Association of scabies with a bullous pemphigoid-like eruption Lewis D. Slawsky, MD, Michele Maroon, MD, William B. Tyler, MD, a and O. Fred Miller HI, M D Danville, Pennsylvania We describe a patient with scabies who had an associated bullous pemphigoid-like eruption. Previous reports of this association have appeared in the literature. However, most case reports have described negative or nonspecific findings with direct immunofluorescence in contrast to the intense linear homogenous band of IgG and C3 found in our patient. (J AM ACAD DERMATOL1996;34:878-9.) Five patients with scabies presenting as bullous pemphigoid-like lesions have been described. 1-4 In four, the results of direct immunofluorescence were negative or nonspecific. W e describe a patient with persistent scabies and multiple bullae with bandlike deposition of IgG and C3, characteristic of bullous pemphigoid. CASE REPORT
A 76-year-old white man had pruritic blisters on his trunk and legs for 6 months. An initial biopsy specimen reportedly showed changes compatible with Grover's disease, but direct immunofluorescence study of later specimens revealed changes consistent with bullous pemphigoid. The patient was treated with prednisone, 60 mg daily, for 6 weeks without improvement. Examination disclosed scattered 2 to 6 mm intact and collapsed flaccid bullae predominantly involving the anterior part of the abdomen, buttocks, inguinal folds, and thighs (Fig. 1). In addition, diffuse areas of erythema and scaling were present on the buttocks, groin, and lower extremities. A few scattered erythematous papules were noted on the arms and legs. Biopsy specimens from the abdomen showed a subepidermal vesicle containing neutrophils, eosinophils, and fibrin (Fig. 2). A superficial perivascular and interstitial inflammatory infiltrate composed of small lymphoid cells, histiocytes, and occasional eosinophils was present in the dermis. Unexpectedly, a scabies mite was noted within the overlying stratum corneum (Fig. 2). Direct immunofluorescence of perilesional From the Departments of Dermatology and Pathology,a Geisinger Medical Center. Presented at the 53rd Annual Meeting of the American Academy of Dermatology Gross and Microscopic Symposium, New Orleans, La., Feb. 5, 1995. No reprints available from author. Copyright © 1996 by the American Academy of Dermatology, Inc. 0190-9622/96 $5;00 + 0 16/4/70037
878
Fig. 1. Flaccid bullae and erosions on thighs. skin revealed strong bandlike deposition of IgG and C3 along the basement membrane zone. Indirect immunofluorescence on monkey and guinea pig esophagus was negative. Prednisone, 60 mg daily, was continued, and administration of tetracycline, 1 gm daily, was begun. The scabies was treated with 5% permethrin cream. Scrapings 2 weeks later were negative. The patient continued to have bullae and erosions with persistent pruritus. Prednisone was increased to 80 mg daily, and azathioprine 50 mg twice daily was begun. One month later, all bullae had cleared. However, a repeat scraping demonstrated the presence of mites. Treatment with 5% permethrin cream was repeated, prednisone dosage was decreased to 60 mg daily, tetracycline was discontinued, and azathioprine was maintained. Three weeks later, the patient remained relatively flee of blisters. However, pruritus continued, and crusted hyperkeratotic plaques had developed on the buttocks and lower extremities (Fig. 3). Multiple burrows were present on the hands. Skin scrapings now revealed numerous mites and larvae. Azathioprine was discontinued, and the prednisone dosage was gradually decreased. Despite
Journal of the American Academy of Dermatology Volume 34, Number 5, Part 2
Fig. 2. Biopsy specimen showing subepidermal vesicle containing neutrophils, eosinophils, fibrin, and scabies mite within stratum comeum. (Hematoxylin-eosin stain; original magnification x200.) multiple weekly courses of both lindane and permethrin, the scabies infestation persisted for several weeks. However, no further bullae appeared. The infestation cleared after two 5-day applications of 6% precipitated sulfur in pelrolatum, 3 months after the patient was first seen. Prednisone (5 to 10 mg every other day) was continued and gradually withdrawn during the next 6 months. There has been no recurrence of bullae or scabies during the ensuing 12 months. DISCUSSION
Deposition of immune reactants within the skin has been previously observed in patients with scabies. Frentz, Veien, and Eriksen 5 studied 11 scabies patients and found C3 and granular IgM deposits at the basement membrane zone in one and C3 in another. Hoefling and Schroeter 6 observed scattered granular C3 staining at the dermoepidermal junction in three of four scabies patients. One also had granular deposits of IgM within the basement membrane zone. Deposition of IgG at the dermoepidermal junction was not found in either of these studies. Previous cases of scabies associated with a bullous pemphigoid-like eruption have also been reported. Viraben and Dupre 1 described a 34-year-old woman with a generalized, erythematous, scaling, papular, bullous eruption of several months' duration that failed to respond to prednisone and azathioprine. Scrapings demonstrated numerous mites. A biopsy specimen revealed a subepidermal blister with a sparse dermal inflammatory infiltrate; however, both direct and indirect immunofluorescence was negative. Ostlere, Harris, and Rustin 2 described a 36year-old woman with typical scabies who subsequently had multiple tense bullae and erosions. A
Slawsky et aL 879
Fig. 3. Crusted hyperkeratotic plaques on buttocks. biopsy specimen showed a subepidermal vesicle and a mixed lymphohistiocytic dermal infiltrate with eosinophils. Direct immunofluorescence demonstrated a linear band of C3 at the derrnoepidermal junction and perivascular C3 and IgM deposition. Indirect immunofluorescence revealed an IgG antibasement membrane antibody at a titer of 1:10 in the roof of salt-split skin. Parodi, Saino, and Rebora 3 described a 69-year-old man with a pruritic bullous eruption and scrapings positive for scabies who also had granular linear deposition of IgM and C3 at the dermoepidermal junction. Bhawan et al.4 described two patients with scabies and bullous pemphigoid, both of whom had deposition of IgG at the basement membrane zone, although the staining pattern was granular in one. Our patient had an intense linear band of both IgG and C3 at the dermoepidermal junction. This pattern is indistinguishable from bullous pemphigoid. This contrasts with previously reported cases that showed a negative or limited granular staining pattern or linear staining with C3 only. Our patient was later infested with Norwegian scabies, presumably because of the immunosuppressive treatment. REFERENCES
1. Viraben R, Dupre A. Scabies mimicking bullous pemphigoid. J AM ACADDEgMATOL1989;20:134-6. 2. Ostlere LS, Harris D, Rustin MH. Scabies associated with a bullous pemphigoid-like eruption. Br J Dermatol 1993; 128:217-9. 3. Parodi A, Saino M, Rebora A. Bullous pemphigoid-like scabies. Clin Exp Derrnatol 1993;18:293. 4. Bhawan J, MilstoneE, Malhotra R, et al. Scabiespresenting as buUouspemphigoid-likeeruption. J AMACADDFa~taTOL 1991;24:179-81. 5. Frentz G, Veien NK, Eriksen K. Immunofluorescence studies in scabies. J Cutan Pathol 1977;4:191-3. 6. Hoefling KK, Schroeter AL. Dermatoimmunopathologyof scabies. J AM ACADDERMATOL1980;3:237-40.
Hypothenar hammer syndrome: An uncommon cause of digital ischemia W. Christopher Duncan, M D Lakeland, Florida Unilateral digital ischemia may occur as a result of repetitive use of the hypothenar eminence as a hammer, with resultant damage to the wall of the ulnar artery and the superficial palmar arch. Secondary thrombus formation from intimal damage and potential embolization may cause signs and symptoms of arterial insufficiency in the second through fifth digits. Three automobile mechanics with unilateral ischemia caused by hypothenar hammer syndrome are described. All three patients had improvement with conservative treatment and continued to work with only minimal disability. (J AM ACAD DERMATOL1996;34:880-3.) Upper-extremity digital ischemia m a y be caused b y primary Raynaud's disease, Raynaud's phenomenon associated with connective tissue disorders, vasculitis, arterial emboli from a cardiac source, thromboangiitis obliterans, atherosclerosis with secondary thrombosis, and thoracic outlet syndrome, l, 2 In this report, I describe three cases o f a less comm o n cause o f digital ischemia, hypothenar hammer syndrome (HHS). W h e n the hypothenar area o f the hand is used to hammer, push, or squeeze hard objects, the superficial palmar branch o f the ulnar artery is compressed against the hook o f the hamate. Repetitive blunt trauma at this point may injure the ulnar artery and lead to its stenosis, occlusion, or aneurysm, with thrombosis and potential emboli. Patients with this syndrome are most c o m m o n l y men who have ischemia of the second, third, fourth, or fifth digits, the vascular supply o f which is derived from the ulnar artery, and report repetitive occupational or recreational use o f the hypothenar palm as a hammer), 4 CASE REPORTS Case 1. A 30-year-old, fight-handed auto mechanic had a lesion on the tip of his right fourth digit. He stated that a month earlier the skin had "darkened, dried, and fallen out, leaving a hole." Subsequently, a similar lesion appeared adjacent to the original "but didn't fail out." The finger was tender. Eleven years earlier, the patient had crushed the tips of four fingers of the right hand, but
From the Section of Dermatology, The Watson Clinic. Reprints not available from author. Copyright © 1996 by the American Academy of Dermatology, Inc. 0190-9622/96 $5.00 + 0 16/4/67685
880
he denied other hand problems. He had a 15 pack/year history of smoking. Examination revealed two 4 mm keratotic papules; one had a small central pit and the other was a hard keratin papule with surrounding erythema that extended to the distal interphalangeal joint on either side of the finger. The nail plate was not distorted (Fig. 1). A 3.5 mm punch biopsy specimen was obtained. The lesion extended to the periosteum, and the biopsy site did not bleed. The specimen was interpreted as a cicatrix with some areas suggestive of verruca. The patient did not return for follow-up. Thirteen months later, the patient returned with a painful lesion that had developed suddenly 10 days earlier on his right third digit. An eschar surrounded by the remnants of a bulla was present (Fig. 2). The fourth and fifth fingers were noticeably cooler than the remainder of the hand. The radial and ulnar pulses were palpable at the wrist. Arteriography demonstrated an ulnar artery that ended abruptly at the wrist. The radial artery was the sole blood supply to the hand. Tiny vessels for the third, fourth, and fifth digits originated from the deep palmar arch, but the digital artery of the fifth finger was never visualized, lntraarterial papaverine had no effect. Treatment with aspirin and pentoxifylline was initiated. The relationship of the patient's hand problems to his work was discussed. He was urged to discontinue smoking. The patient did not return for follow-up, and three years later he had minimal problems with his hand. He had continued taking aspirin and had modified his work habits. He had, however, continued smoking. Case 2. A 55-year-old, right-handed, truck mechanic had painful ulcers on the tips of the right second and third digits. These fingers had been "numb and tingling--like when they were frostbitten 25 years earlier" for several months before onset of pain. The patient denied color changes. He had been seen 1 year earlier. A dermatophyte