopathological examination (H and E). Papillary dermis showed dense collection of histiocyte-like cells having moderate to the abundant cytoplasm and round nuclei with occasional nuclei showing longitudinal grooves. There was moderate collection of polymorphs admixed with histiocytes. Langerhans histiocytes with pale cytoplasm and elongated nucleus (×40)|
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Histopathological examination of the lesion from lower limb plaques showed hyperkeratosis, hypergranulosis, basal cell degeneration, effacement of the rete ridges, and eosinophilic colloid bodies in the lower epidermis and superficial dermis which was suggestive of lichen planus.
Based on histopathology and immunohistochemistry, diagnosis of LCH and lichen planus was made, and the patient is being treated with thalidomide 50 mg/day and topical steroids. The patient showed satisfactory response to the therapy without any systemic involvement or complications till date.
| Discussion|| |
LCH is a group of disorders histologically characterized by the proliferation of Langerhans cells. They are thought to act as antigen-presenting cells during induction of immune responses. Langerhans cells may represent a “ first line” of sensitization of the immune system, leading to clearance of the antigen or to pathological phenomena. It is not known, however, what leads to the proliferation of these cells in the histiocytosis lesion. The etiology and pathogenesis of LCH are still unclear; however, the following two different hypotheses have been proposed: A disorder of immune regulation or a neoplastic process. The presence of aggregates of other immunologically active cells in lesions, the presence of thymic abnormalities, and a deficiency in the number of suppressor T lymphocytes and increased cytokines suggest an exuberant reaction of Langerhans cells to an unknown antigen or neoantigen. However, the monoclonal proliferation of Langerhans cells infers the neoplastic origin of the disease.
Different organs and systems may be affected by LCH, particularly bone, most commonly the skull and maxillary bones. Soft tissue involvement may occur, whereby lymph nodes, the lungs, and mucous membranes are commonly affected.
Skin involvement occurs in 50% of patients with isolated 'skin-only' disease in about 10%. The most common presentation is with a “seborrhea-like” eruption, often initially misdiagnosed as “cradle-cap.” As in our case had typical greasy, scaly skin lesions over seborrheic distribution. Other skin manifestations include papules, vesicles, crusted plaques, nodules, and purpuric nodules. Patients with skin-only LCH may have spontaneous regression or progression, particularly in the infant, to disseminated, sometimes fatal disease. Our case, fortunately, did not have any systemic abnormality and hence had a good prognosis. All young babies with skin-only LCH should be carefully followed as approximately 50% will progress to multisystem disease, which may be fatal. In general, skin-only LCH has a good prognosis and should not be overtreated.
Lichen planus is T-cell mediated autoimmune disease associated with damage to basal keratinocytes, which may have been triggered by viruses, medications, or some exogenous allergen. An extensive search in the literature did not reveal any co-existence of LCH with Lichen planus (LP). There was no any etiopathological link found for this association. We report this case because of its rarity.
| Conclusion|| |
LCH along with lichen planus is an uncommonly encountered diagnosis with a common clinical presentation. Although LCH is rare, it must be one of the differentials while managing a child with seborrhea-like clinical features.
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Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4]