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Psoriasis is a common autoimmune dermatosis representing an interplay between certain genetic predisposing factors along with clonally restricted Th1 T cells responding to epidermal keratinocyte derived antigen. A unique IL17/IL23 cytokine-rich milieu is pathogenetically significant and conducive to its salient histomorphologic features, such as epidermal hyperplasia and intraepidermal influx of neutrophils. The classic cutaneous manifestation is that of plaque psoriasis also referred to as psoriasis vulgaris with characteristic well-circumscribed erythematous plaques covered by silvery scales. Follicular psoriasis is an uncommon variant manifesting as a scaly folliculocentric hyperkeratotic eruption of the trunk and extremities, irrespective of the presence or absence of conventional lesions of psoriasis vulgaris. In this study we present 5 cases of follicular psoriasis, review the literature, and provide a proposal regarding relevant pathologic findings and potential pathogenetic mechanisms. The incidence of follicular psoriasis is unknown, emphasizing its rarity given the overall incidence of conventional psoriasis in the general population. Owing to the lack of awareness, this clinical presentation is often mistaken for other follicular dermatoses, including bacterial folliculitis, pityriasis rubra pilaris, keratosis pilaris, or follicular eczema.

Pustular psoriasis occurs rarely in children but can present with acute toxicity requiring inpatient admission. For the best approach, medical providers should have an evidence based and systematic treatment algorithm in their armamentarium. Toillustrate this point, we describe a 13-year-old girl who was admitted with generalized pustular psoriasis and who responded dramatically to "wet wrap" therapy and cyclosporine bridged to etanercept. Using this case example, we highlight the three most important considerations in any patient with new onset pustularpsoriasis: avoidance of disease complications, acute "rescue" therapy, and maintenance therapy.

Lymphomatoid drug reactions can mimic endogenous T and B cell lymphoproliferative diseases.We present a novel form of cutaneous drug reaction with features of pityriasis lichenoides (PL), a recognized form of T cell dyscrasia.Ten cases were studied where a cutaneous eruption exhibiting semblance to PL within a few weeks to months after starting a particular drug.The patient cohort comprised 7 females and 3 males with the mean age of 60 years. Widely distributederythematous cutaneous lesions were present in 6 cases whereas a more localized distribution was seen in three cases. The most frequently implicated drugsassociated with the eruption were antidepressants and statins. Histologic examination showed a morphologic picture identical to PL including marked epitheliotropism of mildly atypical lymphocytes, psoriasiform epidermal hyperplasia, dyskeratosis, hemorrhage, and a thick parakeratotic scale. Therewas a significant reduction in the expression of CD7 and CD62L amid the T cells. Regression of the eruption occurred in all cases excluding one.Thefindings conform the categorization of this process as a form of T-cell dyscrasia albeit one that is reversible, dependent on the drug withdrawal. The limitationof our study includes the retrospective design of the study.

Hydralazine-induced small vessel vasculitis is a rare entity with a limited number of cases reported in the dermatologic literature. A characteristic pattern of acral pseudoembolic vesiculopustules with necrosis and ulceration has been suggested along with involvement of the aerodigestive tract, indicating mucosal involvement is an important feature of this disease. We report the case of a patient with hydralazine-induced vasculitis who exemplified this characteristic presentation associated with severe involvement of the aerodigestive tract and gastrointestinal tract bleeding. In addition to the distinctive clinical presentation, the patient's workup revealed the characteristic antineutrophil cytoplasmic antibody (ANCA)-positive serologic profile. Increased recognition of the clinical and serological features of hydralazine-induced small vessel vasculitis may lead to earlier recognition of this disease and decreased time to discontinuation of hydralazine when appropriate. Drug withdrawal is the cornerstone of therapy, and depending on the severity of symptoms, additional immunosuppressive treatment such as corticosteroids may be necessary.

Metastatic Crohn disease is a rare cutaneous manifestation of Crohn disease characterized by granulomatous lesions discontinuous with the diseased areas of the gastrointestinal tract. We report a case of a 32-year-old woman with history of Crohn disease who was admitted for treatment of cellulitis after presenting with a tender erythematous plaque of the left calf. Microbiological tests including tissue cultures were negative. A skin biopsy revealed granulomatous dermatitis consistent with metastatic cutaneous Crohn disease. Owing to concomitant perianal fistulas and abscesses and prior infusion reaction to infliximab, the patient was treated with certolizumab, a pegylated tumor necrosis factor (TNF) inhibitor combined with methotrexate resulting in complete resolution of the skin lesion. This case emphasizes the importance of recognizing this rare skin manifestation of Crohn disease and adds certolizumab as one of TNF inhibitors useful in the treatment of metastatic cutaneous Crohn disease.

Reed syndrome, also known as Multiple Uterine and Cutaneous Leiomyomas (MCUL), is an autosomal dominant defect in the fumurate hydrase gene, leading to a predisposition of leiomyomas of the skin and uterus. Patients with Reed syndrome may present with cutaneous leiomyomas, uterine leiomyomas and/or leiomyosarcomas. A 37-year-old woman presented to the dermatology clinic with several subcutaneous nodules. Punch biopsy was performed and the diagnosis of angioleiomyosarcoma with supervening degenerative changes was made. Medical history was positive for uterine leiomyomas. These concomitant findings led to the diagnosis of Reed syndrome. At the present time, genetic counseling is a suggested screening parameter for patients with multiple cutaneous leiomyomas. The superficial nature of these lesions and the low staging made surgical excision the preferred and actual treatment method. Adjunct radiation and chemotherapy have not been shown to provide clear benefit of survival. Owing to an association with renal cell carcinoma, a referral for nephrology consultation is also recommended.

Sézary syndrome (SS) is a rare, aggressive form of cutaneous T-cell lymphoma. When patients die from SS, it frequently is due to the sequela of the profound endogenous immunosuppression that is typical of this condition. Most cases of SS represent neoplasms of mature postthymic CD4(+) T cells. We present a case of SS that exhibited an unusual double-positive phenotype in which the neoplastic T cells demonstrated CD4 and CD8 expression. The patient's clinical course was unusually aggressive with rapid clinical demise occurring less than 1 year from the initial cutaneous eruption. Our patient had documented involvement of the skin, peripheral blood, and lymph nodes. We also review other anecdotal reports of postthymic T-cell lymphomas manifesting as a double-positive phenotype primarily in the context of adult T-cell leukemia and T-cell lymphoma. The evolution of the postthymic double-positive T-cell phenotype, especially with regard to SS, and the benign lymphocyte counterpart also is discussed.

The first report of spinous follicular lichen nitidus with perifollicular granulomas was by Madhok and Winkelmann in 1988. Since this report, a few cases of follicular or periappendageal lichen nitidus have been described, in a more localized form or without perifollicular granulomas. We describe a 5-year-old girl with the rare generalized spinous follicular variant of lichen nitidus with perifollicular granulomas.

Lymphomatoid papulosis (LyP) and cutaneous anaplastic large cell lymphoma (ALCL) belong to the spectrum of cutaneous CD30+ lymphoproliferative disorders, an indolent form of T-cell lymphoproliferative disease. We reviewed 21 cases of CD30+ lymphoproliferative lesions expressing cytotoxic profile (CD8+). Seven cases of cutaneous ALCL, 2 cases of systemic ALCL involving the skin, and 12 cases of LyP. The cases of LyP were predominated by small lymphocytes exhibiting a prominent epidermotropic pattern consistent with either type B or type D LyP. Four cases showed co-expression of CD56. The ALCL cases included myxoid features, pseudoepitheliomatous change, and an intravascular component. In all cases that were primary in the skin an indolent clinical course was seen while one patient with systemic myxoid ALCL is in remission following systemic multiagent chemotherapy. The paucity of other neutrophils and eosinophils and concomitant granulomatous inflammation were distinctive features in cases of type B and type D LyP. CD30 and CD45 Ro positivity and a clinical course typical of LyP were useful differentiating features from an aggressive cytotoxic CD8+ T cell lymphoma. In all cases that were primary in the skin an indolent clinical course was observed. CD30 and CD45 Ro positivity and a clinical course typical of LyP were useful in preventing a misdiagnosis of an aggressive cytotoxic CD8+ T cell lymphoma.

We describe 9 patients with a novel variant of lymphomatoid papulosis characterized by prominent localization of the infiltrate around the eccrine coil, resulting in nodular expansion of the coil accompanied by variable granulomatous inflammation. Light microscopy, immunohistochemical analysis using antibodies to CD2, CD3, CD4, CD5, CD7, CD8, and CD30 in 6 cases, and polymerase chain reaction--single-stranded conformational polymorphism analysis for T-cell receptor gamma chain gene rearrangement in 5 cases revealed 2 cytomorphologic patterns (large cell dominant with polymorphous inflammation, small cell lymphocyte--rich with an inconspicuous large cell component [phenotypes, CD30+/CD3+/CD4+/CD7-for large atypical cells; reactive for small lymphocytes]) and clonal restriction in 4 and polyclonality in 1 of the lymphocyte-dominant cases. During an average 6-year follow-up, no lymphomas developed. Recognition of this variant is important--accentuation of the infiltrate around the eccrine coil and cutaneous nerves, presence of granulomatous inflammation, dominance of small lymphocytes in the dermis, and variable extension into the panniculus may lead to diagnostic confusion with entities such as discoid lupus erythematosus, Jessner lymphocytic infiltrate of skin, subcutaneous T-cell lymphoma, and persistent arthropod bite reaction. Our findings suggest that pruritus, a younger age at diagnosis, and a more indolent course are defining clinical features.

Although the histologic characteristics of cryofibrinogenemia have been described in skin lesions, the literature is largely devoid of descriptions of this disorder in other organs. This series is the first to document the histopathologic manifestations of intrapulmonary, intramuscular, and renal cryofibrinogenemia. We describe the histopathologic manifestations of cryofibrinogenemia in 10 cases with manifestations in 4 organ systems: skin in 7 cases, skeletal muscle in 2, lung in 2, and kidney in 1. Irrespective of anatomic site, all lesions showed an occlusive thrombotic diathesis comprising eosinophilic refractile deposits within vessel lumina with extension into the intima, with or without an accompanying characteristic granulomatous vasculitic component. Ultrastructural examination of the renal deposits showed fibrillary material within glomerular capillary lumina with unique morphologic features not previously described. Analysis of plasma from several cases revealed a cold-precipitable protein, which in most cases included a monoclonal paraprotein. The laboratory and histologic distinctions between cryofibrinogenemia and cryoglobulinemia are addressed. We provide guidelines for the proper handling of patient specimens in the workup of cryofibrinogenemia.

The purpose of this study was to correlate the histologic findings of skin lesions clinically suspicious for epidermodysplasia verruciformis with the viral findings in patients with organ transplants or AIDS.Thirty-seven skin biopsies from 17 patients (six with AIDS and 11 with transplants) were studied as a non-randomized, controlled, unblinded case series by in situ hybridization for HPV DNA.Nineteen (51%) of these biopsies were HPV-positive by in situ hybridization either for HPV type 5 (five cases), type 8 (10 cases), type 16 (four cases) or HPV 31 (one case, with one case of dual infection). Only eight of the 19 HPV-positive tissues (42%) showed the classic histologic features of verruca planae. The more common histologic feature significantly associated with HPV detection was a focally thickened and disrupted granular layer (13/19 [68%] vs. 8/18 [44%], p0.04). Dysplasia was evident in 12/19 HPV-positive tissues (63%), which was significantly greater than in patients with congenital epidermodysplasia verruciformis (20%).Oncogenic HPV types are detected in about one-half of skin biopsies from immunocompromised patients with a clinical presentation suspicious for epidermodysplasia verruciformis. Many of these lesions lack the histologic features of verruca planae, a focally thickened granular layer is a marker for viral detection, and the risk for dysplasia in such lesions is much higher than in epidermodysplasia verruciformis not associated with acquired immunosuppression.

We report five cases of aseptic meningitis presenting with high cerebrospinal fluid (CSF) cell counts (260-600 cells/cmm) and mononuclear pleocytosis, suggesting the diagnosis of CNS malignant lymphoma. In four of five cases a reactive background of small lymphocytes, monocytes, and eosinophils was seen. Immunocytochemical studies in all five cases revealed that 100% of the lymphoid cells were T-cells. Three of four cases evaluated for lymphocyte subsets displayed a CD4 to CD8 ratio of 3:1. In the fourth case the CD4:CD8 was 1 to greater than 10; this patient was subsequently proven to have AIDS with HIV meningitis. In this study the cytologic features of the benign atypical lymphoid pleocytosis of aseptic meningitis in contrast to malignant lymphomas in CSF specimens included small or indistinct nucleolus, regular perinucleolar area, smaller widely separated chromatin aggregates, generally ample cytoplasm with perinuclear clearing and polyclonal T-cell immunophenotype.