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Vesiculobullous systemic lupus erythematosus
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Vesiculobullous systemic lupus erythematosus A report of four cases Charles Camisa, M.D. Columbus, OH A vesiculobullous eruption is now recognized as a specific but rare cutaneous complication of systemic lupus erythematosus. Four additional cases are reported in whom the five previously proposed criteria were met. Increased activity of systemic lupus erythematosus affecting other organ systems was documented in three of four cases. All four patients demonstrated a positive lupus band, and three of four showed granular deposits of IgA along the basement membrane zone (BMZ). Evidence of glomerulonephritis was obtained in three of four cases, which resulted in death in one. The higher than expected incidence of IgA deposits in skin and renal disease in patients with vesiculobullous eruption of systemic lupus erythematosus is again confirmed. The eruption cleared in all four cases with either dapsone, 50 mg daily, or high doses of eorticosteroids and immunosuppressive agents. (J AM ACAD DERMATOL 1988; 18:93-100.)
Cutaneous manifestations of systemic lupus erythematosus occur in 76% of patients during the course o f their disease, but blistering lesions are relatively uncommon. Vesiculobullous lesions may be systemic lupus erythematosus-nonspecific (Table I) 1-12 or systemi c lupus erythematosusspecific, including cases in which severe liquefacrive degeneration of the basal cell layer and dermal edema result in separation of the epidermis and the dermis 13 and cases in which skin biopsy examination shows subepidermal vesicles containing neutrophils with microabscesses, nuclear "dust," and fibrin at the tips of dermal papillae, resembling dermatitis herpetiformis. 14 The latter eruption has been termed vesiculobullous systemic lupus erythematosus. It may or may not be associated with increased visceral activity of systemic lupus erythematosus and usually responds rapidly and completely to dapsone.
From the Department of Medicine, Division of Dermatology, The Ohio State University College of Medicine. Accepted for publication June 29, 1987. Reprint requests to: Dr. Charles Camisa, Cleveland Clinic Foundation, 9500 Euclid Ave. A61, Cleveland, OH 44106.
Four additional cases of vesiculobullous systemic lupus erythematosus are briefly reported here to increase awareness of this treatable cutaneous complication of systemic lupus erythematosus. CASE REPORTS
Case 1 A 33-year-old white woman had a 4-year history of systemic lupus erythematosus diagnosed on the basis of malar rash, Raynaud's phenomenon, positive antinuclear antibodies (ANAs) and lupus erythematosus cell test, hemolytic anemia, and thrombocytopenia. Current treatment included prednisone, 60 rag, and hydroxychloroquine, 400 mg daily. Six weeks prior to admission she developed progressive generalized swelling. Five days prior to admission she developed a mildly pruritic rash. Cutaneous examination showed diffuse facial erythema. Petechiae, papules, vesicles, and bullae (Fig. 1) were present on the dorsal aspects of hands and feet, elbows, knees, trunk, palms, and soles. The white blood cell (WBC) count was 7900/ram3, hemoglobin, 10.0 gm/dl, hematocrit, 29.8%, reticulocytes, 1.5% (normal, 0.5-1.5), platelets, 220,000/ram 3, C'3, 70 mg/dl (normal, 97-155), C'4, 15 mg/dl (1344), CH50, 111 U (70-206), and ANA 1 : 160, speckled pattern. 93
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T a b l e I. Systemic lupus erythematosusnonspecific blisters Bullous impetigo Herpesvirus infections Erythema multiforme ~ Leukocytoclastic vasculitis 2 Toxic epidermal necrolysis 3 Bullous pemphigoid 4-7 Linear IgA disease 8 Dermatitis herpetiformis 9,~° Porphyria cutanea tarda" Epidermolysis bullosa acquisita ~z
Evaluation of renal function showed a urinalysis with "small" blood, 3 + protein, >50 hyaline casts/lowpower field; 24-hour urinary protein was 9900 mg (normal, <150 rag), blood urea nitrogen (BUN), 60 mg/dl (normal, 4-24), creatinine, 1.0 mg/dl (0.6-1.3), and total protein, 3.1 gm/dl (normal, 6.1-7.7). These findings were indicative of the nephrotic syndrome. Renal biopsy revealed glomeruli with hyaline thrombi, "wire loops," mild to moderate mesangial proliferation, and occasional fibroepithelial' crescents. Electron microscopy showed massive mesangial subendothelial and intracapillary deposits and intramembranous and a few subepithelial deposits consistent with active diffuse lupus nephritis. The patient was treated with prednisone 40 mg, cyclophosphamide 75 mg, dipyridamole 225 mg, and aspirin 81 mg daily, with eventual resolution of her skin lesions.
Case 2 A 29-year-old white woman had a 6-year history of systemic lupus erythematosus based on fever of unknown origin, arthralgias, photosensitivity, malar rash, alopecia, oral ulcerations, and seizures. A renal biopsy performed 1 year prior to this presentation revealed diffuse proliferative glomerulonephritis with crescents consistent with lupus nephritis. Anti-deoxyribonucleic acid (DNA) antibody tests showed negative findings, She was treated successfully with plasmapheresis. She complained of a pruritic eruption of 3 weeks' duration while being maintained on prednisone 20 mg on alternate days. Cutaneous examination revealed clear blisters arising on normal skin, crusts, and urticarial lesions surmounted by vesiculation on the elbows (Fig. 2), knees, subclavicular areas (Fig. 3), waist area, and buttocks. WBC count was 12,700/mm 3, hemoglobin, 14 gm/dl, hematocrit, 42.4%, platelets,
300,000/ram 3, C'3, 107 mg/dl, and C'4, 9 mg/dl. Ad~ ditional laboratory tests revealed BUN, 19 mg/dl, cre, atinine, 1.0 mg/dl, 24-hour urinary protein, 157 mg (up to 150 rag), ANA, 1 : 160, diffuse pattern and nor real G6PD screen. She was treated with dapsone 50 mg daily and th~ same dose of prednisone, and her skin cleared within 2 weeks. Discontinuation of dapsone resulted in recu~ rence of blisters after 3 days. Restarting dapsone agai~ resulted in rapid resolution of blisters.
Case 3 A 31-year-old white man was transferred to 0hi~ ..... State University Hospital in 1982 for treatmeni of thrombotic thrombocytopenia. WBC count was ll,100/mm 3, hemoglobin, 8.4 gm/dl, hematocrit; 22.9%, and platelets, ll,000/mm "~, The peripheral smear contained spherocytes and schistocytes compat! ible with microangiopathic hemolytic anemia. ANA was >1:320, speckled pattern. He failed to respond adequately to 100 mg preclnisone daily and plasma e x change and subsequently underwent splenectomy 1983 he developed arthralgias and pericarditis, and the diagnosis of systemic lupus erythematosus was made. In 1984 he was readmitted because of confusion, agitation, dysphoric mood, and a vesiculobullous eruption after prolonged sun exposure. Examination of the skin showed urticarial papules and vesicles on an erythematous base on the sides of the neck (Fig. 4). Purpuric macules and blisters (measuring 0.5-1.5 cm in diameter) were present on the backs of both hands. There was arthritis of several metacarpophalangeal and proximal interphalangeal joints; Lumbar puncture revealed clear cerebrospinal fluid with 28 WBC/mm -~ (2% polymorphonuclear leukocytes, 93% lymphocytes, and 5% "others"), protein, 113 mg/dl, and glucose, 80 mg/dl. Bacterial, fungal, and viral cultures showed negative findings. Computed tomography showed mild cerebral atrophy but no focal abnormalities. The findings were consistent with lupus cerebritis. Further laboratory evaluation revealed: WBC count, 9,500/mm3; hemoglobin, 14.6 mg/dl; hematocrit, 44.6%; platelets, 610,000/mm3; C'3, 53 mg/dl; O4, 11 mg/dl; CH50, 10l U; BUN, 32 mg/dl; creatinine, 1.1 mg/dl; 24-hour urinary protein, 64 rag; ANA, >1:320, fibrillar pattern; negative anti-DNA antibodies; and normal urinalysis. The patient was treated with methylprednisolone, 250 mg every 6 hours intravenously and azathioprine, 150 mg daily. After a stormy 25-day hospital course
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Fig. 1. Case t. Photograph of knees demonstrates polymorphic eruption consisting of petechiae, urticarial papules, intact and ruptured bullae. Fig. 2. Case 2. Demonstrates vesicles and crusted excoriations of the elbows reminiscent of dermatitis herpetiformis. Fig. 3. Case 2. Right subclavicular area with grouped urticarial papules surmounted by vesicles. Fig. 4. Case 3. Photograph of left lateral aspect of neck shows urticarial to petechial papules, tense vesicles, and flaccid bullae.
his mental status normalized, and the skin eruption resolved.
Case 4 A 20-year-old black man was diagnosed as having systemic lupus erythematosus 11 years previously at Columbus Children's Hospital because of a rash and arthritis. Current treatment included prednisone 40 mg and hydroxychloroquine 400 mg daily. He had a 6-month history of a pruritic blistering eruption. The rheumatologist's diagnosis was pityriasis rosea and scabies; he then discontinued hydroxychloroquine for 3 weeks without improvement before requesting dermatologic consultation.
Dermatologic examination showed several tense blisters on the dorsal surfaces of the arms and hands, chest, back, and face. Hypopigmented macules persisted where blisters had spontaneously resolved. A few erosions were seen on the soft palate. Complete blood count showed 10,700 WBC/mm 3, hemoglobin, 14.3 gm/dl, hematocrit, 47%, and platelets, 276,000/mm 3. Urinalysis revealed > 1000 rag% protein, microscopic hematuria, and hyaline and cellular casts. Further laboratory studies revealed C'3, 73 mg/dl, BUN, 9 mg/dl, creatinine, 1.2 mg/dl, ANA, 1:512, homogeneous pattem, positive anti-DNA antibodies, normal screen for urinary porphyrin, and normal G6PD screen.
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T a b l e I I . Summary of histopathologic features of four cases of vesiculobullous systemic lupus erythematosus
Subepidermal blister with polymorphonuelear leukocytes and nuclear "dust"
Papillary mieroabscesses
1
+
-
-
+
2 3 4
+ + +
+ + +
+ +
+
Case
Dapsone, 50 mg daily, caused a rapid resolution of all blisters. There was no recurrence upon discontinuation of dapsone 2 months later. A kidney biopsy was performed 4 months later. It showed membranoproliferative glomerulonephritis with focal crescents, consistent with lupus nephritis. Electron microscopy showed subepithelial deposits indicative of membranous nephmpathy. He subsequently developed chronic hypertension, renal failure, and Pneumocystis carinii pneumonia and died 9 months after the skin biopsy. Histopathology. The results of routine light microscopy of hematoxylin and eosin-stained sections of lesional biopsy specimens are summarized in Table II. All four patients showed subepidermal vesicles containing serum, neutrophils, and nuclear debris (Figs. 5 and 6). Microabscesses at the tips of dermal papillae were seen in three of four patients. Evidence of leukocytoclastic vasculitis in the superficiaI and mid dermis was present in two of four patients. Vacuolar alteration of the basal cell layer was seen lateral to the blisters in two of four patients. Immunofluorescence s t u d i e s . The results of direct immunofluorescence (IF) studies of skin and kidney biopsy specimens are summarized in Table III. These studies were not performed on the kidney biopsy specimen of Patient 2. In all skin biopsies performed at the time of the vesiculobullous eruption, granular staining for IgG and/or IgM was noted at the basement membrane zone (BMZ). IgA was found in three of four patients. The earlier lupus band test in Patient 3 (during thrombotic thrombocytopenia) showed only IgM. When cerebritis and the skin eruption developed 22 months later, IgM was absent but IgG and IgA were present. Indirect immunofluorescence of sera of Patients 1 and 2 was performed on intact and 1.0 M sodium chloride (NaCI) separated normal human adult and newborn foreskin tissue.~5 No circulating autoantibodies to basement membrane zone were found.
Leukoeytoelastic vasculitis in papillary dermis
Vacuolar alteration
-
COMMENTS We have reported four additional cases of vesiculobullous eruption of systemic lupus erythematosus. Their clinical features are summarized in Table IV. Three of these eruptions were associated with increased activity of disease in other organ systems, namely, kidneys, joints, and brain, We TM and others ~6have previously reported the vesiculobullous eruptions of systemic lupus erythe, matosus associated with a :flare of the disease; however, Hall et al ~7 found active visceral disease in only one of four patients. They also reported the dramatic clearing of skin lesions in all four patients with dapsone, as was seen in Patients 2 and 4. It should be noted that the vesiculobullous eruption also clears slowly when high doses of corticosteroids and/or immunosuppressive drugs are administered to control life-threatening visceral manifestations of systemic lupus erythematosus, as exemplified by Cases 1 and 3. All four patients met the proposed criteria for establishing the diagnosis of vesiculobullous eruption of systemic lupus erythematosus: (1) a diagnosis of systemic lupus erythematosus based on the revised American Rheumatism Association criteria, (2) vesicles and bullae arising upon but not limited to sun-exposed skin, (3) histopathologic features compatible with dermatitis herpetiformis, (4) negative indirect immunofluorescence for circulating basement membrane zone (BMZ) antibodies, and (5) direct immunofluorescence revealing IgG and/or IgM and often IgA at the basement membrane zone. The detection of circulating autoantibodies to the epidermolysis bullosa acquisita antigen in four of five cases of vesiculobullous systemic lupus erythematosus ~8,j9 and in one of twenty cases of
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Fig. 5. Photomicrograph of section from blister shown in Fig. 4 demonstrates subepiderrnal blister containing polymorphonuclear leukocytes. The papillary dermis shows infiltration of polymorphonuclear leukocytes in and around walls of blood vessels. (Hematoxylineosin stain; × 100.) Fig. 6. Higher magnification of photomicrograph shown in Fig. 4 reveals remarkable vacuolar degeneration of basal cell layer of hair follicles, dermal edema, encircling accumulations of polymorphonuclear leukocytes, and separation from the dermis. (Hematoxylin-eosin stain; x 200.)
systemic lupus erythematosus patients without blisters ~8has recently been reported. Both groups used intact or 1.0 M NaC1 separated human skin as 'substrates in their indirect immunofluorescence assays. Therefore, it may be necessary to revise criterion 4 as follows: "Negative or positive indirect IF for circulating BMZ antibodies using separated human skin as substrate." We examined the sera of Patients 1 and 2 and our two previously reported patients with the use of the separated human skin technique; negative results were obtained in all four patients? ° The significance of the increased prevalence of granular IgA deposits along the basement membrane zone in perilesional and nonlesional skin of patients with vesiculobullous systemic lupus erythematosus is uncertain. Including the present series, IgA has been detected in 22 of 29 (76%) 14'Ia,19,2I26 reported cases compared to a prevalence of about 17% in unselected patients
with systemic lupus erythematosus in the literature. 14,27-29 IgA may simply be a marker for increased disease activity. Interestingly, the single patient without igA at the time of the skin eruption in this study (Case 2) was also the only one with stable systemic lupus erythematosus (arthritis, mild proteinuria, and decreased creatinine clearance). In summary, vesiculobullous eruption of systemic lupus erythematosus shares some morphologic, histologic, and immunopathologic features with dermatitis herpetiformis. Moreover, with occasional exceptions,~9'3° both entities respond dramatically to dapsone. Some cases previously reported as the coexistence of dermatitis herpetiformis and systemic lupus erythematosus probably represented vesiculobullous systemic lupus erythematosus. 9,3~.3zIn at least two such cases, a beneficial response was apparently obtained with a gluten-free diet. °
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Table III. Direct immunofluorescence studies on skin and kidney biopsy specimens of patients with vesiculobullous systemic lupus erythematosus* Case
Date 12/84 12/84
]
Biopsy
I
Non-sun-exposed normal skin Kidney
2
9/83
Perilesional skin
3
9/82
Normal skin
7/84
Perilesional skin
1/83
Non-sun-exposed normal skin Non-sun-exposed normal skin Kidney
4
4/83
IgG
]
+ + + granular BMZ + + + granular GBM mesangium + + vessel walls
IgA
]
+ + + granular BMZ + + + globular smudgy GMB
IgM + + + granular BMZ + + globular smudgy GBM
+ + + granular BMZ + granular BMZ + + + granular BMZ
-
+ granular BMZ + + + diffuse granular GBM, r u e s angium, Bowman' s capsule
+ + granular BMZ + + granular BMZ + granular BMZ + diffuse granular GBM, mesangium, Bowman's capsule
+ + + granular BMZ + granular BMZ
BMZ:Basementmembranezone;GBM:glomerularbasementmembrane;ND: not done. *Intensityof immunofluoresceneeis gradedfrom - to + + +.
Table IV. Summary of clinical features of four cases of vesiculobullous systemic lupus erythematosus
I
Durationof SLE (yr)
Case
Sex
Race
Age
prior to eruption
1
F
W
33
4
2 3
F M
W W
29 33
6 2
4
M
B
20
11
B: Black;W: white;SLE:systemiclupuserythematosus.
Visceral flare?
Treatment
Nephrotic syndrome; diffuse lupus nephritis None Arthritis; cerebritis
Cleared with reduction of prednisone 60 to 40 mg daily and addition of cyclophosphamide 75 mg daily Cleared with 50 mg dapsone daily Cleared gradually with intravenous methylprednisolone 1000 mg and azathioprine 150 nag daily Cleared with 50 nag dapsone daily
Lupus nephritis
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Vesiculobullous systemic lupus erythematosus
C'3
C'4
+ + granular BMZ + + + globular and smudgy in glomeruli + + vessel walls
+ granular BMZ + + same pattern as C'3
ND + -t- granular B M Z
+ granular B M Z + + diffuse granular GBM, mesangium
REFERENCES 1. Rowell NR, Anderson JR. Lupus erythematosus and erythema multiforme-like lesions. Arch Dermatol 1963; 88:176-80. 2. Callen JP. Cutaneous bullae following acute steroid withdrawal in systemic lupus erythematosus. Br J Dermatol 1981;105:603-6. 3. Braverman IM. Skin signs of systemic disease. 2nd ed. Philadelphia: WB Saunders, 1981:287. 4. Jordan RE, Muller SA, Hale WL, Beutner EH. Bullous pemphigoid associated with systemic lupus erythematosus. Arch Dermatol 1969;99:17-25. 5. Miller JF, Downham TF, Chapel TA. Coexistent bullous pemphigoid and systemic lupus erythematosus. Cutis 1978;21:368-73. 6. Kumar V, Binder WL, Schotland E, et al. Coexistence of bullous pemphigoid and systemic lupus erythematosus. Arch Dermatol 1978;114:1187-90. 7. Clayton CA, Burnham TK. Systemic lupus erythematosus and coexisting bullous pemphigoid: immunofluorescent investigations. J AM ACAD DERMATOL 1982; 7:236-45.
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8. Szab6 E, Husz S, Kov~cs L. Coexistent atypical bullous pemphigoid and systemic lupus erythematosus. Br J Dermatol 1981;104:71-5. 9. Thomas JR, Su WPD. Concurrence of lupus erythematosus and dermatitis herpetiformis. Arch Dermatol 1983; 119:740-5. 10. Aronson AJ, Soltani K, Aronson IK, Oug RT. Systemic lupus erythematosus and dermatitis herpetiformis. Arch Dermatol 1979;115:68-70. 11. Cram DL, Epstein JI-I, Tuffanelli DL. Lupus erythematosus and porphyria: coexistence in seven patients. Arch Dermatol 1973;108:779-84. 12. Dotson AD, Raimer SS, Pursley TV, Tschen J. Systemic lupus erythematosus occurring in a patient with epidermolysis bullosa acquisita. Arch Dermatol 1981;117: 422-6. 13. Aekerman AB. Histologic diagnosis of inflammatory diseases. Philadelphia: Lea & Febiger, 1978:631. 14. Camisa C, Sharma HM. VesiculobuUous systemic lupus erythematosus. J AM ACADDERMATOL1983;9:924-33. 15. Gammon WR, Briggaman RA, Inman AO HI, Queen LL, Wheeler CE. Differentiating anti-lamina lucida and anti-sublamina densa anti-BMZ antibodies by indirect immunofluorescence on 1.0 M sodium chloride-separated skin. J Invest Dermatol 1984;82:139-44. 16. Penneys NS, WileyHE. Herpetiform blisters in systemic lupus erythematosus. Arch Dermatoi 1979; 115:1427-8. 17. Hall RP, LawleyTJ, Smith HR, Katz SL Bullous eruption of systemic lupus erythematosus. Dramatic response to dapsone therapy. Ann Interm Med 1982;97:165-70. 18. Gammon WR, Woodley DT, Dole KC, Bdggaman RA. Evidence that antibasement membrane zone antibodies in bullous eruption of systemic lupus erythematosus recognize epidermolysis bullosa acquisita autoantigen. J Invest Dermatol 1985;84:472-6. 19. Barton DD, Fine J-D, Gammon WR, Sams WM Jr. Bullous systemic lupus erythematosus: an unusual clinical course and detectable circulating autoantibodies to the epidermolysis bullosa aequisita antigen. J AM ACAoDERMATOL1986;15:369-73. 20. Camisa C, Grimwood RE. Indirect immunofluorescenee in vesiculobullous eruption of systemic lupus erythematosus. J Invest Dermatol 1986;86:606. 21. Morel P, Brouet J-C, Anxionnaz S, Mazel C, Civatte J. Lupus 6ryth~mateux syst6mique avec bulles. Ann Dermatol Venereol 1982;109:791-2. 22. Crick XB, Levet R, Blechet-Butaye F, Vissuzaine C, Belaich S. Lupus 6ryth6mateux syst6mique avec 16sions bulleuses. Ann Dermatol Venereol 1983;110:455-9. 23. Saurat J-H, Chavaz P, Didierjean L, Schaer B. Bullous eruption of systemic lupus erythematosus: "Herpetiform pemphigus" [letter]. J AM AC_ADDEaMATOL1983;9:163. 24. Tani M, Shimizu R, Ban M, Murata Y, Tamaki A. Systemic lupus erythematosus with vesiculobullous lesions: Immunoelectron microscopic studies. Arch Dermatol 1984; 120:1497-1501. 25. Aboobaker J, Ramsaroop R, Abramowitz I, Naran AD. Bullous systemic erythematosus. A case report. S Aft Med J 1986;69:49-51. 26. Miller JA, Dowd PM, Dudeney C, Isenberg DA. Vesiculobullous eruption in systemic lupus erythematosus:
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demonstration of common anti-DNA antibody idiotype at the detmo-epidcrmal junction. J R So<: Med 1986; 79:365-7. 27. Provost "IT. Lupus band test. Jut J Dermatol 1981; 20:475-8I. 28. Jacobs MI, Schned ES, Bystryn J-C. Variability of the lupus band test. Results in 18 patients with systemiclupus erythematosus. Arch Dermatol 1983;119:883-9. 29. Smith CD, Marino C, Rothfield NF. The clinical utility of the lupus band test. Arthritis Rheum 1984;27:382-7.
30. Alarcon GS, Sams WM, Barton DD, Reveille J. Bullous lupus erythematosus rash worsened by dapsone [letter]. Arthritis Rheum 1984;27:1071-2. 31. Davies MG, Marks R, Waddington E. Simultaneous sys, temie lupus erythematosus and dermatitis herpetiformis. Arch Dermatol 1976;112" 1292-4. 32. Moncada B. Dermatitis herpetiformis in association with systemic lupus erythematosus. Arch Dermatol 1974;109: 723-5.
ABSTRACTS
Reversal of advanced m u r i n e lupus in NZB/NZW F1 mice by treatment with monodonal antibody to L3T4
Squamous cell carcinoma of the skin in black patients with discoid lupus erythematosus
Wofsy D, Seaman WE. J Immunol 1987;138:3247-53
Caruso WR, Stewart ML, Nanda VK, et al. J Rheumatol 1987;14:156-9
Successful preventative therapy of lupus erythematosus is described in regard to the murine disease. Can this be done for the human form of lupus erythematosus? Philip C. Anderson, M.D.
Black persons with intensely pigmented skin seldom have squamous cell carcinoma. However, be watchful of their skin lesions of discoid lupus erythematosus becoming keratinizing cancer, as reported here in seven cases. Philip C. Anderson, M.D.
Protection of guinea pigs from primary a n d recurrent herpes simplex virus (HSV) type 2 cutaneous disease with vaecinia virus recombinants expressing HSV glycoproteln D
Psoriatic arthritis: clinical subgroups and histocompatibility antigens
Wachsman M, Aurelian L, Smith CC, et al. J Infect Dis 1987;155:1188-97
McHugh NJ, Laurent MR, Treadwell BL, ct al. Ann Rheum Dis 1987;46:184-8
To build a better herpes vaccine one uses the basic chassis of the smallpox virus and then adds some custom features of herpesvirus. When glycoprotein D from herpesvirus is expressed, the resulting vaccine protects young guinea pigs well. Progress on a vaccine for humans is slew but determined. Philip C. Anderson, M.D.
Sixty patients were studied. Remember, only the patients with spendylitishave a remarkable incidence of the B27 antigen. Antigen DR7 was associated with severe chronic peripheral arthritis.Neither of these markers is pathognomonic alone. Philip C. Anderson, M.D.
Traumatic arteriovenous fistula of the scalp. Case report
naevi may be related to metabolic activity
Variations in S-100 protein expression in naevocellular
Badejo L, Rockwood P, J Neurosurg 1987;66:773-4 This vascular fistula was induced by a air gun pellet. These interesting lesions are cured best by excision. Philip C. Anderson, M.D. Vitamin A and D status of black South African women and their babies Faimey A, S]oan MA, Patel KV, et al. Hum Nutr Clin Nutr 1987;41:81-7 Persons living in a poor cultural economy and these who traditionally suffer from poor nutrition often have a high rate of childhood mortality. They have many other medical problems. One reliable index to this deficiencyis the low serum retJnoi values. On the other hand, these people usually have normal vitamin D status because of actinic synthesis in skin+ Philip C. Anderson, M.D.
Paul E, Wen DR, Cochran AJ. Br J Dermatol 1987;116:371-8 The S-100 proteins on nevus cells are not purified. They can be detected by chemical tags, We do not know much about the biochemistry. Staining is extremelyvariable and unexplained. Giantcells and repairing nevi stain strongly for S-100, while more inert nevus cells may not stain at all. Increased metabolic activity seems to cause expression of the S-100 proteins. The utility of this stain for clinical decisions is being decided. Philip C. Anderson, M.D, Spontaneous passage of renal calculi through nephrocutaneous fistula due to calculous pyelonephritis Iseki T, Kawamura M. Br J Urol 1987;59:285-6 In the world of fistulas, almost anything can happen. "Kidney to skin" fistulas are rare, and the passage of renal stones is amazing. Philip C. Anderson, M.D.
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Vesiculobullous systemic lupus erythematosus A report of four cases Charles Camisa, M.D. Columbus, OH A vesiculobullous eruption is now recognized as a specific but rare cutaneous complication of systemic lupus erythematosus. Four additional cases are reported in whom the five previously proposed criteria were met. Increased activity of systemic lupus erythematosus affecting other organ systems was documented in three of four cases. All four patients demonstrated a positive lupus band, and three of four showed granular deposits of IgA along the basement membrane zone (BMZ). Evidence of glomerulonephritis was obtained in three of four cases, which resulted in death in one. The higher than expected incidence of IgA deposits in skin and renal disease in patients with vesiculobullous eruption of systemic lupus erythematosus is again confirmed. The eruption cleared in all four cases with either dapsone, 50 mg daily, or high doses of eorticosteroids and immunosuppressive agents. (J AM ACAD DERMATOL 1988; 18:93-100.)
Cutaneous manifestations of systemic lupus erythematosus occur in 76% of patients during the course o f their disease, but blistering lesions are relatively uncommon. Vesiculobullous lesions may be systemic lupus erythematosus-nonspecific (Table I) 1-12 or systemi c lupus erythematosusspecific, including cases in which severe liquefacrive degeneration of the basal cell layer and dermal edema result in separation of the epidermis and the dermis 13 and cases in which skin biopsy examination shows subepidermal vesicles containing neutrophils with microabscesses, nuclear "dust," and fibrin at the tips of dermal papillae, resembling dermatitis herpetiformis. 14 The latter eruption has been termed vesiculobullous systemic lupus erythematosus. It may or may not be associated with increased visceral activity of systemic lupus erythematosus and usually responds rapidly and completely to dapsone.
From the Department of Medicine, Division of Dermatology, The Ohio State University College of Medicine. Accepted for publication June 29, 1987. Reprint requests to: Dr. Charles Camisa, Cleveland Clinic Foundation, 9500 Euclid Ave. A61, Cleveland, OH 44106.
Four additional cases of vesiculobullous systemic lupus erythematosus are briefly reported here to increase awareness of this treatable cutaneous complication of systemic lupus erythematosus. CASE REPORTS
Case 1 A 33-year-old white woman had a 4-year history of systemic lupus erythematosus diagnosed on the basis of malar rash, Raynaud's phenomenon, positive antinuclear antibodies (ANAs) and lupus erythematosus cell test, hemolytic anemia, and thrombocytopenia. Current treatment included prednisone, 60 rag, and hydroxychloroquine, 400 mg daily. Six weeks prior to admission she developed progressive generalized swelling. Five days prior to admission she developed a mildly pruritic rash. Cutaneous examination showed diffuse facial erythema. Petechiae, papules, vesicles, and bullae (Fig. 1) were present on the dorsal aspects of hands and feet, elbows, knees, trunk, palms, and soles. The white blood cell (WBC) count was 7900/ram3, hemoglobin, 10.0 gm/dl, hematocrit, 29.8%, reticulocytes, 1.5% (normal, 0.5-1.5), platelets, 220,000/ram 3, C'3, 70 mg/dl (normal, 97-155), C'4, 15 mg/dl (1344), CH50, 111 U (70-206), and ANA 1 : 160, speckled pattern. 93
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T a b l e I. Systemic lupus erythematosusnonspecific blisters Bullous impetigo Herpesvirus infections Erythema multiforme ~ Leukocytoclastic vasculitis 2 Toxic epidermal necrolysis 3 Bullous pemphigoid 4-7 Linear IgA disease 8 Dermatitis herpetiformis 9,~° Porphyria cutanea tarda" Epidermolysis bullosa acquisita ~z
Evaluation of renal function showed a urinalysis with "small" blood, 3 + protein, >50 hyaline casts/lowpower field; 24-hour urinary protein was 9900 mg (normal, <150 rag), blood urea nitrogen (BUN), 60 mg/dl (normal, 4-24), creatinine, 1.0 mg/dl (0.6-1.3), and total protein, 3.1 gm/dl (normal, 6.1-7.7). These findings were indicative of the nephrotic syndrome. Renal biopsy revealed glomeruli with hyaline thrombi, "wire loops," mild to moderate mesangial proliferation, and occasional fibroepithelial' crescents. Electron microscopy showed massive mesangial subendothelial and intracapillary deposits and intramembranous and a few subepithelial deposits consistent with active diffuse lupus nephritis. The patient was treated with prednisone 40 mg, cyclophosphamide 75 mg, dipyridamole 225 mg, and aspirin 81 mg daily, with eventual resolution of her skin lesions.
Case 2 A 29-year-old white woman had a 6-year history of systemic lupus erythematosus based on fever of unknown origin, arthralgias, photosensitivity, malar rash, alopecia, oral ulcerations, and seizures. A renal biopsy performed 1 year prior to this presentation revealed diffuse proliferative glomerulonephritis with crescents consistent with lupus nephritis. Anti-deoxyribonucleic acid (DNA) antibody tests showed negative findings, She was treated successfully with plasmapheresis. She complained of a pruritic eruption of 3 weeks' duration while being maintained on prednisone 20 mg on alternate days. Cutaneous examination revealed clear blisters arising on normal skin, crusts, and urticarial lesions surmounted by vesiculation on the elbows (Fig. 2), knees, subclavicular areas (Fig. 3), waist area, and buttocks. WBC count was 12,700/mm 3, hemoglobin, 14 gm/dl, hematocrit, 42.4%, platelets,
300,000/ram 3, C'3, 107 mg/dl, and C'4, 9 mg/dl. Ad~ ditional laboratory tests revealed BUN, 19 mg/dl, cre, atinine, 1.0 mg/dl, 24-hour urinary protein, 157 mg (up to 150 rag), ANA, 1 : 160, diffuse pattern and nor real G6PD screen. She was treated with dapsone 50 mg daily and th~ same dose of prednisone, and her skin cleared within 2 weeks. Discontinuation of dapsone resulted in recu~ rence of blisters after 3 days. Restarting dapsone agai~ resulted in rapid resolution of blisters.
Case 3 A 31-year-old white man was transferred to 0hi~ ..... State University Hospital in 1982 for treatmeni of thrombotic thrombocytopenia. WBC count was ll,100/mm 3, hemoglobin, 8.4 gm/dl, hematocrit; 22.9%, and platelets, ll,000/mm "~, The peripheral smear contained spherocytes and schistocytes compat! ible with microangiopathic hemolytic anemia. ANA was >1:320, speckled pattern. He failed to respond adequately to 100 mg preclnisone daily and plasma e x change and subsequently underwent splenectomy 1983 he developed arthralgias and pericarditis, and the diagnosis of systemic lupus erythematosus was made. In 1984 he was readmitted because of confusion, agitation, dysphoric mood, and a vesiculobullous eruption after prolonged sun exposure. Examination of the skin showed urticarial papules and vesicles on an erythematous base on the sides of the neck (Fig. 4). Purpuric macules and blisters (measuring 0.5-1.5 cm in diameter) were present on the backs of both hands. There was arthritis of several metacarpophalangeal and proximal interphalangeal joints; Lumbar puncture revealed clear cerebrospinal fluid with 28 WBC/mm -~ (2% polymorphonuclear leukocytes, 93% lymphocytes, and 5% "others"), protein, 113 mg/dl, and glucose, 80 mg/dl. Bacterial, fungal, and viral cultures showed negative findings. Computed tomography showed mild cerebral atrophy but no focal abnormalities. The findings were consistent with lupus cerebritis. Further laboratory evaluation revealed: WBC count, 9,500/mm3; hemoglobin, 14.6 mg/dl; hematocrit, 44.6%; platelets, 610,000/mm3; C'3, 53 mg/dl; O4, 11 mg/dl; CH50, 10l U; BUN, 32 mg/dl; creatinine, 1.1 mg/dl; 24-hour urinary protein, 64 rag; ANA, >1:320, fibrillar pattern; negative anti-DNA antibodies; and normal urinalysis. The patient was treated with methylprednisolone, 250 mg every 6 hours intravenously and azathioprine, 150 mg daily. After a stormy 25-day hospital course
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Fig. 1. Case t. Photograph of knees demonstrates polymorphic eruption consisting of petechiae, urticarial papules, intact and ruptured bullae. Fig. 2. Case 2. Demonstrates vesicles and crusted excoriations of the elbows reminiscent of dermatitis herpetiformis. Fig. 3. Case 2. Right subclavicular area with grouped urticarial papules surmounted by vesicles. Fig. 4. Case 3. Photograph of left lateral aspect of neck shows urticarial to petechial papules, tense vesicles, and flaccid bullae.
his mental status normalized, and the skin eruption resolved.
Case 4 A 20-year-old black man was diagnosed as having systemic lupus erythematosus 11 years previously at Columbus Children's Hospital because of a rash and arthritis. Current treatment included prednisone 40 mg and hydroxychloroquine 400 mg daily. He had a 6-month history of a pruritic blistering eruption. The rheumatologist's diagnosis was pityriasis rosea and scabies; he then discontinued hydroxychloroquine for 3 weeks without improvement before requesting dermatologic consultation.
Dermatologic examination showed several tense blisters on the dorsal surfaces of the arms and hands, chest, back, and face. Hypopigmented macules persisted where blisters had spontaneously resolved. A few erosions were seen on the soft palate. Complete blood count showed 10,700 WBC/mm 3, hemoglobin, 14.3 gm/dl, hematocrit, 47%, and platelets, 276,000/mm 3. Urinalysis revealed > 1000 rag% protein, microscopic hematuria, and hyaline and cellular casts. Further laboratory studies revealed C'3, 73 mg/dl, BUN, 9 mg/dl, creatinine, 1.2 mg/dl, ANA, 1:512, homogeneous pattem, positive anti-DNA antibodies, normal screen for urinary porphyrin, and normal G6PD screen.
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T a b l e I I . Summary of histopathologic features of four cases of vesiculobullous systemic lupus erythematosus
Subepidermal blister with polymorphonuelear leukocytes and nuclear "dust"
Papillary mieroabscesses
1
+
-
-
+
2 3 4
+ + +
+ + +
+ +
+
Case
Dapsone, 50 mg daily, caused a rapid resolution of all blisters. There was no recurrence upon discontinuation of dapsone 2 months later. A kidney biopsy was performed 4 months later. It showed membranoproliferative glomerulonephritis with focal crescents, consistent with lupus nephritis. Electron microscopy showed subepithelial deposits indicative of membranous nephmpathy. He subsequently developed chronic hypertension, renal failure, and Pneumocystis carinii pneumonia and died 9 months after the skin biopsy. Histopathology. The results of routine light microscopy of hematoxylin and eosin-stained sections of lesional biopsy specimens are summarized in Table II. All four patients showed subepidermal vesicles containing serum, neutrophils, and nuclear debris (Figs. 5 and 6). Microabscesses at the tips of dermal papillae were seen in three of four patients. Evidence of leukocytoclastic vasculitis in the superficiaI and mid dermis was present in two of four patients. Vacuolar alteration of the basal cell layer was seen lateral to the blisters in two of four patients. Immunofluorescence s t u d i e s . The results of direct immunofluorescence (IF) studies of skin and kidney biopsy specimens are summarized in Table III. These studies were not performed on the kidney biopsy specimen of Patient 2. In all skin biopsies performed at the time of the vesiculobullous eruption, granular staining for IgG and/or IgM was noted at the basement membrane zone (BMZ). IgA was found in three of four patients. The earlier lupus band test in Patient 3 (during thrombotic thrombocytopenia) showed only IgM. When cerebritis and the skin eruption developed 22 months later, IgM was absent but IgG and IgA were present. Indirect immunofluorescence of sera of Patients 1 and 2 was performed on intact and 1.0 M sodium chloride (NaCI) separated normal human adult and newborn foreskin tissue.~5 No circulating autoantibodies to basement membrane zone were found.
Leukoeytoelastic vasculitis in papillary dermis
Vacuolar alteration
-
COMMENTS We have reported four additional cases of vesiculobullous eruption of systemic lupus erythematosus. Their clinical features are summarized in Table IV. Three of these eruptions were associated with increased activity of disease in other organ systems, namely, kidneys, joints, and brain, We TM and others ~6have previously reported the vesiculobullous eruptions of systemic lupus erythe, matosus associated with a :flare of the disease; however, Hall et al ~7 found active visceral disease in only one of four patients. They also reported the dramatic clearing of skin lesions in all four patients with dapsone, as was seen in Patients 2 and 4. It should be noted that the vesiculobullous eruption also clears slowly when high doses of corticosteroids and/or immunosuppressive drugs are administered to control life-threatening visceral manifestations of systemic lupus erythematosus, as exemplified by Cases 1 and 3. All four patients met the proposed criteria for establishing the diagnosis of vesiculobullous eruption of systemic lupus erythematosus: (1) a diagnosis of systemic lupus erythematosus based on the revised American Rheumatism Association criteria, (2) vesicles and bullae arising upon but not limited to sun-exposed skin, (3) histopathologic features compatible with dermatitis herpetiformis, (4) negative indirect immunofluorescence for circulating basement membrane zone (BMZ) antibodies, and (5) direct immunofluorescence revealing IgG and/or IgM and often IgA at the basement membrane zone. The detection of circulating autoantibodies to the epidermolysis bullosa acquisita antigen in four of five cases of vesiculobullous systemic lupus erythematosus ~8,j9 and in one of twenty cases of
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Fig. 5. Photomicrograph of section from blister shown in Fig. 4 demonstrates subepiderrnal blister containing polymorphonuclear leukocytes. The papillary dermis shows infiltration of polymorphonuclear leukocytes in and around walls of blood vessels. (Hematoxylineosin stain; × 100.) Fig. 6. Higher magnification of photomicrograph shown in Fig. 4 reveals remarkable vacuolar degeneration of basal cell layer of hair follicles, dermal edema, encircling accumulations of polymorphonuclear leukocytes, and separation from the dermis. (Hematoxylin-eosin stain; x 200.)
systemic lupus erythematosus patients without blisters ~8has recently been reported. Both groups used intact or 1.0 M NaC1 separated human skin as 'substrates in their indirect immunofluorescence assays. Therefore, it may be necessary to revise criterion 4 as follows: "Negative or positive indirect IF for circulating BMZ antibodies using separated human skin as substrate." We examined the sera of Patients 1 and 2 and our two previously reported patients with the use of the separated human skin technique; negative results were obtained in all four patients? ° The significance of the increased prevalence of granular IgA deposits along the basement membrane zone in perilesional and nonlesional skin of patients with vesiculobullous systemic lupus erythematosus is uncertain. Including the present series, IgA has been detected in 22 of 29 (76%) 14'Ia,19,2I26 reported cases compared to a prevalence of about 17% in unselected patients
with systemic lupus erythematosus in the literature. 14,27-29 IgA may simply be a marker for increased disease activity. Interestingly, the single patient without igA at the time of the skin eruption in this study (Case 2) was also the only one with stable systemic lupus erythematosus (arthritis, mild proteinuria, and decreased creatinine clearance). In summary, vesiculobullous eruption of systemic lupus erythematosus shares some morphologic, histologic, and immunopathologic features with dermatitis herpetiformis. Moreover, with occasional exceptions,~9'3° both entities respond dramatically to dapsone. Some cases previously reported as the coexistence of dermatitis herpetiformis and systemic lupus erythematosus probably represented vesiculobullous systemic lupus erythematosus. 9,3~.3zIn at least two such cases, a beneficial response was apparently obtained with a gluten-free diet. °
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Table III. Direct immunofluorescence studies on skin and kidney biopsy specimens of patients with vesiculobullous systemic lupus erythematosus* Case
Date 12/84 12/84
]
Biopsy
I
Non-sun-exposed normal skin Kidney
2
9/83
Perilesional skin
3
9/82
Normal skin
7/84
Perilesional skin
1/83
Non-sun-exposed normal skin Non-sun-exposed normal skin Kidney
4
4/83
IgG
]
+ + + granular BMZ + + + granular GBM mesangium + + vessel walls
IgA
]
+ + + granular BMZ + + + globular smudgy GMB
IgM + + + granular BMZ + + globular smudgy GBM
+ + + granular BMZ + granular BMZ + + + granular BMZ
-
+ granular BMZ + + + diffuse granular GBM, r u e s angium, Bowman' s capsule
+ + granular BMZ + + granular BMZ + granular BMZ + diffuse granular GBM, mesangium, Bowman's capsule
+ + + granular BMZ + granular BMZ
BMZ:Basementmembranezone;GBM:glomerularbasementmembrane;ND: not done. *Intensityof immunofluoresceneeis gradedfrom - to + + +.
Table IV. Summary of clinical features of four cases of vesiculobullous systemic lupus erythematosus
I
Durationof SLE (yr)
Case
Sex
Race
Age
prior to eruption
1
F
W
33
4
2 3
F M
W W
29 33
6 2
4
M
B
20
11
B: Black;W: white;SLE:systemiclupuserythematosus.
Visceral flare?
Treatment
Nephrotic syndrome; diffuse lupus nephritis None Arthritis; cerebritis
Cleared with reduction of prednisone 60 to 40 mg daily and addition of cyclophosphamide 75 mg daily Cleared with 50 mg dapsone daily Cleared gradually with intravenous methylprednisolone 1000 mg and azathioprine 150 nag daily Cleared with 50 nag dapsone daily
Lupus nephritis
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Vesiculobullous systemic lupus erythematosus
C'3
C'4
+ + granular BMZ + + + globular and smudgy in glomeruli + + vessel walls
+ granular BMZ + + same pattern as C'3
ND + -t- granular B M Z
+ granular B M Z + + diffuse granular GBM, mesangium
REFERENCES 1. Rowell NR, Anderson JR. Lupus erythematosus and erythema multiforme-like lesions. Arch Dermatol 1963; 88:176-80. 2. Callen JP. Cutaneous bullae following acute steroid withdrawal in systemic lupus erythematosus. Br J Dermatol 1981;105:603-6. 3. Braverman IM. Skin signs of systemic disease. 2nd ed. Philadelphia: WB Saunders, 1981:287. 4. Jordan RE, Muller SA, Hale WL, Beutner EH. Bullous pemphigoid associated with systemic lupus erythematosus. Arch Dermatol 1969;99:17-25. 5. Miller JF, Downham TF, Chapel TA. Coexistent bullous pemphigoid and systemic lupus erythematosus. Cutis 1978;21:368-73. 6. Kumar V, Binder WL, Schotland E, et al. Coexistence of bullous pemphigoid and systemic lupus erythematosus. Arch Dermatol 1978;114:1187-90. 7. Clayton CA, Burnham TK. Systemic lupus erythematosus and coexisting bullous pemphigoid: immunofluorescent investigations. J AM ACAD DERMATOL 1982; 7:236-45.
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8. Szab6 E, Husz S, Kov~cs L. Coexistent atypical bullous pemphigoid and systemic lupus erythematosus. Br J Dermatol 1981;104:71-5. 9. Thomas JR, Su WPD. Concurrence of lupus erythematosus and dermatitis herpetiformis. Arch Dermatol 1983; 119:740-5. 10. Aronson AJ, Soltani K, Aronson IK, Oug RT. Systemic lupus erythematosus and dermatitis herpetiformis. Arch Dermatol 1979;115:68-70. 11. Cram DL, Epstein JI-I, Tuffanelli DL. Lupus erythematosus and porphyria: coexistence in seven patients. Arch Dermatol 1973;108:779-84. 12. Dotson AD, Raimer SS, Pursley TV, Tschen J. Systemic lupus erythematosus occurring in a patient with epidermolysis bullosa acquisita. Arch Dermatol 1981;117: 422-6. 13. Aekerman AB. Histologic diagnosis of inflammatory diseases. Philadelphia: Lea & Febiger, 1978:631. 14. Camisa C, Sharma HM. VesiculobuUous systemic lupus erythematosus. J AM ACADDERMATOL1983;9:924-33. 15. Gammon WR, Briggaman RA, Inman AO HI, Queen LL, Wheeler CE. Differentiating anti-lamina lucida and anti-sublamina densa anti-BMZ antibodies by indirect immunofluorescence on 1.0 M sodium chloride-separated skin. J Invest Dermatol 1984;82:139-44. 16. Penneys NS, WileyHE. Herpetiform blisters in systemic lupus erythematosus. Arch Dermatoi 1979; 115:1427-8. 17. Hall RP, LawleyTJ, Smith HR, Katz SL Bullous eruption of systemic lupus erythematosus. Dramatic response to dapsone therapy. Ann Interm Med 1982;97:165-70. 18. Gammon WR, Woodley DT, Dole KC, Bdggaman RA. Evidence that antibasement membrane zone antibodies in bullous eruption of systemic lupus erythematosus recognize epidermolysis bullosa acquisita autoantigen. J Invest Dermatol 1985;84:472-6. 19. Barton DD, Fine J-D, Gammon WR, Sams WM Jr. Bullous systemic lupus erythematosus: an unusual clinical course and detectable circulating autoantibodies to the epidermolysis bullosa aequisita antigen. J AM ACAoDERMATOL1986;15:369-73. 20. Camisa C, Grimwood RE. Indirect immunofluorescenee in vesiculobullous eruption of systemic lupus erythematosus. J Invest Dermatol 1986;86:606. 21. Morel P, Brouet J-C, Anxionnaz S, Mazel C, Civatte J. Lupus 6ryth~mateux syst6mique avec bulles. Ann Dermatol Venereol 1982;109:791-2. 22. Crick XB, Levet R, Blechet-Butaye F, Vissuzaine C, Belaich S. Lupus 6ryth6mateux syst6mique avec 16sions bulleuses. Ann Dermatol Venereol 1983;110:455-9. 23. Saurat J-H, Chavaz P, Didierjean L, Schaer B. Bullous eruption of systemic lupus erythematosus: "Herpetiform pemphigus" [letter]. J AM AC_ADDEaMATOL1983;9:163. 24. Tani M, Shimizu R, Ban M, Murata Y, Tamaki A. Systemic lupus erythematosus with vesiculobullous lesions: Immunoelectron microscopic studies. Arch Dermatol 1984; 120:1497-1501. 25. Aboobaker J, Ramsaroop R, Abramowitz I, Naran AD. Bullous systemic erythematosus. A case report. S Aft Med J 1986;69:49-51. 26. Miller JA, Dowd PM, Dudeney C, Isenberg DA. Vesiculobullous eruption in systemic lupus erythematosus:
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demonstration of common anti-DNA antibody idiotype at the detmo-epidcrmal junction. J R So<: Med 1986; 79:365-7. 27. Provost "IT. Lupus band test. Jut J Dermatol 1981; 20:475-8I. 28. Jacobs MI, Schned ES, Bystryn J-C. Variability of the lupus band test. Results in 18 patients with systemiclupus erythematosus. Arch Dermatol 1983;119:883-9. 29. Smith CD, Marino C, Rothfield NF. The clinical utility of the lupus band test. Arthritis Rheum 1984;27:382-7.
30. Alarcon GS, Sams WM, Barton DD, Reveille J. Bullous lupus erythematosus rash worsened by dapsone [letter]. Arthritis Rheum 1984;27:1071-2. 31. Davies MG, Marks R, Waddington E. Simultaneous sys, temie lupus erythematosus and dermatitis herpetiformis. Arch Dermatol 1976;112" 1292-4. 32. Moncada B. Dermatitis herpetiformis in association with systemic lupus erythematosus. Arch Dermatol 1974;109: 723-5.
ABSTRACTS
Reversal of advanced m u r i n e lupus in NZB/NZW F1 mice by treatment with monodonal antibody to L3T4
Squamous cell carcinoma of the skin in black patients with discoid lupus erythematosus
Wofsy D, Seaman WE. J Immunol 1987;138:3247-53
Caruso WR, Stewart ML, Nanda VK, et al. J Rheumatol 1987;14:156-9
Successful preventative therapy of lupus erythematosus is described in regard to the murine disease. Can this be done for the human form of lupus erythematosus? Philip C. Anderson, M.D.
Black persons with intensely pigmented skin seldom have squamous cell carcinoma. However, be watchful of their skin lesions of discoid lupus erythematosus becoming keratinizing cancer, as reported here in seven cases. Philip C. Anderson, M.D.
Protection of guinea pigs from primary a n d recurrent herpes simplex virus (HSV) type 2 cutaneous disease with vaecinia virus recombinants expressing HSV glycoproteln D
Psoriatic arthritis: clinical subgroups and histocompatibility antigens
Wachsman M, Aurelian L, Smith CC, et al. J Infect Dis 1987;155:1188-97
McHugh NJ, Laurent MR, Treadwell BL, ct al. Ann Rheum Dis 1987;46:184-8
To build a better herpes vaccine one uses the basic chassis of the smallpox virus and then adds some custom features of herpesvirus. When glycoprotein D from herpesvirus is expressed, the resulting vaccine protects young guinea pigs well. Progress on a vaccine for humans is slew but determined. Philip C. Anderson, M.D.
Sixty patients were studied. Remember, only the patients with spendylitishave a remarkable incidence of the B27 antigen. Antigen DR7 was associated with severe chronic peripheral arthritis.Neither of these markers is pathognomonic alone. Philip C. Anderson, M.D.
Traumatic arteriovenous fistula of the scalp. Case report
naevi may be related to metabolic activity
Variations in S-100 protein expression in naevocellular
Badejo L, Rockwood P, J Neurosurg 1987;66:773-4 This vascular fistula was induced by a air gun pellet. These interesting lesions are cured best by excision. Philip C. Anderson, M.D. Vitamin A and D status of black South African women and their babies Faimey A, S]oan MA, Patel KV, et al. Hum Nutr Clin Nutr 1987;41:81-7 Persons living in a poor cultural economy and these who traditionally suffer from poor nutrition often have a high rate of childhood mortality. They have many other medical problems. One reliable index to this deficiencyis the low serum retJnoi values. On the other hand, these people usually have normal vitamin D status because of actinic synthesis in skin+ Philip C. Anderson, M.D.
Paul E, Wen DR, Cochran AJ. Br J Dermatol 1987;116:371-8 The S-100 proteins on nevus cells are not purified. They can be detected by chemical tags, We do not know much about the biochemistry. Staining is extremelyvariable and unexplained. Giantcells and repairing nevi stain strongly for S-100, while more inert nevus cells may not stain at all. Increased metabolic activity seems to cause expression of the S-100 proteins. The utility of this stain for clinical decisions is being decided. Philip C. Anderson, M.D, Spontaneous passage of renal calculi through nephrocutaneous fistula due to calculous pyelonephritis Iseki T, Kawamura M. Br J Urol 1987;59:285-6 In the world of fistulas, almost anything can happen. "Kidney to skin" fistulas are rare, and the passage of renal stones is amazing. Philip C. Anderson, M.D.