MDVI Vol. 40 No.Suplemen Tahun 2013: 32s –35s Case Report

MYCOSIS FUNGOIDES WITH ERYTHRODERMIC MANIFESTATION IN A SIX YEAR OLD BOY

Evelyn Nainggolan, Heru Nugraha, Ridha Rosandi, Siti Aisah Boediardja, Triana Agustin, Githa Rahmayunita, Rahadi Rihatmadja, Sondang Panjaitan Sirait

Dermatovenerology Department Faculty of Medicine Universitas Indonesia/Dr. Cipto Mangunkusumo Hospital, Jakarta,

ABSTRAK

Mikosis fungoides ialah limfoma sel T kutaneus primer yang umumnya terjadi pada orang dewasa. Onset saat anak-anak dapat terjadi pada 0.5-5% kasus. Insidensi yang lebih rendah pada anak-anak terjadi karena ketidakmampuan mengenali manifestasi awal dan keraguan dalam melakukan biopsi serial. Gambaran klinis dapat bervariasi, dapat berupa papul hingga lesi eritematosa dengan skuama dan eritroderma. Kemungkinan mikosis fungoides seharusnya dipertimbangkan pada kasus dermatosis kronik yang rekalsitran terhadap terapi. Anak laki-laki 6 tahun, menderita lesi eritematosa berskuama yang rekuren sejak usia tiga tahun. Awalnya lesi berupa plak eritematosa dengan skuama putih tebal pada wajah, lengan, dan tungkai kemudian menyebar ke seluruh tubuh menjadi eritroderma yang rekalsitran terhadap terapi. Pembesaran kelenjar getah bening ditemukan pada leher, aksila, lipat paha kanan. Pemeriksaan histopatologis serial dari kulit memberikan gambaran yang cocok dengan eritroderma . Biopsi diambil dari kelenjar getah bening inguinal dan diwarnai antibodi monoklonal CD3, CD4, dan CD8 memberikan gambaran lesi limfoploriferatif sel T sehingga menegakkan diagnosis mikosis fungoides. Kemungkinan suatu kelainan Limfoproliferasi Sel T harus dipertimbangkan apabila ditemukan kasus eritroderma dengan limfadenopati multipel dan tidak memberikan respon saat pengobatan. Biopsi serial dan pewarnaan imunohistokimia dari kulit dan kelenjar getah bening dapat membantu dalam konfirmasi diagnosis tersebut. (MDVI 2013; 40/s: 32s – 35s)

Kata kunci: eritroderma, limfadenopati multipel, mikosis fungoides, imunohistokimia

ABSTRACT

Mycosis fungoides is a primary cutaneous T cell lymphoma (CTCL) commonly occuring in adults. Onset during childhood may appear in 0.5-5% of cases. Lower incidence in children due to lack of recognition of initial manifestation and hesitancy to perform serial biopsies in children. Clinical presentations varies, it may appears as papules to red patches with scales and . The possibility of mycosis fungoides should be considered in chronic dermatoses recalcitrant to therapy. A 6-year old boy had suffered from recurring scaly red patches since age three. Initially manifested as erythematous plaques with white thick scales on face, arms, and legs which later spreaded all over the body becoming erythroderma which was recalcitrant to therapy. Lymph nodes enlargements were found on the neck, axilla, and right groin. Serial histopathologic examination from skin matched psoriatic erythroderma. The biopsy taken from inguinal lymph nodes and stained with CD3, CD4, CD8 monoclonal antibody established T-cell lymphoproliferative lesion confirmed the diagnosis of mycosis fungoides. In erythroderma with multiple lymphadenopathies recalcitrant to therapy we should consider the possibility of T cell lymphoproliferative disorder. Serial biopsies accompanied with immunohistochemistry staining from skin and lymph node in this patient confirmed the diagnosis of mycosis fungoides. (MDVI 2013; 40/s: 32s – 35s)

Key words: erythroderma, multiple lymphadenopathies, myosis fungoides, immunohistochemistry Korespondensi:

Jl. Diponegoro No. 71, Jakarta Pusat Telp. 021 – 31935383 Email: [email protected]

32 S

S A Sulistyowati Mycosis fungoides with erythrodermic manifestation

INTRODUCTION CASE

Mycosis fungoides is a primary cutaneous T cell A 6 year-old boy had suffered from recurring scaly red lymphoma (CTCL) that occurs primarily in adults.1-3 patches since age three. The lesions initially manifested as Although 75 % of patients are diagnosed after the age of erythematous plaques with white thick scales on face, arms, 50, onset of the disease during childhood may occur in and legs. On October 2009, the patient was diagnosed as 0.5-5% of cases.1 Many factors may contribute to the psoriasis by a dermatologist, and had been treated with seemingly lower incidence in children, including lack of salicylic acid and liquor carbonis detergent ointment. The recognition of initial manifestation and hesitancy to lesions later spreaded all over the body becoming a perform serial biopsies in children. In general, patients permanent erythroderma. The patient was further treated with mycosis fungoides during childhood are more likely with systemic corticosteroid and cyclosporine but without to present with mild disease and as a result, seem to have any improvement. In 2010, the patient stopped visiting the better disease spesific survival than older patients.1,2 dermatologist due to financial problem, and the lesions The clinical presentations of mycosis fungoides is were only treated with vaseline. Because the lesions variable. Children may show papules to red patches with worsened, the patient came to dr. Cipto Mangunkusumo scales and erythroderma, with variable degrees of pruritus. Hospital in 2012. The lesions may mimic many other skin disorders, including On physical examination there were scaly red psoriasis, atopic dermatitis, , vitiligo, patches distributed all over the body accompanied with alba, and .1-3 The possibility of mycosis lymph nodes enlargements on the neck, axilla, and right fungoides should be considered in chronic dermatoses groin. All of the nails looked hyperkeratotic and club recalcitrant to therapy. The diagnosis of mycosis fungoides is shaped. There were lagophthalmos of both eyes (figure 1). made based on the histologic findings of skin biopsy in A first biopsy from the skin was done. The conjunction with immunohistochemical examination. histopathologic examination matched with psoriatic Histopathologic features in patients with early involement erythroderma (figure 2). Due the presence of suspicious of may be difficult to distinguish from other diseases. Therefore atypical lymphocyte cells, we continued with immu- serial biopsies should be done to confirm the diagnosis.1,2 nohistochemistry staining. Immunohistochemistry staining There are a variety of treatment options for children with CD3, CD4, CD5, CD43, CD8, and Ki-67 showed with mycosis fungoides, but no standard protocol exist and positivity on most of the lymphocyte cells, suggestive a T- the ideal therapy remains unclear. Chemotherapy protocol cell proliferative disorder. However, there were only a few with methotrexate, cyclophosphamide and prednisone is atypical lymphocyte cells. Another biopsy was planned to one of the treatment options.1,4 confirm this result.

Figure 1a&b. Red plaques with white thick scales universalis and lagophthalmos of both eyes

33 S

MDVI Vol. 40 No.Suplemen Tahun 2013: 32s –35s

Figure 2a,b,c. The first histopathologic examination matched with psoriatic erythroderma. There were epidermal thinning, acanthotic, spongiotic, exocytosis of lymphocyte, focal parakeratosis. Inflammatory infiltrates consist of polymorphonuclear leukocytes and histiocytes in papilar dermis, perivascular, and periadnexal

A second biopsy was performed from two part of the A third biopsy was then taken from inguinal lymph skin lesions. One specimen was taken from hyperpigmented node and stained with CD3, CD4, CD8 monoclonal patch and revealed hyperkeratotic of epidermis, focal antibody. These CD3, CD4, CD8 immunohistochemistry parakeratosis, hypogranulosis, acanthotic, mild spongiotic, staining showed positivity on lymphocyte cells, therefore exocytosis of polymorphonuclear leukocytes and established T-cell lymphoproliferative lesion and confirmed lymphocyte. There were chronic inflammatory infiltrates in the diagnosis of mycosis fungoides. papilar dermis, interstitial, perivascular, and periadnexal. The patient was referred to pediatric department for Another specimen was taken from erythematous plaque and chemotherapy protocol and further treated with metho- revealed hyperkeratotic of epidermis, mild parakeratotic, trexate, cyclophosphamide and prednisone. From obser- hypogranulosis, acanthotic, elongation of rete ridges, vation around 3 months the lesions showed a clinical exocytosis of polymorphonuclear leukocytes and lymphocyte. improvement at subsequent follow up. The scaly red The dermis consisted of chronic inflammatory infiltrates patches became thinner and hyperpigmented. The lymph interstitial, perivascular, and periadnexal. The conclusion of node enlargement dissapeared. There was a moonface this second histopathologic examination was consistent with condition due to corticosteroid side effect (figure 3). psoriatic erythroderma.

Figure 3 a&b. Clinical improvement after three series of chemotherapy protocol. The scaly red patches became thinner and hyperpigmented

34 S

S A Sulistyowati Mycosis fungoides with erythrodermic manifestation

DISCUSSION protocol consists of methotrexate, cyclophosphamide and prednisone. After been given three series, clinical and A rare case of mycosis fungoides in a 6-year-old boy laboratory examination showed good results. presenting with erythroderma and multiple lympha- denopathies is reported. Mycosis fungoides is usually REFERENCES reported in adults, although the onset of the disease may occur during childhood, due to lack of recognition of the 1. Paller AS, Mancini AJ. Histiocytoses and malgnant skin diseases. initial manifestation. In: Paller AS, Mancini AJ, editor. Hurwitz clinical pediatric dermatology. Fourth edition. Edinburgh: Elsevier; 2011. p.219-33. The initial manifestation of mycosis fungoides in 2. Kazakov DV, Burg G, Kempf W. Mycosis fungoides. In: Burg G, this patient simulated psoriasis, and made the early Kempf W. Cutaneous lymphomas. New York: Taylor and Francis; diagnosis difficult. The longthy duration of erythrodermic 2005. p.101-12. as the result of extended psoriasis and poor response to 3. Nashan D, Faulhaber D, Staender S, Luger TA, Stadler R. Mycosis fungoides: a dermatological masquerader. Br J Dermatol. 2007; standard psoriasis therapy led us to consider the possibility of 156: 1-10. a T cell lymphoproliferative disorder. None of serial skin 4. Horwitz SM, Olsen EA, Duvic M, Porcu P, Kim YH. Review of the biopsies with immunohistochemistry staining give specific treatment of mycosis fungoides and sezary syndrome: a stage-based results. But, inguinal lymph node biopsy staining with approach. J National Comprehensive Cancer Network. 2008; 6(4): 436-42. monoclonal antibody established the diagnosis of mycosis fungoides. Presently the patient is still under chemotherapy

35 S