Journal of IMAB - Annual Proceeding (Scientific Papers)
, Gospodin Iliev
ISSN: 1312 773X
Issue: 2007, vol. 13, book 1
Subject Collection: Medicine
Online date: November 14, 2007
J of IMAB 2007; 13(1):60-62
FAMILIAL BENIGN CHRONIC PEMPHIGUS (HAILEY-HAILEY DISEASE)
Ivelina Yordanova, Dimitar K. Gospodinov, Valentina Chavdarova, Savelina Popovska*
Department of Dermatology and Venereology, *Department of General and Clinical Pathology, Medical University - Pleven, Bulgaria
Familial benign chronic pemphigus (Hailey–Hailey disease) is a blistering dermatosis, which is inherited as an autosomal dominant trait and usually presents around the third and fourth decades. Painful erosions, vesico-pustules and scaly erythematous plaques appear at sites of friction such as the sides of the neck, the axillae, the groins and the perineum. A case of familial benign chronic pemphigus in a 54 year-old woman is presented. The disease started at the age of 35. The areas of predilection are the axillary and submammary folds and genital area. Erythematous, macerated plaques were found with multiple painful fissures, vesicles and crusts in its periphery. The diagnosis was established on the basis of physical examination and was confirmed by histological examination of a skin biopsy. A suprabasal cell separation (acantholysis) of the epidermis was found. A family history is present. An autosomal dominant mode of inheritance was confirmed by genealogical analysis. Thirteen members have been affected by the same disease. In the presented case topical and systemic antibiotics, antimycotics, corticosteroids and retinoids were applied with a transient result. The patient was treated with topical Pimecrolimus which showed a good result. Postlesional hyperpigmentations were found after this treatment.
Key words: Familial benign chronic pemphigus, Hailey–Hailey disease, acantholysis, Pimecrolimus.
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Please cite this article as:
Yordanova I, Gospodinov DK, Chavdarova V, Popovska S. Familial benign chronic pemphigus (Hailey-Hailey disease). J of IMAB. 2007; 13(1):60-62. doi: 10.5272/jimab.2007131.56
1. Jemal A, Tiwari RC, Murray T et all, Cancer Statistics, 2004. CA Cancer J Clin 2004;54:8–29.
2. Glimelius B, Hofman K, Graf W at all, Quality of life during chemotherapy in patients with symptomatic advanced colorectal cancer. The Nordic Gastrointestinal Tumour Adjuvant Therapy Group. Cancer 1994; 73: 556–562.
3. Machover D. A comprehensive review of 5-fluorouracil and leucovorin in patients with metastatic colorectal cancer. Cancer 1997; 80: 1179–1187.
4. Meta-analysis Group in Cancer. Efficacy of IV continuous infusion of fluorouracil compared with bolus administration in advanced colorectal cancer. J Clin Oncol 1998; 16: 301–308.
5. Pommier Y, Tanizava A, Kohn KW. Mechanisms of topoisomerase I inhibition by anticancer drugs. In: Liu LF, ed. Advanced in pharmacology. New York: Academic Press 1994; 29B; 73-92
6. Saltz LB, Cox JV, Blanke C et al. Randomized trial of irinotecan versus fluorouracil by continuous infusion after fluorouracil failure in patients with metastatic colorectal cancer. Lancet 1998; 352: 1407-1412
7. Douillard JY, Cunningham D, Roth AD et al. Irinotecan combined with fluorouracil compared with fluorouracil alone as first- line treatment for metastatic colorectal cancer: a multicentre randomized trial. Lancet 2000; 355: 1371
8. Miller AB, Hoogstraten B, Staquet M et al. Reporting results of cancer treatment. Cancer 1981; 47; 207-214
9. Brimdage MD, Pater JL, Zee B: Assessing the reliability of two toxicity scales: Implications for interpreting toxicity data. J Natl Cancer Inst 1993; 85; 38-48
10. Kaplan EL, Meyer P: Non-parametric estimation from incomplete observations. J Am Stat Assoc 1959; 53; 457- 481
11. Tournigand C, Andre T, Achille E et al. FOLFIRI followed by FOLFOX6 or the reverse sequence in advanced colorectal cancer: a randomized GERCOR study. J Clin Oncol 2004; 22; 229-237
12. Goldberg RM, Sargent DJ, Morton RF et all: A randomized controlled trial of fluorouracil plus leucovorin, irinotecan, and oxalplatin combinations in patients with previously untreated metastatic colorectal cancer. J Clin Oncol 2004; 22; 23-30
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