Diprosopus with associated malformations: report of two cases

We report on two patients with craniofacial duplication and anencephaly. In addition to these anomalies, one patient showed vertebral fusion and diaphragmatic hernia, and the other one presented cheilognathopalatoschisis.     

Aberrant twinning (diprosopus) associated with anencephaly

A case of Monocephalus diprosopus, associated with craniorachischisis and duplication of most of the foregut derivates is presented. The major part of the cardiovascular system remained single but the heart exhibited severe defects, including a complete persistent atrioventricular canal, transposition of the great arteries and atresia of the pulmonary valve. This report further supports the hypothesis that certain types of incomplete twinning and neural tube defects may be caused by a single teratogenic mechanism.     

Post-transplantation pancreatitis associated with cytomegalovirus (report of a case)

Cytomegalovirus inclusions were identified at autopsy in the pancreas and skin of a patient with acute pancreatitis and herpes zoster who had had a renal transplant. No inclusions were found in any of the other organs examined. Etiologic implications are discussed.     

Gilles de la Tourette's syndrome associated with head injury: a case report.

A case of Gilles de la Tourette''s syndrome in a Kuwaiti woman is described, and the possibility of antecedent head injury as the organic etiological factor is discussed.     

Absence of the musculocutaneous nerve associated with a supernumerary head of biceps brachii: a case report

Unusual neural and muscular variations in the right upper limb were noted during anatomical dissection of a 54-year-old male cadaver. The absence of the musculocutaneous nerve was correlated with a three-headed biceps brachii muscle. The distribution of muscular branches innervating the anterior compartment of the muscles of the arm was different to the cases described in literature hitherto. This report contains a comparison of various instances where the musculocutaneous nerve is absent. We discuss the embryological explanation for this anomaly and its clinical results. It is important to be aware of such possible anatomical variations during clinical practice.     

Osteosarcoma associated with acromegaly: a case report.

A case of acromegaly who developed an osteosarcoma is recorded, with persistently raised levels of growth hormone. It is possible that there was some aetiological relationship between the increased rate of bone formation and the development of the osteosarcoma.     

Iniencephaly with cyclopis (a case report)

Iniencephaly is a rare neural tube defect. We report a rare association of iniencephaly with cyclopia, probably the third such report in the literature.     

Waldenstrom's macroglobulinemia associated with Hodgkin's lymphoma: a case report

Waldenstrom''s macroglobulinemia/lymphoplasmacytic lymphoma (WM/LPL) is a low-grade B-cell non-Hodgkin''s lymphoma with an indolent clinical course. Higher-grade non-Hodgkin lymphoma (NHL) and therapy-related myelodysplasia/acute leukemia (t-MDS/AML) have been reported in patients with WM/LPL in previous studies. However, only two cases with WM/LPL were reported to develop to Hodgkin lymphoma (HL). Here, we report the first case of WM/LPL who developed classical HL simultaneously 3 years after initial nucleoside analog-based chemotherapy.     

AA-type amyloidosis associated with non-Hodgkin's lymphoma: a case report

Amyloid-associated protein (AA)-type systemic amyloidosis has been referred to as secondary amyloidosis because it is secondary to an associated inflammatory condition. It is extremely rare in patients with non-Hodgkin's lymphoma (NHL). Here we report an autopsy case of follicular small cleaved cell lymphoma with focal large B-cell lymphoma transformation in association with systemic AA-type amyloidosis. Formalin-fixed, paraffin-embedded tissues from autopsy and the patient's previous surgical specimen were studied by Congo red stain; electron microscopy; and immunostaining with antibodies against AA protein, P component, and kappa and lambda light chains. There was a marked AA amyloid deposition in the glomeruli of both kidneys, the retroperitoneal lymphoma mass, the blood vessels, the adrenal glands, and the adipose tissues. The patient's previous surgical specimens were negative for amyloid. We propose that this patient's systemic AA-type amyloidosis developed along the course of his NHL.     

Darier disease associated with HIV infection: a case report

AIDS is characterized with immune dysregulation and depletion of CD4+ T cells and increased vulnerability to different pathological conditions, including many dermatoses. The present report describes features of Darier disease (DD) in an HIV-positive patient. The subject of this report was a 45-year old Ghanaian man referred to the dermatology unit of the Iranian Red Crescent Society in Accra, Ghana. He presented with numerous follicular keratotic red-brown papules on the scalp, face, retroauricular regions, ears, sternum, upper trunk, hands, axilla, and inguinal regions. The lesions first appeared on the dorsal surface of the hands and face and subsequently appeared on the other parts of the body including the outline of the scalp. This is the first report of DD in the setting of HIV infection. Significant features of this case associated with HIV in comparison with non-HIV cases are negative family history of DD, late onset, and wide distribution of the disease without abnormality on the nails and mucous membranes beginning a few years after involvement with HIV virus.     

Favre-Racouchot syndrome associated with eyelid papilloma:a case report

A 55-year-old Chinese man presented with an asymptomatic pedunculated elevation on his left lower eyelid which had been gradually increasing in size during the past three years.The patient was diagnosed with eyelid papilloma by pathological examination.Concomitantly,the patient developed open comedones with a bilateral linear distribution,along with oblique wrinkle lines in his infraorbital regions.These lesions were noninflammatory and remained unchanged for two years.To the best of our knowledge,this distribution of open comedones,especially in combination with eyelid papilloma,has not been reported previously in Favre-Racouchot syndrome.     

Acquired hemophilia associated with bullous pemphigoid: a case report

The development of factor VIII inhibitors in non-hemophilic patients is rare and may occur in healthy individuals, mostly elderly and women in postpartum period, and in patients with malignant neoplasia or autoimmune diseases, such as bullous pemphigoid. We described the case of a 60-year-old female patient who developed bullous pemphigoid for 3 month and presented with bleeding tendency and hematoma in the tongue. Therapy with methylprednisolone, cyclophosphamide, intravenous immunoglobulin and factor VIII reposition was instituted, resulting in a remission of the bleeding and negativity for antibodies against factor VIII titers. We concluded that, despite its rarity, the presence of acquired factor VIII inhibitors should be investigated when patients with autoimmune diseases develop bleeding manifestations.     

Acute promyelocytic leukemia associated with insulinoma: a case report

We report a case of acute promyelocytic leukemia (APL) coexisting with insulinoma in a 61-year-old female. Two years before the onset of APL, she was diagnosed as having insulinoma and underwent resection of the body and tail of the pancreas, but no insulinoma was found in the resected pancreas. The clinical symptoms of hyperinsulinism, however, continued after the operation and were treated with diazoxide (100 mg daily, 37 g in total) for about two years until the onset of APL. During the induction therapy for APL, she died of hemorrhage and infection; autopsy revealed the presence of insulinoma (13 X 9 mm in size) in the residual pancreas head. This is the first case report of coexistence of APL and insulinoma.