Successful treatment of oral linear IgA disease using mycophenolate.

Linear IgA disease (LAD) is a rare acquired autoimmune bullous disorder, characterized by linear deposition of IgA along the dermoepidermal basement membrane zone. The clinical presentation of LAD consists of vesiculobullous lesions affecting the skin and mucosal surfaces. The present case report presents a rare presentation of this vesiculobullous disorder. Although more than 50% of LAD patients present with oral lesions, there are few reported cases of involvement of the mouth as the sole manifestation. A 79-year-old female presented with a sore mouth and erosions affecting the palate. The symptoms resolved following the provision of mycophenolate, an antiproliferative immunosuppressant which has not previously appeared to have been reported in the long-term successful management of linear IgA disease limited to the mouth. We found that mycophenolate is a useful adjunct to the successful treatment of oral linear IgA when the uses of other immunosuppressants are contraindicated.

Diagnostic surgical pathology: the importance of second opinion in a developing country.

OBJECTIVE: To review the cases sent to the section of histopathology, Aga Khan Universty (AKU) for second opinion and see whether there are significant differences in the original outside diagnosis and the subsequent diagnosis submitted by us. METHODS: A retrospective study of all consecutive cases for second opinion in the form of paraffin blocks from 1st Novemver 2001 to 31st July 2002. The primary submitted diagnosis in each case was compared with the subsequent AKU diagnosis. RESULTS: The study included a total of 381 cases. However, in 45 cases (11.81%), initial histopathological diagnosis was not provided. Out of the remaining 336 cases, there were differences between the original diagnosis and the subsequent AKU diagnosis in 120 cases (35.71%). Out of these 120 cases, immunohistochemistry was performed in 65 cases (54.16%) only. CONCLUSION: In a developing country like Pakistan, where few laboratories are equipped to function as modern histopathology units, second opinion on difficult cases is very important. Worldwide, the concept of second opinion in surgical pathology is well established.

Spectrum of cutaneous appendage tumors at Aga Khan University Hospital.

OBJECTIVE: To determine the frequency of various types of cutaneous appendage tumors in our practice. METHOD: This is a partly retrospective and partly prospective study conducted at the Department of Pathology, Histopathology Section, The Aga Khan University Hospital, Karachi between 1st January 1997 and 31st December 2001. RESULTS: One hundred sixty six skin appendage tumors were diagnosed during the study period. 87.3% were benign, while 12.6% were malignant. Male female ratio was almost equal. Mean age was 41.72 years. 37.34% showed eccrine differentiation, 14.45% showed apocrine differentiation and 41.56% showed pilosebaceous differentiation, 6.62% exhibited mixed differentiation. The 5 commonest tumors were pilomatricoma, nodular hidradenoma (eccrine acrospiroma), syringocystadenoma papilleferum, eccrine poroma and eccrine spiradenoma. The commonest malignant tumors were porocarcinoma and sebaceous carcinoma. Pilomatricoma were common in children. CONCLUSION: Most of our findings roughly correlate with the western published data. However, commonest site for eccrine poromas in our study was head and neck. Also, not a single case of eccrine spiradenoma was seen in the first two decades of life. These findings differ significantly from western data.