bluuebunny/crossref_metadata_2025 huggingface dataset:
Photosensitivity in an HIV‐Infected Man
Authors: Manion D. , Tamjidi P. , Santmyire‐Rosenberger B. , Dugan E.
Published: August 2010

Since the emergence of the AIDS epidemic, various associated skin lesions have been recognized. One interesting phenomenon is chronic photosensitivity dermatitis. We report the case of a 44‐year old homeless male with a 14‐year history of HIV and a CD4 count of 350. He presented to us with a 5‐month history of a pruritic photodistributed eruption. Physical examination revealed that he had erythematous, scaly, lichenified, and crusted plaques involving the face, neck, upper chest, and extensor extremities. Sparing of the areas under his watch and bracelet was found. A skin biopsy specimen showed psoriasiform dermatitis with an overlying thick, impetiginized, and hemorrhagic crust. There was prominent single cell necrosis throughout all epidermal layers. A lichenoid dermal infiltrate consisted mainly of lymphocytes and eosinophils. The patient’s presentation was consistent with a photosensitive psoriasiform interface dermatitis. The pathogenesis of this photodermatitis is unclear. Proposed mechanisms include the formation of a neoantigen due to UV exposure, depressed levels of endogenous scavengers leading to increased oxidative stress, and the onset of a pellagra‐like dermatosis due to niacin‐tryptophan deficiency. It is likely that several of these processes are acting in concert to cause this photoeruption.
Onychoblastoma – Hamartoma of the Nail Unit: A New Entity?
Authors: Misciali C. , Fanti P.A. , Iorizzo M. , Piraccini BM , Tosti A.
Published: August 2010

A 50‐year‐old Peruvian man presented with a 4–5 years history of a 1,2 × 0,7 cm subungual verrucous mass that extended from the proximal nail fold to the hyponychium of the 2nd left toe. Histology of the nail bed showed a nodular tumour with multiple structures, similar to the infundibulum of the follicles, and germ like aggregations often joined to a follicular papilla. Few scattered neoplastic cells were necrotic. The stroma surrounding the nodular aggregations was made up of delicate fibrillary bundles of collagen associated with numerous oval fibrocytes. The histological findings remind us a neoplasm with follicular differentiation: trichoblastoma. A tumor with these histological aspects on the nail unit has never been reported. We name this benign neoplasm, which probably represents an hamartoma, onychoblastoma. Differential diagnosis of this case includes basal cell carcinoma (BCC) of the nail unit which is rare and mostly localised in the nail folds. The main histopathologic findings of nodular BCC are asymmetry, ulceration and its poorly circumscription. The hair follicle and the nail share similar embryological and anatomical features and the occurrence of a tumor with follicular differentiation in the nail further demonstrates the common origin of these adnexal structures.
Clinicopathological and Immunohistochemical Study of Eccrine Porocarcinoma (Malignant Eccrine Poroma)
Authors: Ogawa F. , Arai E. , Tsuchida T. , Kuramochi A. , Mitsuhashi T. , Ban S. , Shimizu Y. , Hirose T. , Shimizu M.
Published: August 2010

Porocarcinomas are thought to be differentiated into the acrosyringeal portion of the sweat duct, and they are relatively rare. It may develop from a long standing eccrine poroma undergoing malignant transformation or may arise de novo. We evaluated pathological and immunohistocheimical findings of six cases of eccrine porocarcinoma. Special stains (PAS and PAS with diastase) and immunohistochemical stainings (CEA, CAM5.2, CK7, bcl‐2, p53 protein, and MIB‐1) were performed. We observed asymmetrical architecture, cytological pleomorphism, prominent nucleoli, ductal differentiation, intracytoplasmic lumina, mitotic activity, and melanin colonization. Three patients were male and four patients were female. The average age was 76 years. The time interval from tumor onset to treatment ranged from 6 months to 10 years. The mean size was 3.2 cm, and the mean depth of invasion was 1.0 cm. Four tumors were found on the head, one on the hip, and one on the foot. Regional lymph node metastases were noted in one case. Microscopically, luminal spaces and intracytoplasmic lumina were noted in all cases. Immunohistochemically, the mean MIB‐1 index was 41%. P53 immunoreactivity was detected in five cases. Interestingly, a case of porocarcinoma with lymph node metastases showed high mitotic figures as well as less lymphocytic infiltration.
Ectopic Meningothelial Hamartoma of the Scalp
Authors: Osunkoya A.O. , Foster T. , Pitha J
Published: August 2010

A 7‐month‐old male infant presented with a 2 month history of a solitary, well circumscribed, 1.2 cm right parietal scalp lesion. An MRI excluded intracranial extension of the lesion. Grossly the excised lesion was tan‐grey, soft to rubbery with focal areas of hemorrhage. On histology, the lesion demonstrated elongated strands of haphazardly arranged fibrous tissue and meningothelial elements with clusters of plump, cuboidal epitheloid cells extending along connective tissue planes in the dermis and subcutaneous tissue. The meningothelial elements were intimately admixed with mature adipose tissue and proliferation of small to medium sized vessels. The overlying epidermis was unremarkable. No mitotic figures or areas of necrosis were identified. The meningothelial cells were positive for Vimentin and Epithelial Membrane Antigen. S‐100 protein was negative and CD‐34 was positive only in the endothelial cells. The gross, histologic and immunohistochemical features are consistent with ectopic meningothelial hamartoma of the scalp. The meningothelial elements characteristic of this entity are believed to arise from displaced, ectopic meningothelial rests in the scalp and are thus regarded as simply representing a proliferation of the hamartomatous process.
A Case Report of Unusual Multiple Minute Digitate Hyperkeratosis
Authors: Pierson D. , Seilstad K. , Rogers R.
Published: August 2010

We report a 43‐year‐old woman, in otherwise excellent health, who presented with a 4 year history of an asymptomatic, papular condition described as ‘tiny little spines’ involving the skin. On examination hundreds of discrete, palpable 1‐mm projections were present on the upper torso and arms. Histopathology showed a small focus of acanthotic epidermis forming a small spire. Overlying the subtle epidermal elevation was a column of compact hyperkeratosis with focal parakeratosis. Epidermal atrophy and dermal lymphocytic inflammation, characteristic of Flegel’s disease, were not observed. No features of keratosis pilaris were noted. A diagnosis of multiple minute digitate hyperkeratoses was rendered. Relatively rare multiple minute digitate hyperkeratoses is a non‐follicular disorder of keratinization of unclear etiology. Previously ascribed names include, but are not limited to, disseminated spiked hyperkeratosis and minute aggregate keratoses. A classification scheme with sporadic, post‐inflammatory (recently challenged as another entity) and familial (autosomal dominant) types has been proposed. Other classifications are based on topography and histology or age of onset: early (20’s and 30’s) and late (40’s‐50’s). Paraneoplastic syndromes have been associated with late onset type. In summary, we report a sporadic case of late‐onset multiple minute digitate hyperkeratosis, an unusual and relatively rare disorder.
Incontinentia Pigmenti in a Male with Mosaic Klinefelter Syndrome: The Role of Fish Analysis
Authors: Reutter J. , Goldstein J. , Prose N. , McDonald M. , Selim M.
Published: August 2010

Incontinentia Pigmenti (IP) is an X‐linked dominant genodermatosis with cutaneous, skeletal, ocular, neurological, and dental abnormalities. It has been mapped to chromosome Xq28 encoding the NF‐kB essential modulator (NEMO). NEMO regulates many genes, such as those that prevent apoptosis. IP is usually lethal in males. However, patients with Klinefelter syndrome (47, XXY) are able to survive due to one normal X chromosome. We report a case of a male who presented after birth with multiple vesicles in a linear distribution on the calf progressing to persistent hyperpigmentation. At one month of age a punch biopsy showed verrucous hyperplasia with mild hypergranulosis, necrotic dyskeratinocytes and a lichenoid infiltrate composed of lymphocytes, eosinophils and melanophages. A diagnosis of IP was rendered. No additional physical findings were noted. The patient was evaluated for Klinefelter syndrome. While his karyotype was 46, XY by conventional cytogenetics, a dual color interphase FISH detected 92.5% 46, XY and 6.5% 47, XXY. Our patient’s unique genetic array allowed a mechanism to escape and otherwise fatal disorder. This case illustrates the potential utility of using FISH analysis to detect Klinefelter syndrome in a male patient with IP and normal conventional chromosomal analysis.
Malignant Myoepithelioma of the Sole
Authors: Sakaki Mika , Hirokawa Mitsuyoshi , Wakatsuki Shingo , Yoshida Eri , Toshiaki Sano
Published: August 2010

76‐year‐old man presented with a hen’s egg‐sized elevated nodule in his left sole in 4/04. He had been hospitalized because of icterus since 3/04. Although cancer of the pancreatic head had been strongly suspected on the imaging, he had refused surgical operation. No metastasis of the cancer had been found on the imaging. A dermatologist who biopsied the nodule in his left plantar recognized the swelling of inguinal lymph nodes on the same side. Histologically medium sized oval cells formed solid nests in the dermis, rendering them lobulated appearance. Some of the nests showed myxoid change in the stroma where tumor cells often showed cord‐like arrangement. The border with surrounding tissue was relatively clear. The cells had round, and often eccentrically located nuclei with one or two small nucleoli and moderate to scarce amount of eosinophilic cytoplasm. Mitosis was readily identifiable; >50/10HPF. Necrosis was not found. Immunohistochemically tumor cells were reactive for AE1/AE3, vimentin and S‐100 protein and negative for ??SMA, CK14, EMA, HMB 45 and Melan A. MIB‐1 labeling index was over 50%. The lesion was diagnosed as malignant myoepithelioma. The positivity for S‐100 protein seen in this case necessitated us to differentiate it from melanoma.
CD10 and CD20 Expression in a Primary Cutaneous Lymphoma of Mature T Cell CD8 Phenotype.
Authors: Seilstad K , Porcu P , Gupta K , Magro C
Published: August 2010

Introduction: Most primary cutaneous T cell lymphomas are derived from mature T lymphocytes. We encountered a case of primary cutaneous T cell lymphoma with an undescribed phenotypic profile as defined by CD3+, CD8+ lymphocytes that co‐expressed CD10, CD20 and CD30. Results: The patient, a 65‐year‐old woman, presented in 2001 with a thigh mass that was biopsied and held to represent a lymphoblastic T cell lymphoma based on CD10 expression. Two years later the patient developed a recurrence. The pathology showed a large cell lymphoma manifesting a CD8 phenotype although with CD10/CD20 expression. Conclusion: The importance in this case lies in the novel phenotype, namely a mature post thymic T cell lymphoma exhibiting CD10 and CD20 positivity. CD10 expression in primary cutaneous T cell lymphoma in the literature is largely limited to two case reports. CD10, a neutral endopeptidase, cleaves proteins with inflammatory or proinflammatory activity released by dying malignant and benign T cells including those of post thymic origin and may also degrade cytokines which inhibit T‐cell apoptosis. Another unique aspect of our case was CD20 positivity whereby only a small number of prior citations exist, all with a CD8 phenotype as was the case in our patient.
Genetic Identity Analysis, a Procedure that may Have Applications in Clinical Practice
Authors: Skelton H. , Sun W. , Smith K.
Published: August 2010

Genetic identity analysis is a procedure that may be done on paraffin blocks to determine genetic identity or non‐identity of tissues. On occasion biopsy specimens from different patients are mixed up. This becomes a major problem if the subsequent diagnoses require further therapy or have other significant medical implications. We recently had such a case in which three biopsy specimens from a middle age man were possibly mixed up with that from a pre‐teen age boy. The biopsy from the pre‐teen was originally reported as a malignant skin tumor. Initially, the three biopsy specimens from the middle age man were sent for genetic identity analysis, which showed that one biopsy specimen was genetically different. The biopsy specimen from the pre‐teen was then sent, and found to be genetically similar to the two genetically identical specimens originally submitted for the middle age man. Further confirmation of genetic identity could have been obtained by obtain further samples from both patients, but was not felt necessary in this case. We review the procedure used for genetic identity analysis as well as its other applications for this procedure in clinical practice.
Myoepithelioma of the Skin with P63 Expression
Authors: Smith‐Zagone M.J. , Reed J.A. , Schwartz M.R.
Published: August 2010

A case of cutaneous myoepithelioma is reported. A 37 year‐old woman presented with a 2 cm well‐circumscribed dermal nodule of the forehead. The tumor was enucleated with the clinical diagnosis of an epidermal inclusion cyst. Histologically, the tumor was located within the dermis and was well circumscribed. It was composed of spindle‐shaped and epithelioid cells arranged in organoid nests. Focal areas of extracellular hyalinized stroma were present. Well‐defined glandular structures, chondroid matrix, and significant nuclear pleomorphism were absent. The tumor expressed widespread cytoplasmic positivity for cytokeratin (using AE1/AE3) and nuclear positivity for p63. Variable reactivity was noted with EMA and S‐100. The tumor was negative for smooth muscle actin, GFAP, chromogranin, synaptophysin, and CEA. These immunohistochemical results supported myoepithelial differentiation. Myoepitheliomas of the skin are rare neoplasms that have only recently been recognized in the skin. A single publication has reported the diagnostic utility of p63 in the diagnosis of myoepithelial tumors of the skin. Myoepitheliomas often display variable expression of myoepithelial markers, with no single marker that is 100% sensitive. The current case highlights the need for a battery of markers, including p63, to detect myoepithelial differentiation.
Deep Trichophyton Rubrum Infection in the Immunocompromised Host: an Entity Distinct from Majocchi’s Granuloma
Authors: Swick Brian L. , Messingham Michael J.
Published: August 2010

A 64‐year‐old white male presented with a three‐month history of multiple crusted erythematous nodules on the lower legs. The patient was immunosuppressed on prednisone and tacrolimus secondary to liver transplant. Biopsy revealed a fibrinopurulent crust overlying an interstitial, non‐follicular oriented, neutrophilic infiltrate within the dermis. PAS and GMS positive septate hyphae were present within the stratum corneum while yeast‐like forms with pseudohyphal projections, suggestive of Blastomyces dermatitidis, were found within the dermis. There was no evidence of follicular rupture or granulomatous inflammation. Subsequent culture grew Trichophyton rubrum confirming the diagnosis of deep dermatophytosis. This case illustrates several histologic differences in deep dermatophyte infection in the immunocompromised host from invasive dermatophyte infection, Majocchi’s granuloma, occurring in the immunocompetent patient. In the immunocompromised patient there is typically the following: no evidence of fungal invasion via a ruptured hair follicle, suppurative inflammation as opposed to granulomatous inflammation, and atypical fungal forms consisting of swollen hyphae and round, yeast‐like, arthroconidia, as opposed to the slender hyphae seen in Majocchi’s granuloma. In addition, because of the clinical appearance of the lesions, immunocompromised state of the patients, and atypical fungal forms seen on biopsy, an initial diagnosis of systemic mycosis is often incorrectly made.
Scalp Histology in RAPP‐Hodgkin Syndrome
Authors: Thompson CT. , Roberts J.
Published: August 2010

We report unique histologic findings in scalp biopsies from two family members with Rapp‐Hodgkin syndrome. Rapp‐Hodgkin and the related AEC syndrome show a primary scarring alopecia and pili torti. The syndromes, which are related to ectodactyly, ectodermal dysplasia and cleft lip/palate syndrome, have been credited to mutations in the TP63 gene. Most notable on biopsy is a primary scarring alopecia and abnormal hair shaft shape. Calcification in the outer root sheath epithelium, the basement membrane of the lower segment and within naked hair shafts is present. Neutrophils and bacterial rods and cocci are adjacent to the hair shaft in the superior segment, associated with dilated follicular ostia. Perifollicular fibrosis is associated with a variety of inflammatory cell types, mostly lymphocytic. One sample shows interfollicular irregular psoriasiform epidermal hyperplasia with parakeratosis, compact orthokeratosis, hypergranulosis, and intraepidermal neutrophils. Follicular architecture is abnormal with abrupt and linear loss of nucleated matrical cells (B fringe of Adamson). The line of Ayber measures 0.30 mm on average. Hair shafts measure up to 0.16 mm, with variable abnormal cross sectional shape, usually square. Outer root sheath epithelium is disorganized with slight cellular pleomorphism, and the cuticle of the inner and outer root sheath is irregular in thickness.
Impact of the Poisson Distribution on Lymph Node Staging of Malignant Melanoma
Authors: Turner J. , Vollmer R.
Published: August 2010

BACKGROUND: Melanoma patients without lymph node metastases (Stage II) have a variable five‐year survival. The influence of falsely negative lymph nodes on this variability is unknown. DESIGN: The Poisson distribution describes the probability of finding no positive lymph nodes when a certain number are examined. Using this model, we analysed lymph node dissection results from 780 patients with melanoma and published survival results for Stage II patients. RESULTS: In the 294 patients without positive nodes, 18% had fewer than 7 nodes examined. The Poisson probability of finding no positive nodes when 1 to 6 were examined ranged from 0.4 to to 0.8 (mean 0.6). We partitioned survival for stage II into two categories: those with truly negative nodes with a five‐year survival of 80% and those with a five‐year survival matching Stage III (46%). The observed five‐year survival of 77% in Stage II patients implied that the fraction with false negative nodes was also 18%, which corresponded with the subgroup with fewer than 7 nodes examined. CONCLUSION: The Poisson model suggests that the variable survival in Stage II patients may reflect the incorrect staging of patients with stage III melanoma who have limited numbers of lymph nodes examined.
Patients with Complete Digeorge Syndrome and Cutaneous Manifestations: A Histopathologic and Immunohistochemical Study
Authors: Turner J , Markert ML , Burchette J , Herman C , Selim MA
Published: August 2010

BACKGROUND: DiGeorge Syndrome is a congenital anomaly with a constellation of findings including thymic hypoplasia. Approximately 1 in 250 patients has complete DiGeorge syndrome with athymia. Many athymic patients develop severe eczema/erythroderma associated with oligoclonal circulating T cells. DESIGN: Nine biopsies from eight athymic patients with severe rash and circulating oligoclonal T cells were studied. Immunohistochemistry with CD3, CD4, CD8 and TIA‐1 was performed. RESULTS: The stratum corneum showed parakeratosis (78%), most commonly confluent. Neutrophilic abscesses were commonly identified (44%). The epidermis exhibited exocytosis (100%), spongiosis (89%), dyskeratosis (89%), acanthosis (56%), and satellitosis (44%). Dermal changes included perivascular (56%), perifollicular (33%), and perieccrine (11%) lymphocytic infiltrates. Eosinophils were identified (78%); most commonly in both the dermis and epidermis. The majority of lymphocytes were CD3 positive with a slight CD4 over CD8 predominance in the dermis and epidermis. 67% of cases contained TIA‐1 positive cells. CONCLUSION: Skin lesions from patients with complete DiGeorge syndrome who develop a rash that clinically resembles severe eczema/erythroderma display a constellation of histologic findings. The presence of dyskeratinocytes, satellitosis, and parakeratotic scale with neutrophils distinguishes these lesions from eczema and other spongiotic disorders. The pathophysiologic role of the inflammatory cells in this entity requires additional study.
Human Herpesvirus‐8: A Useful Marker for Distinguishing Kaposi Sarcoma and Kaposi Sarcoma‐like Pyogenic Granuloma
Authors: Uzieblo A. , Kohler S.
Published: August 2010

Kaposi’s sarcoma (KS) is a vascular neoplasm associated with human herpesvirus‐8 (HHV‐8) infection. On occasion, KS may histologically mimic pyogenic granulomas (PG), a common benign vascular tumor of the skin. Using immunoperoxidase stains, we examined 28 PG and 4 PG‐like KS for HHV‐8 to determine the specificity of positive staining in this setting. All PG‐like KS demonstrated nuclear staining for HHV‐8. No staining was identified in any of the PG. Furthermore, histologic criteria often used to differentiate between these two entities were not helpful in difficult cases. The only distinguishing features were the presence/absence of HHV‐8 staining and, in some cases, clinical history. The presence of HHV‐8 nuclear staining appears to be a specific marker for KS when comparing PG and PG‐like KS. Given the lack of distinguishing morphologic criteria, we suggest performing immunoperoxidase stains for HHV‐8 on any PG occurring in a clinically atypical setting.
Hyaline Cell‐Rich Chondroid Syringoma‐ Report of Two Cases.
Authors: Veeramachaneni R. , Sanusi I. D. , Li M.
Published: August 2010

Case 1: A 38 year‐old man presented with a painless right post auricular mass, slowly growing for ten years. Clinical impression was a salivary gland lesion. The excised mass measured 3 cm and was gelatinous. Case 2: A 28 year‐old man presented with a lump in the region of the left heel, slowly growing for several years. Excision revealed a lobulated pink‐gray mass measuring 2 cm. In both cases, histology demonstrated a circumscribed encapsulated proliferation of tumour cells surrounded by abundant myxoid stroma. There were almost exclusively hyaline cells with plasmacytoid features, arranged predominantly in nests and sheets with abundant pink cytoplasm and hyaline inclusions. Round to oval nuclei with occasional small nucleoli and rare mitotic figures were seen. Both cases stained strongly positive for low molecular weight cytokeratin, S 100 and vimentin and were negative for desmin, smooth‐muscle actin and muscle‐specific actin. We describe two cases of hyaline cell‐rich chondroid syringoma, a histologically benign entity. The importance in recognizing this entity is that these tumors can easily be confused with malignant neoplasms including malignant melanoma, myxoid chondrosarcoma and alveolar soft part sarcoma. Follow up for a year has shown no evidence of metastases or recurrence in both cases.
Hypertrophy of Pacinian Corpuscles in a Young Patient with Neurofibromatosis
Authors: Yan Shaofeng , Harris Brent T
Published: August 2010

A 24‐year‐old female with a lifelong history of neurofibromatosis type 1 presented with left ring finger pain and decreased sensation for over a year. She has multiple café au lait spots over her entire trunk, upper and lower extremities. She also had 11 surgeries to excise superficial neurofibromas over her body. She first noticed a painful swelling of the proximal phalanx of the left ring finger. She then gradually noticed a loss of sensation distally and a constant numb and tingling feeling distal to the palm and the left ring finger. The clinical impression was neurofibroma. MRI of the tender lesion found no evidence of neurofibroma. Biopsy of the lesion showed hypertrophy of Pacinian Corpuscles. Hypertrophy of Pacinian Corpuscles is a rare condition which has been reported very infrequently. It should be considered in the differential diagnosis of any patient with persistent pain localized to the distal palm or digits in addition to glomus tumor and neuroma. It was thought not to be connected with neurofibromatosis. The finding of this lesion in a patient with neurofibromatosis is somewhat intriguing and the relationship of these two entities is unclear.
CD8+ Lymphomatoid Papulosis in a Patient with Simulataneous Type A, Type B, and Type C Lesions
Authors: Abbott J. , Weenig R.
Published: August 2010

Lymphomatoid papulosis (LyP) is a cutaneous CD30+ lymphoproliferative disorder characterized by recurrent papulonecrotic or papulonodular lesions with histologic features of a malignant lymphoma and an excellent prognosis. Three morphologic types (A, B, and C) of LyP exist and typically display an immunophenotype of CD4+, CD8−, and CD30+. We present a case of a 63‐year‐old woman with a one‐year history of relapsing papulonecrotic skin eruptions. Biopsy of a lesion on the forearm showed a superficial and deep dermal wedge‐shaped infiltrate with large lymphoid cells (Type A LyP). A lesion on the abdomen showed a band‐like superficial dermal infiltrate composed of small lymphoid cells with extensive epidermotropism and cerebriform nuclei (Type B LyP). Lastly, a lesion on the buttock histologically showed a dense, diffuse dermal infiltrate composed of a mixture of small and large lymphoid cells (Type C LyP). In all three lesions, the atypical lymphocytes were immunoreactive to CD8 and CD30 and nonreactive to CD4. CD8 positive cases of LyP are rare in the literature and tend to occur in pediatric patients. Moreover, while approximately 10% of patients may present with lesions of both the type A and type B types, cases showing all three types of LyP are exceedingly rare.
Epstein Barr Virus Related Cutaneous T‐Cell Lymphoma Associated with Juvenile Rheumatoid Arthritis and Methotrexate Therapy
Authors: Barry C.I. , Rashidi H.H. , Bifulco C.B. , DeHoratius D.M. , Heald P.W. , Howe J.G. , Cowper S.E.
Published: August 2010

The risk of lymphoma is significantly increased in patients with juvenile rheumatoid arthritis (JRA) treated with methotrexate (MTX). The most common lymphoma subtypes affecting these patients belong to the B‐cell Non‐Hodgkin category, and are associated with Epstein‐Barr virus (EBV) infection in approximately 25% of cases. EBV rarely infects T‐cells, and is not known to cause T‐cell cutaneous lymphoproliferative disorders in this clinical setting. A 78‐year‐old male, with a long term history of JRA, presented with raised red skin nodules on his right knee, thigh, and shin while undergoing MTX therapy. Histological examination of the lesional biopsy revealed a subcutaneous infiltrate composed of medium to large atypical CD3 positive lymphocytes showing focal positivity in the largest forms for CD30. The presence of EBV in the T‐cells was demonstrated by in situ hybridization for EBV encoded small RNA (EBER). Immunohistochemical stains for CD56, CD8, CD4, and CD15 were negative. To the best of our knowledge, this is the first report of an EBV positive cutaneous T‐cell lymphoma, in a patient with a past medical history of JRA treated with MTX.
Congenital Panfollicular Nevus Associated with Polydactyly: a Second Case Report
Authors: Kim J. , McNiff J.M.
Published: August 2010

A 2 year‐old girl presented with ulnar duplication of the left thumb distal to the interphalangeal joint and syndactyly of the first web space. She also had several asymptomatic pink‐tan cutaneous nodules involving the first and second ray of the left hand and wrist, clinically resembling a linear epidermal nevus. The patient underwent reconstruction of the duplicated left thumb, release of the syndactyly, and excision of the left wrist and hand skin lesions. Microscopically, the cutaneous nodules consisted of numerous well‐circumscribed aggregates of basaloid epithelium regularly distributed beneath the epidermis. No normal hair follicles were identified. There was evidence of incomplete hair follicle differentiation, with follicular germ and papillae, and there was a distinct fibrous sheath around the abortive follicular structures. The features were remarkably similar to a novel entity described by Finn and Argenyi in 2005 as congenital panfollicular nevus. In our case, the congenital panfollicular nevus was associated with polysyndactyly, which may provide evidence to support an important link between limb patterning and skin development. In summary, we describe an unusual congenital nevus comprised of many stages of hair follicle differentiation, and associated with a defect in limb patterning in this child.
HSHV/HHV8‐Associated Primary Cutaneous Plasmablastic Lymphoma in a Patient with Castleman Disease and Kaposi Sarcoma
Authors: Liu W. , Lacouture M. , Jiang J. , Kraus M. , Soltani K. , Shea C.R.
Published: August 2010

We report a unique case of KSHV/HHV8‐associated lymphoma in a 56‐year‐old man with a history of AIDS, Castleman disease, Kaposi sarcoma, and idiopathic thrombocytopenic purpura. Three months following the diagnosis of Kaposi sarcoma of a left cervical lymph node and Castleman disease with bone marrow involvement, he presented with a subcutaneous tender lesion of his left arm. A skin biopsy demonstrated a superficial and deep interstitial nodular infiltrate of severely atypical lymphoid cells showing plasmacytoid features, numerous mitotic figures, and frequent individual apoptotic tumor cells. The morphologic features were those of plasmablastic lymphoma (PBL). Immunohistochemical study showed that the lymphoma cells were strongly positive for CD45, CD30, and KSHV/HHV8 latency‐associated nuclear antigen. The KSHV/HHV8 was also positive in the biopsy sections of the patient’s Kaposi sarcoma and Castleman disease. Epstein‐Barr virus in situ hybridization was diffusely positive. In situ hybridization demonstrated kappa light chain restriction. Although KSHV/HHV8 has been individually associated with Kaposi sarcoma, Castleman disease and PBL, this appears to be the first reported case in which all three entities were present simultaneously in one person, suggesting a critical role of KSHV/HHV8 as a common denominator in the pathogenesis of these diseases.
IDIOPATHIC RETROPERITONEAL FIBROSIS
Authors: Juhl Erik
Published: August 2010

Abstract A case of idiopathic retroperitoneal fibrosis of an unusual site, at the upper pole of the right kidney, is reported. The case was confirmed histologically and treated with glucocorticoid for nine months. This resulted in definite clinical improvement and complete normalization of the elevated ESR and abnormal serum electrophoresis. Compression of the inferior vena cava, demonstrated by cavography, was significantly less marked after the corticoid medication. The patient has been followed for eight months after discontinuation of the treatment, and throughout this period he has remained symptom‐free and fully capable of working. As a conclusion from this case of idiopathic retroperitoneal fibrosis, and from reports in the literature, the author feels that all cases of idiopathic retroperitoneal fibrosis should be treated with glucocorticoid in high doses over a long period, also when surgery is indicated.
FATTY ACID COMPOSITION OF THE PLASMA LIPIDS IN HYPOTHYROID SUBJECTS
Authors: Dyerberg Jørn
Published: August 2010

Abstract A study of seven hypothyroid patients showed an abnormal composition of the esterified fatty acids in the plasma lipids. The results were compared with an investigation of thirty‐one normal persons. Occurrence of long‐chain saturated fatty acids, not observed in the normal persons, was seen rather uniformly. A possible connection between these observations and the hyperlipidemia in hypothyrosis is pointed out.
DOUBLE BLIND TRIAL OF LONG‐TERM ANTICOAGULANT TREATMENT AFTER MYOCARDIAL INFARCTION
Authors: Meuwissen O. J. A. T. , Vervoorn A. C. , Cohen O. , Jordan F. L. J. , Nelemans F. A.
Published: August 2010

Abstract A prospective double blind trial of long‐term anticoagulant medication following myocardial infarction was carried out in Utrecht from May 1, 1964 to January 15, 1966. With the collaboration of all Utrecht cardiologists, all their patients with acute myocardial infarction were included in this trial after a 4‐month interval, to be distributed strictly at random over a phenprocoumon and a placebo group. Supplemental therapy and follow‐up were identical in both groups. Of the total of 138 patients included in the trial, 68 received phenprocoumon and 70 were given a placebo. One patient died in the phenprocoumon group, and eight patients died in the placebo group. This difference is significant (P < 0.01). The difference in the number of reinfarctions was not significant (5 cases and 7 cases, respectively).

After January 15, 1966, when the difference between the two groups had become significant, all patients were further treated with phenprocoumon. Nevertheless, the difference in mortality continued through 1966, with five deaths in the original placebo group and only one death in the original phenprocoumon group.

The mean thrombotest level was 10.7%; patients remained within the therapeutic limits (5–15% thrombotest) during an average of 91% of the entire period of investigation. The mean daily phenprocoumon dose was 3.21 mg. No serious haemorrhagic complications occurred.

The results of our study are consistent with those of several other recently published investigations. The intensity of the hypocoagulability attained is probably of decisive importance in this respect. However, correct calculation of the mean level of hypocoagulability is difficult.

The fact that in the original placebo group the results remained less favourable even after the switch to phenprocoumon emphasizes the necessity of long‐term treatment with adequate doses of anticoagulants following myocardial infarction.
ON THE MECHANISM OF RENIN STIMULATION
Authors: Nielsen Ingolf , Møller Inge
Published: August 2010

Abstract The correlation between hemoconcentration (expressed by increase in colloid osmotic pressure (ΔCOP)) and plasma renin activity increase (ΔPRA) has been investigated during 1) change of posture from supine to standing position in normals, who were salt‐depleted the three previous days (10 mEq Na+ per day, on the third day 100 mg chlortalidone (Hygrotone®)); and 2) in exercise experiments on normals on liberal salt intake in sitting position on bicycle ergometer (medium work load‐terminal pulse rate 120–140). The correlation ΔCOP‐ΔPRA was in 1) linear and significant, r =0.83, p < 0.001. The slope of the line was significantly steeper than in corresponding experiments on liberal salt intake (PRA increase per unit COP increase was greater). Mean supine PRA was significantly higher than on liberal salt intake, but the range overlapped. In 2) a significant PRA increase in all experiments appeared, but no correlation ΔCOP‐ΔPRA. It is concluded that salt depletion of some days' duration (chronic) sensibilizes the renin releasing mechanism for the postural stimulus. The lack of correlation ΔCOP‐ΔPRA for the exercise studies suggests a mechanism for renin stimulation different from the one in postural shift and acute salt depletion.
MECHANICAL HEMOLYSIS IN AORTIC VALVULAR DISEASE AND AORTIC BALL‐VALVE PROSTHESIS
Authors: Myhre Erik , Rasmussen Knut
Published: August 2010

AbstractUsing serum haptoglobin concentration as an index, the frequency of hemolysis has been determined in 110 consecutive, non‐selected patients with aortic valvular lesions admitted to Medical Department B, Rikshospitalet, during one year from February 1968. Marked lowering or absence of serum haptoglobin was seen in 11 of 65 patients with aortic valvular disease, hemolysis being about twice as frequent in aortic insufficiency as in aortic stenosis. Values indicating hemolysis were observed in 37 of 45 patients with a Starr‐Edwards aortic ball‐valve prosthesis. In most cases hemolysis was slight; no case with clinically important hemolytic anemia was seen. Marked lowering of the serum haptoglobin concentration seems to be the most sensitive qualitative indicator of hemolysis, the serum lactic dehydrogenase activity probably giving additional information about the rate of hemolysis.
A CASE OF TRANSIENT DIABETES MELLITUS IN CONNECTION WITH ACUTE PANCREATITIS
Authors: Nielsen Ole Saxtrup , Simonsen Erik
Published: August 2010

Abstract. A case of diabetes mellitus which developed in a male aged 40 years in association with acute pancreatitis is reported. The patient was admitted with a blood sugar of 1028 mg%. The diabetes required treatment with insulin but the patient recovered completely after 4 1/2 months. The literature available is reviewed.
ANOREXIA NERVOSA, SECONDARY ALDOSTERONISM AND ANGIOPATHY
Authors: Pasternack A.
Published: August 2010

Abstract.A female patient, 29 years old, is presented. She exhibited an abnormal personality leading to anorexia nervosa, diarrhoea of the ulcerative colitis type, surreptitious vomiting, severe hypokalemia and secondary hyperaldosteronism. The occurrence of severe arterial and arteriolar thickening in conjunction with normal blood pressure and high blood renin activity is stressed.
PLASMA‐INSULIN AFTER TOLBUTAMIDE IN DIABETICS AND NON‐DIABETICS
Authors: Deckert Torsten , Mogensen Peter
Published: August 2010

Abstract.Following intravenous injection of 1.0 g tolbutamide, the serum insulin and glucose levels have been determined in 27 normal subjects and 11 patients with stable diabetes mellitus, all non‐obese. All the normal subjects showed an abrupt increase in the concentration of serum insulin, reaching a peak 2 min after the injection. Young persons gave a greater insulin response than elderly, but the difference was not significant. Patients with stable diabetes gave very little insulin response, but in most cases the maximum insulin concentration was also attained in 2 min. This finding, viz. that in diabetics the increase in the insulin concentration following tolbutamide stimulation occurs immediately, while after an i.v. glucose stimulation it is delayed, indicates that the target of tolbutamide in the islet tissue of the beta cells differs from that of glucose.
EFFECT OF INTRAVENOUS PROSTAGLANDIN E1ON NORADRENALINE‐STIMULATED MOBILIZATION OF PLASMA FREE FATTY ACIDS IN MAN
Authors: Carlson Lars A. , Ekelund Lars‐Göran , Orö Lars
Published: August 2010

Abstract.Prostaglandin E1(PGE1) has been given intravenously during continuous noradrenaline infusion into healthy male subjects. Control experiments were also performed in which saline was given instead of PGE1. There were four subjects in each group. Noradrenaline was given at a rate of 0.1 μg/kg/min i.v. during 210 min. When noradrenaline had been given for 80 min, PGE1was infused simultaneously in three doses, each during 30 min, the dose being successively increased from 0.10 μg/kg/min to 0.18 and finally to 0.32 μg/kg/min. The PGE1infusion induced a flush in all subjects. Three of the four subjects given PGE1had headache and two abdominal cramps. In one of the subjects the PGE1infusion with the highest dose, 0.32 μg/kg/min, had to be stopped after 25 min because of respiratory distress. The noradrenaline infusion increased the blood pressure with a slight decrease in heart rate. When PGE1was infused simultaneously, the blood pressure decreased and the heart rate increased. The concentration of free fatty acids (FFA) in blood plasma increased markedly during the noradrenaline infusion. This rise in FFA concentration was not significantly influenced by the simultaneous administration of PGE1. The fact that no inhibition of FFA mobilization could be demonstrated does not rule out a physiological effect of PGE1in this regard, but suggests that PGE1is then not transported by the blood to adipose tissue.
EXPERIENCES OF A CONTOUROGRAPH IN ECG‐MONITORING AND ARRHYTHMIA DIAGNOSIS1
Authors: Hulting J. , Matell G. , Wigertz O.
Published: August 2010

Abstract. An electrocardiocontourograph has been constructed and tested in a medical intensive care unit on patients with different types of ECG disturbances. The contourograph employs the contourogram principle for the presentation of ECG data. The ECG signal is recorded on a cathode‐ray storage screen linewise, with one or more complexes per line, starting at the left lower edge of the screen. A slight positive base line shift is added for each line. A new method for “intensity modulation” of the oscilloscope beam may reinforce the contours of successive R waves. The contourogram shows characteristic patterns in arrhythmias, heart frequency variations or QRS abnormalities. The presentation time was extended by up to 6 min by increasing the number of lines (= reduced spacing between the lines) and/or by reducing the sweep rate. When the recording has covered the entire screen the beam returns to the starting point where the screen, due to continuous erasing, is ready for a continuing recording. The contourographic patterns were examined in some 60 cases with various disturbances of ECG activity. ECG contourography revealed various forms of premature beats and frequency variations and facilitated the diagnosis of, e.g., disturbances of the A‐V conduction. In rhythm disturbances, which developed slowly, changes and trends to changes in the heart rate were easily detectable.
COMPARISON OF THE MAIN LIPIDS IN PLATELETS AND PLASMA IN MAN
Authors: Nordøy Arne , Bjørge Johanne Margrethe , Strøm Ellen
Published: August 2010

Abstract The concentration of total cholesterol, total phospholipids, triglycerides (TG) and free fatty acids (FFA) in plasma and platelets has been evaluated in 11 healthy male subjects. The distribution of the different fatty acids in FFA and TG from the two compartments was measured. All subjects had plasma lipid concentrations within the normal limits. No significant correlation could be established between the concentrations of any of the main lipid fractions in plasma and platelets. A highly significant correlation was found between FFA stearic acid in plasma and platelets, and a correlation was also established for palmitoleic and oleic acids, whereas a non‐significant correlation was found for FFA palmitic acid. No correlation could be established for FFA polyunsaturated fatty acids or for triglyceride fatty acids.
SERUM DIGOXIN VALUES FOLLOWING A DOSAGE REGIMEN BASED ON BODY WEIGHT, SEX, AGE AND RENAL FUNCTION
Authors: Christiansen N. J. B. , Kølendorf K. , Siersbæk‐Nielsen K. , Hansen J. Mølholm
Published: August 2010

Abstract. Serum digoxin has been determined by a radio‐immunological method in 99 patients treated with digoxin. Digoxin was administered using a dosage regimen with a daily maintenance dose equivalent to (14 +creatinine clearance/5) per cent of loading dose. Three loading doses were used: 0.01 mg/kg b.wt., 0.015 mg/kg and 0.02 mg/kg, respectively. Creatinine clearance was calculated from a rapid bedside evaluation based on a nomogram. This nomogram requires a knowledge merely of the age of the patient as well as sex, b.wt. and serum creatinine. The serum digoxin mean values were 1.0 ± 0.3 ng/ml, 1.1 ± 0.5 ng/ml and 1.4 ± 0.6 ng/ml in the three groups of patients with maintenance doses corresponding to the three loading doses. The frequency of digoxin intoxication in the three groups was found to be 0, 4 and 8 %. The dosage regimen described seems to be a valuable aid to digoxin therapy.
RED CELL 2,3‐DIPHOSPHOGLYCERATE IN OBESE PATIENTS
Authors: Monti M.
Published: August 2010

Abstract. Erythrocyte 2,3‐DPG has been found to be significantly increased in a group of 22 obese patients. This is interpreted as the manifestation of a compensatory mechanism, secondary to the respiratory distress, promoting oxygen release from the red blood cells through decreased Hb affinity for oxygen.
Hypertension in End‐stage Renal Disease
Authors: Kornerup H. J.
Published: August 2010

ABSTRACT. Blood pressure (BP), plasma renin concentration (PRC), plasma renin substrate concentration (PRSC) and exchangeable sodium (ES) have been studied in 27 patients undergoing regular hemodialysis because of end‐stage renal disease. PRC was significantly higher in the hypertensive than in the normotensive patients. The pattern of PRSC was similar in the groups of patients but with a marked individual variation. ES was slightly lower in the hypertensives than in the normotensives but the difference was not statistically significant. Multiple regression analysis demonstrated a significant correlation between mean BP, the natural logarithm of PRC and ES, but the effect of ES was negligible. PRC was negatively correlated to ES in all patients, including the hypertensives. These results strongly suggest that the renin‐angiotensin system is the most important factor involved in the pathogenesis of hypertension in end‐stage renal disease, when sodium balance is adequately controlled. A clinical application of the predictive value of PRC concerning the effect of bilateral nephrectomy on hypertension is outlined.
Problems Encountered in Long‐term Treatment with Anticoagulants
Authors: Husted S. , Andreasen F.
Published: August 2010

ABSTRACT.The course of long‐term anticoagulant therapy in 114 out‐patients has been evaluated over a three‐month period. The evaluation was based on the registration of information from the clinical records and from two personal interviews with each patient. The patients had attended the Anticoagulation Clinic for 141 weeks on the average. The prothrombin complex activity (PP%) level was significantly lower in patients with bleeding episodes. An apparently higher PP% level in patients with thromboembolic manifestations was not significant. No bleeding was observed when the PP% was above 25. Warfarin resulted in relatively fewer bleeding episodes and more PP% values within the desired range (10–25) than phenprocoumon and bishydroxycoumarin. The role of age, but not of moderate hypertension, as a risk factor was confirmed. A probable adverse interaction of the anticoagulant and other drugs was found in 37.5 % of the situations in which an interaction could be expected according to the literature.
Hereditary Angioneurotic Oedema in Finland
Authors: Ohela Kyllikki
Published: August 2010

ABSTRACTA total of 7 families with hereditary angioneurotic oedema (HANE) have been found in Finland. Six HANE patients have died from laryngeal oedema, 27 patients with diagnosed HANE are alive and 21 members have a haematological abnormality typical of HANE, i.e. a deficiency of the inhibitor of the activated first component of complement (C1‐INH), but no manifest symptoms. The largest family has 363 living members, 303 of whom were investigated for C1‐INH, C4 and C3. Fourteen patients had HANE, 18 relatives were symptomless but had C1‐INH deficiency, and 3 members of the family had died from laryngeal oedema. In two families only one case of HANE was diagnosed, the parents in both cases being symptomless with normal C1‐INH levels. All patients who had died from laryngeal oedema and 10 of the 27 HANE patients still alive had a typical triad of paroxysmal abdominal pain, peripheral oedema and laryngeal oedema. Six patients have had abdominal attacks alone, three peripheral oedema alone and two only laryngeal oedema. The age at onset of symptoms was 1–51 years. Three patients, who have received continuous methyltestosterone therapy, had hardly any symptoms during the treatment. Thirteen patients have received tranexamic acid, either during an attack or continuously, with positive effects in all except two. Cinnarizine treatment was beneficial in three out of four cases, both when given continuously or during an attack.