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A 21-year-old female-to-male transgender individual with a history of Crohn disease presented with enlarging perianal papules that initially were misdiagnosed as condyloma acuminatum. A biopsy specimen demonstrated granulomatous inflammation characteristic of Crohn disease. Although the patient's Crohn disease had been quiescent for years, a subsequent evaluation revealed the presence of a rectal stricture that was then dilated. Perianal skin tags with granulomatous inflammation are one of many perianal manifestations of Crohn disease. Increasing numbers or size of these lesions may herald worsening of more proximal Crohn disease, as in our patient. We review the epidemiology, classification, and management of perianal skin tags in patients with Crohn disease.

A 58-year-old lung transplant patient developed worsening shortness of breath and indurated erythematous plaques on the lower left leg. A skin biopsy specimen revealed a dense angiocentric and angioinvasive infiltrate in the mid to reticular dermis and panniculus containing large, atypical lymphocytes with convoluted nuclei and prominent nucleoli. Immunohistochemical stains showed that the atypical cells were of B-cell origin, and that Epstein-Barr virus was present. Molecular studies demonstrated B-cell clonality. The patient was successfully treated with rituximab. The clinical and pathologic features in this case represent posttransplantation lymphoproliferative disease with features of lymphomatoid granulomatosis. The case also highlights the importance of clinical and pathologic examination of cutaneous lesions in the evaluation of lymphoproliferative disorders for patients undergoing transplantation.

18F-Fluorodeoxyglucose (FDG)-positron emission tomography (PET) is a unique functional/metabolic imaging modality that is efficacious in nodal staging and detection of extranodal involvement for a variety of lymphomas. We report its novel use in evaluating tumor burden and response to therapy in two patients with cutaneous lymphomas. A 24-year-old woman with aggressive subcutaneous panniculitic T-cell lymphoma associated with fever, arthralgias, lymphadenopathy, mild anemia, and widespread painful lesions refractory to multiple treatment strategies exhibited intense uptake of a glucose analogue at sites of clinically apparent (and clinically imperceptible) disease. Denileukin diftitox therapy resulted in clinical remission, and a repeat PET scan failed to detect residual foci of malignancy. A 38-year-old man with a more indolent multifocal primary cutaneous follicle center B-cell lymphoma characterized by few systemic symptoms and slowly evolving lesions demonstrated only mild glucose analogue uptake at sites of disease. Remission was achieved by radiotherapy and intravenous rituximab, and confirmed by a repeat PET scan. Extracutaneous disease was not evident in either patient by this technique. These preliminary data suggest that FDG-PET may be useful in determining disease activity at the time of initial diagnosis, after treatment, and evaluating a suspected recurrence.

Annular lichenoid dermatitis (ALDY) is a rare dermatosis that is most often seen in children and young adults and is characterized by annular patches with raised borders, most frequently on the trunk and the groin. A distinct lichenoid tissue reaction involving the base of the rete, resulting in squared-off rete ridges, helps to differentiate this from other lichenoid dermatoses and mycosis fungoides (MF). Herein, we report an additional case of this condition in a 7-year-boy, whose biopsy exhibited the typical quadrangular rete alteration and also contained distinct aggregates of CD8+ lymphocytes, Langerhans cells and colloid bodies within the involved rete. A literature review with emphasis on the clinical and histopathological differential diagnosis reveals additional clinical features of ALDY to potentially help differentiate this entity from annular presentations of mycosis fungoides. [ABSTRACT FROM AUTHOR]

Perifollicular fibroma is a cutaneous hamartomatous proliferation of the pilar connective tissue sheath. We describe a patient with multiple perifollicular fibromas and analyze the literature on this topic. Histologically, perifollicular fibroma is characterized by a concentric arrangement of collagen fibers surrounding a generally unaltered hair follicle. Clinically, it is usually multiple and occurs predominantly on the face and upper trunk. This clinical presentation is similar to that observed in patients with the Birt-Hogg-Dubé syndrome where, in addition to perifollicular fibromas, fibrofolliculomas, trichodiscomas, and acrochordans are found. Several reports of multiple peifollicular fibroma prior to the recognition of this syndrome may, in fact, represent cases of the Birt-Hogg-Duké syndrome. [ABSTRACT FROM AUTHOR]

The article discusses the treatment of a 53-year-old white woman suffering from Sézary syndrome. Extracorporeal photopheresis, psoralen, UV-A, and interferon alfa-2b, 1.8 million units were injected subcutaneously three times weekly. Oral bexarotene in the quantity of 150 mg daily was also added to the medication. The patient's erythroderma and pruritus showed improvement but three months later the patient's erythroderma and pruritus recurred. Treatment with psoralen-UV-A was stopped.

Cutaneous T cell lymphomas (CTCLs) are a heterogenous group of lymphoproliferative disorders caused by clonally derived, skin-invasive T cells. Mycosis fungoides (MF) and Sezary syndrome (SS) are the most common types of CTCLs and are characterized by malignant CD4(+)/CLA(+)/CCR4(+) T cells that also lack the usual T cell surface markers CD7 and/or CD26. As MF/SS advances, the clonal dominance of the malignant cells results in the expression of predominantly Th2 cytokines, progressive immune dysregulation in patients, and further tumor cell growth. This review summarizes recent insights into the pathogenesis and immunobiology of MF/SS and how these have shaped current therapeutic approaches, in particular the growing emphasis on enhancement of host antitumor immune responses as the key to successful therapy. [ABSTRACT FROM AUTHOR]