An 80-year-old African-American man, with a past medical history of childho
od rheumatic fever, hypertension, coronary artery disease, unstable angina,
and asbestosis, underwent cardiac catheterization 37 years ago for unstabl
e angina. The postcatheterization course was complicated by a right brachia
l arterial thrombosis. A venous brachial bypass graft was placed, with vasc
ular supply to the affected arm restored.
Postbypass, the patient recovered full use of the arm, but suffered from pe
rsistent arm pain. Surgical sympathectomy was performed 1 year later. The p
atient has been pain-free since, but noted soon after sympathectomy, unilat
eral scaly dry skin, unilateral hypohidrosis of the axilla, and increased t
emperature sensitivity on the affected limb. In the 37 subsequent years, de
spite application of emollients, he reported no significant improvements or
changes in the skin findings.
The patient denied that other family members suffered from similar skin pro
blems. He denied ever having atopy or other skin problems prior to the curr
ent episode.
On physical examination, dry plate-like scales were seen on the dorsum of t
he right hand (Fig. 1A,B), right shoulder, and upper back, extending to the
midline (Fig. 1C), in an area of distribution supplied by the injured nerv
es. Mild follicular prominence of the affected extensor arm was also seen a
s compared with the uninvolved arm (not shown).
Bilateral 4-mm skin punch biopsies were obtained from the skin of the dorsu
m of the affected hand and unaffected control hand, in identical locations
between the first and second digits. Tissue specimens were split and each s
ent in formalin and glutaraldehyde for routine histology and electron micro
scopic evaluation, respectively.
For electron microscopy specimens, samples were fixed in 3% glutaraldehyde
solution, postfixed in ferrocyanide osmium tetroxide, and then samples were
dehydrated in graded series of ethanol and embedded in epoxy resin. Ultrat
hin sections were cut with a diamond knife on Sorval MT-2b ultramicrotome,
mounted on copper grids, and examined in a JEOL EM 100 electron microscope.
On routine microscopic evaluation, the affected skin demonstrated a thick h
yperkeratotic and compact stratum corneum (Fig. 2), as compared with the lo
ose basketweave appearance of the stratum corneum of the skin of the contro
l hand (Fig. 3).A thin and hypodense granular layer with more diffuse and s
mall keratohyaline granules was seen in the affected skin as compared with
the control. The overall epidermal thickness, however, appeared unchanged.
On electron microscopic evaluation, fewer, smaller, and more irregular kera
tohyaline granules in the granular layer of the affected skin were noted (F
ig. 4), as compared with the larger, more numerous stellate, polygonal conf
iguration of the control skin (Fig. 5). In an area of comparable epidermal
thickness, the affected skin granular layer was approximately 1-1.5 cells t
hick, whereas the control skin granular layer was 2.5-3 cells thick. Striki
ngly, the cornified layer in the affected skin was thickened with an abnorm
ally dense ultrastructure, as compared with the control skin (Fig. 6).