Case 7 -

IPEX Syndrome and Pemphigoid Nodularis James W. Patterson University of Virginia Health Sciences Center Charlottesville, VA

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Clinical History 14 year old boy with a history of chronic diarrhea, sinopulmonary infections, and asthma. He had high titers to islet cell and thyroid microsomal antigens. Cutaneous findings in the past had included eczematous dermatitis. He recently developed prurigo nodularis-like lesions on abdominal skin and on the extremities. A biopsy was taken of a nodular lesion from the right forearm Case 7 - Figure 1 - This view shows pseudoepitheliomatous hyperplasia and a mixed dermal infiltrate. Subtle subepidermal clefting can be seen. There is evidence for "vertical streaking" of papillary dermal collagen. Ulceration was apparent on low power magnification. Case 7 - Figure 2 - On high power magnification, numerous eosinophils can be seen in the dermal infiltrate. Click here to download a PDF of this handout. References Cliff S, Holden CA. Pemphigoid nodularis: a report of three cases and review of the literature. Br J Dermatol 1997 ;136(3):398-401. Nieves DS, Phipps RP, Pollock SJ, Ochs HD, Zhu Q, Scott GA, et al. Dermatologic and immunologic findings in the immune dysregulation, polyendocrinopathy, enteropathy, X-linked syndrome. Arch Dermatol 2004;140(4):466-472. Wildin RS, Smyk-Pearson S, Filipovich AH. Clinical and molecular features of the immunodysregulation, polyendocrinopathy, enteropathy, X linked (IPEX) syndrome. J Med Genet 2002;39(8):537-545.