Regional lymphomatoid papulosis in a child--treatment with a UVB phototherapy handpiece.

Lymphomatoid papulosis (LyP) is a rare skin lymphoproliferative disorder that has been reported only rarely in children. It is included in the World Health Organization classification of cutaneous lymphomas. We report a case of LyP in a 13-year-old Caucasian girl who presented with a 6-month history of recurrent papular lesions on the left upper arm. Histopathologic examination showed a diffuse cellular infiltration of small and medium-sized T lymphocytes CD30+ in the superficial dermis. Treatment was started using a UVB phototherapy handpiece (twice weekly), with resolution of the lesions after 6 weeks of treatment. There was a relapse after 9 months with a good response after six more sessions of treatment. The patient is in good health without lesions after 12 months of follow-up.

[Behçet's disease - Infliximab in the treatment of mucocutaneous manifestations]. / Doença de Behçet - infliximab no tratamento das manifestações mucocutâneas.

Behçet's disease (BD) is chronic, relapsing and multisystem inflammatory disorder, whose diagnosis is essentially clinical. In recent studies, the antagonists of tumor necrosis factor alpha have shown good results in the treatment of mucocutaneous manifestations of BD. The authors describe three cases of BD with serious mucocutaneous involvement treated with infliximab. CLINICAL CASE 1: A 16-year-old female patient with an 8-year history of severe bipolar aphtosis. A rapid clinical response to biological therapy was verified, with partial and total remission of ulcers at 2nd and 6th week of treatment, respectively. CLINICAL CASE 2: A 56-year-old female patient with chronic nodular panniculitis of lower limbs, arthralgias and orogenital ulceration. After 6 weeks of treatment with infliximab, a complete clinical remission was observed. CLINICAL CASE 3: A 50-year-old female with a 15-year history of relapsing oropharyngeal ulceration. A good clinical response to biologic therapy was observed. At 22nd week it was interrupted because frequent dental abscesses. In our experience, infliximab is an effective therapeutic alternative that should be considered in the presence of mucocutaneous manifestations resistant to conventional therapies.

Localized abdominal idiopathic lipodystrophy.

Localized loss of subcutaneous tissue can occur after panniculitis, injections of corticosteroids and other drugs, or associated with infectious, autoimmune or neurologic diseases. The "idiopathic lipoatrophies" are a group of poorly characterized diseases, with focal disappearance of subcutaneous fat, and usually the thighs, abdomen or the ankles are affected. Three subtypes have been described based on clinical presentation: lipoatrophia semicircularis, annular lipoatrophy of the ankles and centrifugal lipodystrophy. We describe a 52-year-old female patient who developed a localized atrophy of the abdominal areas over a period of 3 months without any inflammatory signs over the evolution of the disease. The patient denied any previous local trauma or medication of any type. The atrophy stabilized, showing no progression over the last 6 years. The histopathological examination was normal except for the absence of subcutaneous fat, although the biopsy was taken down to the fascia. There was no clinical or serologic evidence of autoimmune diseases and laboratory testing for Borrelia burgdorferi infection was negative. Other causes of localized lipoatrophies were excluded and the final diagnosis was localized idiopathic lipodystrophy. Our patient is the second report on an abdominal lipodystrophy, with no previous inflammatory signs, absence of subcutaneous fat and no associated pathogenic factor. There is no established treatment for idiopathic lipodystrophy, and the lesions do not tend to resolve spontaneously.

Papulose Bowenóide: um caso clinico / Bowenoide papulosis: a clinical case

Descrevemos o caso clínico de um doente com papulose bowenóide localizada na pele da prega inguino-escrotal direita, tendo sido feita comprovaçao histopatológica e identificaçao de Papilomavírus Humano tipo 16 em material de biópsia.