UPMC Dermatopathology "Case of the Month" Presentations

UPP - Department of Dermatology, Dermatopathology Unit

5230 Centre Avenue (412) 864-3860 Pittsburgh, PA 15213 (412) 864-3890 FAX

Adar Berghoff, MD, Jill Buckthal-McCuin, MD, Arash Radfar MD, PhD, Laura Ferris MD, PhD

MAY 2008 CASE OF THE MONTH

CLINICAL FINDINGS

Clinical History

A 35 year old white man presented to our clinic on referral from the Rheumatology clinic with a 5-6 month history of swelling and tightening of his bilateral hands and bilateral lower extremities. He reported that his symptoms began distally and progressed proximally. He also noted pain with the tightening, beginning first in his lower extremities, progressing to his trunk, and then to his proximal upper extremities. He had no complaints consistent with Raynaud’s syndrome and no shortness of breath. He had initially received a course of prednisone for treatment, but his blood glucose could not be well controlled on this medication. His past medical history is significant for Diabetes Mellitus (Type I), for which he takes insulin through an insulin pump. He also notes a 15 year history of reflux for which he takes lansoprazole, and is also taking trazodone and ramipril.

Physical exam

The lower extremities were significant for tightness and firmness of the skin without epidermal changes present. The patient’s upper arms were noted to have an irregular and lumpy texture also without changes of the overlying epidermis. A “dry riverbed” sign was present (see figures 1, 2). No lesions consistent with calcinosis cutis, digital tip ulcerations, or telangiectasias are noted.

Histopathology

The first punch biopsy revealed deep dermal and subcutaneous sclerosis with a perivascular lymphoplasmacellular infiltrate. A Verhoeff-van Giesen stain was performed that illustrated a loss of elastic fibers in the deep dermis. PAS was performed and was negative for fungus and basement membrane thickening. Colloidal iron revealed a minimal increase in dermal mucin.

A second, deeper punch biopsy was performed for further evaluation. This specimen revealed less dermal fibrosis versus the earlier biopsy but a mild to focally moderately dense inflammatory cell infiltrate composed of lymphocytes and plasma cells was present. The infiltrate was noted to be predominantly perivascular and consistent with lymphocytic vasculitis. Of note, no eosinophils were described. See figures 3-6 for histopathology

Labs

Notable eosinophilia (7%), normal WBC, LFTs, H/H, TSH, ESR; positive ANA 1:160 with homogenous pattern, negative SCL70, SSA, SSB, ANCA

Differential Diagnosis

The differential diagnosis included scleroderma versus eosinophilic fasciitis versus other connective tissue disease. The patient was referred to dermatology for evaluation and skin biopsy. Two punch biopsies were performed at subsequent office visits.

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