Katsumi Ogata

CD56: a useful marker for diagnosing Merkel cell carcinoma

BACKGROUND Merkel cell carcinoma (MCC) of the skin is an aggressive but rare malignant neuroendocrine tumor. For its pathological diagnosis, we use a panel of immunohistochemical markers, such as cytokeratin 20 (CK 20), epithelial membrane antigen (EMA), chromogranin A, neuron specific enolase (NSE), synaptophysin, and Leu7 (CD57) to demonstrate its epithelial and neuroendocrine features. CD56, or neural cell adhesion molecule (NCAM), has been demonstrated recently as the tumor marker of the pulmonary neuroendocrine cell system. Its expression in MCC, however, has still rarely been investigated. Furthermore, in such very few previous studies on NCAM expression in MCC, all the tumor cells were not necessarily demonstrated to express NCAM. OBJECTIVES To study the immunoreactivity of CD56 in MCC, especially using a monoclonal antibody of a clone 1B6, different from those adopted in the previous reports. METHODS We reexamined CD56 expression immunohistochemically in five MCC cases, along with the conventional panel of markers described above, using paraffin-embedded tissue sections. RESULTS CD56 revealed the most diffuse and intense positive staining, which was noted along the cell borders, in all specimens compared with other neuroendocrine tumor markers. CONCLUSIONS The results of our study indicate that CD56, especially a new monoclonal antibody (clone 1B6), is a useful immunohistochemical marker for MCC.

New entity, definition and diagnostic criteria of cutaneous adult T-cell leukemia/lymphoma : Human T-lymphotropic virus type 1 proviral DNA load can distinguish between cutaneous and smoldering types

Adult T‐cell leukemia/lymphoma (ATLL) has been divided into four subtypes up to now: (i) acute; (ii) lymphoma; (iii) chronic; and (iv) smoldering. Skin lesion(s) may be present and the cases showing less than 5% abnormal T‐lymphocytes in peripheral blood without involvement of other organs, have been classified as smoldering ATLL. However, this type of ATLL with skin manifestations had a worse prognosis than that without skin lesions. This study aimed to define and distinguish cutaneous ATLL lacking nodal lymphoma and leukemic change from smoldering ATLL. We propose an entity of cutaneous ATLL, which has less than 5% abnormal T lymphocyte in peripheral blood, a normal lymphocyte count (i.e. <4 × 109/L), no hypercalcemia and lactate dehydrogenase values of up to 1.5 times the normal upper limit. At least one of the histologically proven skin lesions should be present accompanying monoclonal integration of human T‐cell lymphotropic virus type 1 (HTLV‐1) proviral DNA in the skin lesion. Blood samples were collected from 41 HTLV‐1‐infected patients, 21 asymptomatic carriers, 16 patients with cutaneous ATLL and four patients with smoldering ATLL. HTLV‐1 proviral loads, soluble interleukin‐2 receptors and other parameters were examined in each case. HTLV‐1 proviral DNA loads in smoldering ATLL group are significantly higher than those in asymptomatic carrier and cutaneous ATLL group. Cutaneous ATLL may be a distinct entity that should be separated from smoldering ATLL clinically and virologically.

Primary cutaneous Langerhans cell histiocytosis showing malignant phenotype in an elderly woman: report of a fatal case

Background: Langerhans cell histiocytosis (LCH) is a proliferating disorder of Langerhans cells (LC) that are characterized by the presence of Birbeck granules. LCH has been considered to be a disease of childhood and there have been limited cases of adult LCH. We report here a fatal case of histiocytic tumor showing Langerhans cell phenotype, arising in the skin of a 74‐year‐old woman.

CONGENITAL ONYCHODYSPLASIA OF THE INDEX FINGERS: A POSSIBLE EXPLANATION OF RADIALLY-PRONOUNCED INVOLVEMENT OF THE NAIL IN THIS DISORDER

Two cases of congenital onychodysplasia of the index fingers (COIF), one of them with an abnormal range of active motion of the digits, were reported. The characteristic location of a nail, or nails in micronychia and polyonychia of COIF and the involvement of the nail and the bone may be explained by the anatomy of the digit. That is, the radially more pronounced involvement of the nail may be related to the relatively smaller size of the artery of the radial aspect. This may support the transient ischemia theory of Ohta and Haseda (1) in fetal life, though the true etiology of COIF still remains obscure.

Aeromonas sobria infection with severe soft tissue damage and segmental necrotizing gastroenteritis in a patient with alcoholic liver cirrhosis

A 49‐year‐old man, who had a 3‐year history of liver dysfunction but had not been treated, was admitted to the hospital with a sudden onset of fever and generalized muscle pain. He subsequently developed generalized purpura with scattered hemorrhagic bullae of the skin and massive bloody stools. Aeromonas sobria was proven by culture of both blood and bullous fluid. In spite of the extensive treatment with antibiotics and other medications in the intensive care unit (ICU), the patient went into septic shock and died 2 days after admission. Pathological examination on autopsy revealed segmental necrotizing gastroenteritis with bacterial colonies and alcoholic liver cirrhosis, in addition to extensive severe soft tissue damage involving cellulitis and rhabdomyolysis and epidermolysis. Although the prognosis for Vibrio vulnificus infection with severe soft tissue damage in patients with liver cirrhosis, malignancy, diabetes mellitus or other pre‐existing diseases is poor, the unfavorable progression of Aeromonas species, especially A. sobria infection is rare. This is thought to be the first report of an autopsied case.

Vertically growing ectopic nail Nature's experiment on nail growth direction

The cul-de-sac in which the nail is formed determines that it should grow outward instead of upward according to Kligman. This view was questioned by Baran based on clinical observations and review of the literature. A case of vertically growing ectopic nail of congenital origin indicated that the cul-de-sac was directed to the skin surface and the nail was growing vertically to the skin. The anatomy of the cul-de-sac, which may be influenced by local factors, appears to determine the direction of the nail.

A Case of Cryoglobulinemic Gangrene in Myeloma with Fatal Outcome Despite Successful Skin Grafting

Extensive necrosis of the skin, muscle and bone was produced after prolonged cooling in a case of myeloma with cryoglobulinemia. Skin grafting was beneficial.

Two sisters with guttate psoriasis responsive to tonsillectomy: case reports with HLA studies.

An 11‐year‐old girl and her 7‐year‐old sister, both with guttate psoriasis, both showed chronic tonsillitis and proteinuria. The ASO titer of the elder sister was 480 units and that of the younger sister 320 units. Tonsillectomies were performed on both sisters because they developed guttate psoriasis after colds with severe sore throats and proteinuria. Skin lesions improved markedly and proteinuria disappeared, but ASO titers did not change significantly. HLA studies were made of the sisters and their parents; A1 and B37 were confirmed in both sisters and their mother.

Bilateral lichen striatus.

We describe a very rare case of bilateral lichen striatus on the lower extremities with a history of more than ten years. Histopathologically, the lesions demonstrated a lichenoid tissue reaction with foci of spongiosis and perivascular inflammatory cell infiltration. In addition, the finding of lymphocytic infiltrations around the eccrine duct was observed. They were treated successfully with topical application of corticosteroid ointment. To the best of our knowledge, no other lichen striatus case has been reported with bilateral distribution and such long‐term persistence.

Isolation of Multiple Cytomegalovirus Strains from a Patient with Adult T Cell Leukemia

A 66-year-old male with adult T cell leukemia had an ulcer on the left medial thigh. The biopsy of the skin lesion revealed enlarged endothelial cells with acidophilic intranuclear inclusion bodies, suggesting cytomegalovirus (CMV) infection. At autopsy, CMV was isolated from a nodular skin lesion of the scrotum. The urine constantly tested positive for CMV. Restriction endonuclease cleavage analysis of DNA of the isolates from the skin and urine indicated that this patient was infected with two different strains of CMV.