B. Ilie

Dorfman-Chanarin syndrome: A case report and a review

Dorfman-Chanarin syndrome in two sisters of Jewish Iraqi origin is reported. This heritable disorder of the metabolism of neutral lipids was manifested by congenital ichthyosis, vacuoles in the leukocytes, and variable involvement of liver, muscle, central nervous system, and the auditory system. In two asymptomatic members of the family leukocyte vacuoles were found as the only sign of the syndrome. Clinical, pathologic, ultrastructural, and biochemical findings are described. Previous reported cases are reviewed.

Dorfman-Chanarin syndrome: morphologic studies and presentation of new cases.

Dorfman-Chanarin syndrome, or neutral lipid storage disease with ichthyosis, is a rare inherited metabolic disorder characterized by accumulation of neutral lipids in different tissues. Variability in dermatologic severity is not understood. We report two new cases, compare their features with other reported cases, and examine the possible relationship between the severity of the dermatologic condition and lipid accumulation in various types of skin cells. The localization of lipid droplets was determined in various dermal and epidermal cells by light microscopy of epoxy resin semithin sections and electron microscopy in four cases of Dorfman-Chanarin syndrome. Lipid droplets in the dermis were detected in fibroblasts, Schwann cells in both myelinated and unmyelinated nerves, smooth muscle cells, and sweat gland cells, but not in endothelial cells of blood capillaries. The droplets were not surrounded by a membrane. Findings indicate that there is no correlation between the clinical severity of the disease and the microscopic findings.

Kaposi’s Sarcoma after Steroid Therapy for Pemphigus foliaceus

Rapid evolution of Kaposi’s sarcoma terminating in death, appearing in a pemphigus patient treated by corticosteroids, is described and the literature is reviewed.

Propylthiouracil-induced cholestatic jaundice

The typical propylthiouracil (PTU)-linked hepatotoxicity, is known to manifest itself by hepatocellular injury with greatly increased serum transaminase values and evidence of hepatic necrosis on liver biopsy. Herewith presented is a 33-year old, thyrotoxic woman who developed cholestatic jaundice two weeks after initiation of PTU therapy. The diagnosis was confirmed by liver biopsy. A causal link between PTU treatment and the cholestatic jaundice was suggested by: the time of onset, typical skin rash and a positive migration inhibition factor (MIF) test to PTU. Awareness of this rare complication of PTU treatment may prevent the performance of unnecessary, expensive and possibly harmful diagnostic procedures.

Multiple Telangiectases Preceding the Appearance of Undifferentiated Bronchogenic Carcinoma

A patient with undifferentiated bronchogenic carcinoma, which was preceded by skin manifestations, namely telangiectases on the palms, soles, fingers, toes, lips and tongue, is described. It is possible that these multiple vascular lesions are one of the cutaneous markers of internal malignancy.

Psoriasis of the Lips: The Unusual Köbner Phenomenon Caused by Protruding Upper Teeth

Psoriasis of the lips was observed in a 54-year-old female who had suffered from psoriasis vulgaris for 18 years. The psoriasis of the lips was preceded by protrusion of the upper teeth. Topical corti

Spinal epidural compression in chronic lymphocytic leukemia

Spinal epidural compression is a rare neurologic complication in patients with lymphoma. It occurs mostly in those with intermediate‐grade to high‐grade malignancy disease. This type of neurologic involvement has not been described in chronic lymphocytic leukemia (CLL). A patient with a long, stable CLL course developed spinal epidural compression and consequently died. The frequency of spinal epidural compression in lymphoma, according to the histologic subtypes and the considerations in making the right choice of therapy are discussed in light of the presented case.

Jadassohn-Type Anetoderma in Association with Protrusion of the Teeth

A case of anetoderma is described in which progressive protrusion of the anterior teeth was a concomitant finding. A possible interrelationship between these two conditions is suggested, based upon the anatomy and physiology of the oxytalan fibers of the periodontal ligament.

Perforating Cutaneous Mucinosis

A case is described in which a papulonodular eruption developed all over the body. Histologic examination of one of the lesions revealed the dermis to contain mucin (hyaluronic acid). In some areas there was perforation through the epidermis of the mucinous material.