P105 Mycosis fungoides or coexistence pemphigus and mycosis fungoides

Abstract

Introduction Mycosis fungoides is the most common type of cutaneous T-cell lymphoma (CTCL). The disease usually develops and progresses slowly and chroniclly. It generally affects the skin, but may progress internally over time, when the neoplastic process involves the entire lymphoreticular system, the lymphonodes and internal organ. Mycosis fungoides is usually diagnosed after the age of 50, but has been seen as early as childhood. Mycosis fungoides develops twice as often in men as in women and is more common in people of African than of European origin. Virus infections, allergies, environmental chemicals and genes have all been suggested as possible causes of this cancer. Mycosis fungoides is a skin cancer characterized by patches, plaques, nodules and tumors with or without ulcers, poikiloderma, erythrodermic form and other more rare symptoms are also seen, including itching alone. Clinical course, prognosis, and management are different from one patient to another. Case report Clinical featuresWe describe an 81-year-old man,admitted to the Clinic of Dermatovenerology in Banjaluka with a several month history of numerous pustules and green-yellow colored crusting on normal and erythematous skin of face, hull and the upper extremities. The patient had a lot of documentaion about his previous disease. The first skin changes appeared in 1999 as erythematous plaques on the whole body, when the patient was treated, in his city, with local corticostreoid therapy. Skin changes have completely disappeared and patient had no skin changes over 8 years. During the year of 2007, new skin changes appeared in the form of many erythematousqamosus plaques. Then the biopsy was made and diadgnosed as psoriasis vulgaris. He was treated with Azathioprine 150 mg daily and local corticostreoid therapy. After several months there was a worsening of the disease and the patient was hospitalized at our Clinic with many pustulas and green-yellow crusts on normal and erythematous skin of face, hull and the upper extremities. On the scalp was erythema and silvery-white scale. Nikolsky's sign was positive. Laboratory findings SE 17/38,WBC 12,22; other biochemical examinations were in normal values, markers of hepatitis were negative; ANA: was negative; Peripheral blood smear was normal ; PAT (after regression of the disease, because patient was unable to do it previously): was negative Histopathology Intraepidermal bulla filled with many granulocytesites, fibrin and necrotic cells, and it looked more like pustula. Inflamatory cells forming bands and penetrate in dermis, deep between dermal papillae. There was a dense perivascular infiltrate composed of neutofils, eosinophils, lymfocits, nuclear powder and blob of hemosiderin pigment. Pathohistological image corresponds to pustulosis form of pemphigus vulgaris. Treatment Nirypan (methylprednisolone) 60 mg daily during the 21 days, after that we gradually reduced the dose to 4 mg per week, Alfa D3, H-2 blockers, oral antibiotics and local anthiseptic therapy. After the therapy, all of skin changes disappeared, and patient was treated with methylprednisolone 8 mg during the next two years. In June of 2009, two livid plaques came to the appearance on the scalp and back, that were resistant to therapy. Then we made skin biopsy from livid plaq on the back. Histopathology Epidermis was atrophic with overlying hyper and parakeratosis, under epidermis lichenoid lymphonocytic inflammation. There were many areas of epidermotrophism with collections of atypical lymphocytes. The pigmentory incontinence in the dermis was also marked. Imunohistochemical analysis shows: CD3+; CD5+,CD4+; CD8+ T cells in upper dermi. Conclusion Mycosis fungoides is a rare form of T-cell lymphoma of the skin and has variable phenotypic and histopathologic presentations. The diagnosis is made through a combination of the clinical picture and examination, and is confirmed by biopsy and imunohistochemical analysis We present an uncommon and rare case of mycosis fungoides in pustular form, and we are still in dilemma whether it was mycosis fungoides or coexistence pemphigus and mycosis fungoides.