British journal of Dertiiatology (1992) 12b, 614-f)16.
Unilateral buUous pemphigoid in a hemiplegic patient C.C.LONG. L.R.LEVER AND R.MARKS Department of Dermatology. Universitu of Wales College of Medicine. Heath Park. Cardiff CF4 4XN. U. K.
Accepted for publication 9 January 1992
Summary
A 78-year-oId man with a long-standing left-sided hemiplegia presented with bulious pemphigoid which affected his paralysed side only. Although the rash was unilateral, direct immunofluorescence demonstrated IgG antibody at the dermo-epidermal junction on both sides of the body. Indirect immunofluorescence was also positive. A suction-blister test showed increased skin fragility on the affected side.
Case report A 78-year-old man presented to the dermatology department of the University Hospital of Wales with a 1-year history of a bulious rash affecting the left side of his scalp, left arm. and left leg (Fig. 1). He gave no history of oral lesions or of involvement of the right side of his body. Fourteen years previously he had suffered a cerebrovascular accident resulting in a persistent dense left-sided hemiplegia. On examination he had crusts on the left side of the scalp, and bullae arising on erythematous. urticated skin on the anterior surface of his left upper arm, left thigh, and the posterior aspect of his left shoulder. The skin on the right side of the body was normal. There were no oral lesions. He had a dense left-sided hemiplegia with absent sensation to pinprick and light touch. General examination was otherwise normal. Laboratory studies, including a full blood count, urea, electrolytes, liver function tests, and thyroid function tests were normal. A chest radiograph was normal apart from some pleural thickening at the right base. A biopsy of the rash showed a subepidermal blister with a moderate perivascular infiltrate in the upper dermis. Direct immunofiuorescence examination of perilesional skin showed IgG. C3. and fibrin deposited at the dermo-epidermal junction. An indirect immunofluorescence test on the patient's serum demonstrated a circulating antibasement membrane IgG antibody at a titre of I in 100. A diagnosis of bulious pemphigoid was made. Because of the unusual distribution of the eruption, biopsies of uninvolved skin were taken from the volar surface of both forearms for direct immunofluorescence examination. Both biopsies showed IgG deposited at the Correspondence: Dr C.CLong,
614
dermo-epidermal junction. To investigate the asymmetry of the blistering tendency suction caps were applied to the skin of the volar surface of both forearms and a negative pressure of 200 mmHg was applied. A confiuent blister developed on the left side within 21 min. There was no evidence of blister formation on the right side after 60 min of suction. He was initially treated with prednisolone 60 mg/day and later azathioprine 50 mg twice daily was added. The blistering was subsequently controlled with prednisolone 10 mg/day and azathioprine 50 mg b.d. although he continues to develop occasional new blisters.
Discussion Localiiied variants of bulious pemphigoid have been described and usually aflect the head and neck.' or the legs in women.- Bulious pemphigoid has also been reported as occurring at the sites of previous trauma including amputation stumps, surgical and traumatic scars,' and colostomy sites.'* Unilateral bulious pemphigoid occurring in a hemiplegic individual has not been reported previously, to our knowledge. We felt it unlikely that our patient was suffering from epidermolysis bullosa acquisita. In this condition the bullae usually arise on traumatized skin, whereas the rash In our patient predominantly affected the upper arm and anterior thigh and did not occur on pressure areas. In epidermolysis bullosa acquisita the lesions usually heal with atrophy, scarring and milia formation,'' a feature not seen in our patient. Various changes have been noted in the skin of patients suffering from hemiplegia as a result of cerebrovascular accidents.'^"'- A decrease in the minimum erythema dose of ultraviolet radiation, as well as a tendency to tan more readily on the hemiplegic side have
UNILATERAL BULLOUS PEMPHIGOID
Figure 1, Cnisled lesions due to healing bulious peniphigoid on left (bcmiplegici leg,
been reported.^ ^ Bernardi cl al.^ found decreased electrical impedence of the skin surface on the hemiplegic side in several patients. The differences became less marked following a course of physiotherapy. A decrease in the axillary temperature, unilateral finger clubbing, and oedema of the hemiplegic side have all been reported.''"'~ A decrease in the sebum excretion rate of the forehead on the affected side of patients with unilateral lower facial nerve palsies has been described,'' and similar changes have been noted on the thighs of paraplegic patients.'^ Decreased blood flow with a corresponding decrease in skin temperature, as well as decreased motor neurone conduction velocities on the affected side have also been Changes may occur in the distribution of some skin diseases in the presence of hemiplegia or a peripheral nerve injury. Unilateral livedo reticularis affecting only the hemiplegic side of the body has been described.'" Bettley and Marten''^ reported a case of seborrhoeic eczema occurring on the alTected side of the face following injury to the facial nerve. In some diseases the hemiplegic side of the body is spared. Troilius and Moller'" reported five cases of 'endogenous' eczema which mainly affected the normal, non-hemiplegic side. Thomsen'' reported a patient who developed Beau's lines on the fingernails of his nonparalysed side following an episode of generalized cxfoliativc dermatitis (which had affected both sides equally). Scleroderma sparing the hemiplegic side of the body has been described.•'' Rheumatoid arthritis, acute gout, and osteoarthritis have also been found to spare paretic limbs.-'-^" In our patient the rash remained confined to the hemipiegic side although direct immunofiuorescence demonstrated the deposition of IgG antibodies at the dermoepidermal junction on both sides of the body. A
615
circulating antibody to the basement membrane zone was also demonstrated. The tendency to blister was increased on the affected side, as demonstrated by the reduced suction blister time. A confiuent blister developed within 21 min on the left side, with no evidence of blister formation on the right side after 1 h. Normally a blister would not be expected to be seen before 90 min on normal skin. It has been suggested that the distribution of lesions in BP is dependent upon the distribution of the bulious pemphigoid antigen in the skin.-' Our finding that the patient had an IgG antibody along the basement membrane bilaterally, but reduced suction blister time on the affected side indicates that other factors are also important. The precise nature of the effect of the hemiplegia on the skin which led to the clinical expression of BP in this patient is unknown, but may relate to decreased cutaneous blood fiow and altered autonomic nervous function on the paralysed side.
References 1 Bruristing i ^ , Terry HO, Benign pempbigoid,- A report of seven cases with chronic scarring, herpeliform plaques about the head and neck. Arch Dermatol 1957: 75: 4H9-5()1. 2 Person |R, Rogers RS MI, Perry HO. l,()calized pemphigoid. Br / Devmtno! I97(i;95: 5 51-4, ! .Macfarlane AW, Verbov jL, Trauma-induced bulious pemphigoid. Clin l-xp Dermatol I 489: 14: 245-9, 4 Siilomon R), Briggaman RA, WernikoffSV, Kayne AL, l,ocalized bulltius pemphigoid. Arch Dermatol 1987: 121: i89-92, 5 Braun-Palco O, Plewig G. Wolff HH, Winklemann RK, Vesicular and bulious diseases. In: Dermatoloiiif. Berlin: Spritiger-Verlag. 1991:474, (S Cox NH, Williams S|, Lxjwered ultraviolet minimal erytbema dose in hemiplegia. Postgrad Med j 1985: 61: 575-7, 7 Cox NH, Asymmetrical tanning in hemiplegia. Practitioner 1984: 228: ll()f)-7, 8 Bernardi I., Ferrera M, Bazzini G. L'impedeiiza elettrica cutanea in soggetti emiplcgica. Boll Soc Ital Biol Sper 1982: 58: 1425-31, 9 Mulley G. Axillary temperature differences in hemipiegia. Postgrad Med I 1 980: 56: 248-9, H) Alveraz AS, McNair I), WildmanJ.HewsonlW, Unilateral clubbing of the tingcrnails In patients with hemiplegia, Geromo! Clm ] 975: 17: 1-6, 1 1 Denham M|, Hodkinson HM, Wright BM. Unilateral clubbing in hemiplegia. C.erontol Clin I97S: 17: 7-12, 12 Exton-Smitb AN, Crockett D], Nature of oedema In paralysed limbs of hemiplegic patients, Br Med } 1957: 2: 1280-3. 1 } Burton \L. Cunliffe W|, Saunders IGG. Shuster S, The effect of facial nerve paresis on sebum excretion, Hr j Dermatol 1971:84: 1 J5-8. ! 4 Thomas SIi, Conway ]. Ebting l'\G. Harrington Cl, Measurement of sebum excretion rate and skin temperature above and below the neurological lesion In paraplegic patients. Hr / Dermato! 1985; 112: 569-7J, 15 Adams WC, Imms h'\. Resting bkK)d flow in the paretic and nonparetic lower legs of hemiplegic persons: relation to local skin temperature. Arch Phys Med Rehabil 198 J: 64: 423-8,
616
C.C.LONG et al
16 Takebe K. Narayan M("l. Kukulka C. Busmajian ]V. Slowing of nerve conduction velocity in hemiplegia: possible factors. Arch Phys Med Rehabil 1975; 56: 285-9, 17 Chokroverty S. Medina J. Electrophysiological study of hemiplegia. Motor nerve conduction velocity, brachia! plexus latency and electromyography. Arch Neurol 1978: 35: 360-3. 18 Bork K, Korting C,W. Vasculitis racemosa hemiplegica (Ger) (Eng Abstr), Dermatohgica 1979; 158: 275-8, 19 Bettley FR. Marten RH, Unilateral seborrheic dermatitis following a nerve lesion, Areh Dermatol 1956; 73: 110-15. 20 Troilius A. Moller H, Unilateral eruption of endogenous eczema after hemiparesis, Acta Derm Venereol iStockh) 1989; 69: 256-8. 21 Thomsen K. Unilateral skin conditions after hemiparesis. Acta Derm Venereol (Storkh) 1989: 69: 544,
22 Sethi S, Sequeira W. Sparing effect of hemipiegia on scleroderma. Ann Rheum /)isl99(): 49: 999-1000, 2 3 Thomason M, Bywaters RC, Unilateral rheumatoid arthritis following hemiplegia, Ann Rheum Dis 1962; 2 1 : 370-7. 24 Click EN, Asymmetrical rheumatoid arthritis after poliomyelitis. BrMedl 1967: ii: 26-8, 25 Glyn JJ. Clayton ML, Sparing effect of hemiplegia on tophaceous gout. Ann Rheum Dis 1976; 35: 534-5, 26 Winter S, Unilateral Heberden's nodes in a case of hemiplegia. NY State I Merf 1952; 52; 349-50. 27 Goldberg DJ, Sabolinski M, Bystyrn J-C, Regional variation in the expression of bulious pemphigoid antigen and location of lesions in bulious pemphigoid, / Invest Dermatol 1984: 82: J26-8.
Unilateral buUous pemphigoid in a hemiplegic patient C.C.LONG. L.R.LEVER AND R.MARKS Department of Dermatology. Universitu of Wales College of Medicine. Heath Park. Cardiff CF4 4XN. U. K.
Accepted for publication 9 January 1992
Summary
A 78-year-oId man with a long-standing left-sided hemiplegia presented with bulious pemphigoid which affected his paralysed side only. Although the rash was unilateral, direct immunofluorescence demonstrated IgG antibody at the dermo-epidermal junction on both sides of the body. Indirect immunofluorescence was also positive. A suction-blister test showed increased skin fragility on the affected side.
Case report A 78-year-old man presented to the dermatology department of the University Hospital of Wales with a 1-year history of a bulious rash affecting the left side of his scalp, left arm. and left leg (Fig. 1). He gave no history of oral lesions or of involvement of the right side of his body. Fourteen years previously he had suffered a cerebrovascular accident resulting in a persistent dense left-sided hemiplegia. On examination he had crusts on the left side of the scalp, and bullae arising on erythematous. urticated skin on the anterior surface of his left upper arm, left thigh, and the posterior aspect of his left shoulder. The skin on the right side of the body was normal. There were no oral lesions. He had a dense left-sided hemiplegia with absent sensation to pinprick and light touch. General examination was otherwise normal. Laboratory studies, including a full blood count, urea, electrolytes, liver function tests, and thyroid function tests were normal. A chest radiograph was normal apart from some pleural thickening at the right base. A biopsy of the rash showed a subepidermal blister with a moderate perivascular infiltrate in the upper dermis. Direct immunofiuorescence examination of perilesional skin showed IgG. C3. and fibrin deposited at the dermo-epidermal junction. An indirect immunofluorescence test on the patient's serum demonstrated a circulating antibasement membrane IgG antibody at a titre of I in 100. A diagnosis of bulious pemphigoid was made. Because of the unusual distribution of the eruption, biopsies of uninvolved skin were taken from the volar surface of both forearms for direct immunofluorescence examination. Both biopsies showed IgG deposited at the Correspondence: Dr C.CLong,
614
dermo-epidermal junction. To investigate the asymmetry of the blistering tendency suction caps were applied to the skin of the volar surface of both forearms and a negative pressure of 200 mmHg was applied. A confiuent blister developed on the left side within 21 min. There was no evidence of blister formation on the right side after 60 min of suction. He was initially treated with prednisolone 60 mg/day and later azathioprine 50 mg twice daily was added. The blistering was subsequently controlled with prednisolone 10 mg/day and azathioprine 50 mg b.d. although he continues to develop occasional new blisters.
Discussion Localiiied variants of bulious pemphigoid have been described and usually aflect the head and neck.' or the legs in women.- Bulious pemphigoid has also been reported as occurring at the sites of previous trauma including amputation stumps, surgical and traumatic scars,' and colostomy sites.'* Unilateral bulious pemphigoid occurring in a hemiplegic individual has not been reported previously, to our knowledge. We felt it unlikely that our patient was suffering from epidermolysis bullosa acquisita. In this condition the bullae usually arise on traumatized skin, whereas the rash In our patient predominantly affected the upper arm and anterior thigh and did not occur on pressure areas. In epidermolysis bullosa acquisita the lesions usually heal with atrophy, scarring and milia formation,'' a feature not seen in our patient. Various changes have been noted in the skin of patients suffering from hemiplegia as a result of cerebrovascular accidents.'^"'- A decrease in the minimum erythema dose of ultraviolet radiation, as well as a tendency to tan more readily on the hemiplegic side have
UNILATERAL BULLOUS PEMPHIGOID
Figure 1, Cnisled lesions due to healing bulious peniphigoid on left (bcmiplegici leg,
been reported.^ ^ Bernardi cl al.^ found decreased electrical impedence of the skin surface on the hemiplegic side in several patients. The differences became less marked following a course of physiotherapy. A decrease in the axillary temperature, unilateral finger clubbing, and oedema of the hemiplegic side have all been reported.''"'~ A decrease in the sebum excretion rate of the forehead on the affected side of patients with unilateral lower facial nerve palsies has been described,'' and similar changes have been noted on the thighs of paraplegic patients.'^ Decreased blood flow with a corresponding decrease in skin temperature, as well as decreased motor neurone conduction velocities on the affected side have also been Changes may occur in the distribution of some skin diseases in the presence of hemiplegia or a peripheral nerve injury. Unilateral livedo reticularis affecting only the hemiplegic side of the body has been described.'" Bettley and Marten''^ reported a case of seborrhoeic eczema occurring on the alTected side of the face following injury to the facial nerve. In some diseases the hemiplegic side of the body is spared. Troilius and Moller'" reported five cases of 'endogenous' eczema which mainly affected the normal, non-hemiplegic side. Thomsen'' reported a patient who developed Beau's lines on the fingernails of his nonparalysed side following an episode of generalized cxfoliativc dermatitis (which had affected both sides equally). Scleroderma sparing the hemiplegic side of the body has been described.•'' Rheumatoid arthritis, acute gout, and osteoarthritis have also been found to spare paretic limbs.-'-^" In our patient the rash remained confined to the hemipiegic side although direct immunofiuorescence demonstrated the deposition of IgG antibodies at the dermoepidermal junction on both sides of the body. A
615
circulating antibody to the basement membrane zone was also demonstrated. The tendency to blister was increased on the affected side, as demonstrated by the reduced suction blister time. A confiuent blister developed within 21 min on the left side, with no evidence of blister formation on the right side after 1 h. Normally a blister would not be expected to be seen before 90 min on normal skin. It has been suggested that the distribution of lesions in BP is dependent upon the distribution of the bulious pemphigoid antigen in the skin.-' Our finding that the patient had an IgG antibody along the basement membrane bilaterally, but reduced suction blister time on the affected side indicates that other factors are also important. The precise nature of the effect of the hemiplegia on the skin which led to the clinical expression of BP in this patient is unknown, but may relate to decreased cutaneous blood fiow and altered autonomic nervous function on the paralysed side.
References 1 Bruristing i ^ , Terry HO, Benign pempbigoid,- A report of seven cases with chronic scarring, herpeliform plaques about the head and neck. Arch Dermatol 1957: 75: 4H9-5()1. 2 Person |R, Rogers RS MI, Perry HO. l,()calized pemphigoid. Br / Devmtno! I97(i;95: 5 51-4, ! .Macfarlane AW, Verbov jL, Trauma-induced bulious pemphigoid. Clin l-xp Dermatol I 489: 14: 245-9, 4 Siilomon R), Briggaman RA, WernikoffSV, Kayne AL, l,ocalized bulltius pemphigoid. Arch Dermatol 1987: 121: i89-92, 5 Braun-Palco O, Plewig G. Wolff HH, Winklemann RK, Vesicular and bulious diseases. In: Dermatoloiiif. Berlin: Spritiger-Verlag. 1991:474, (S Cox NH, Williams S|, Lxjwered ultraviolet minimal erytbema dose in hemiplegia. Postgrad Med j 1985: 61: 575-7, 7 Cox NH, Asymmetrical tanning in hemiplegia. Practitioner 1984: 228: ll()f)-7, 8 Bernardi I., Ferrera M, Bazzini G. L'impedeiiza elettrica cutanea in soggetti emiplcgica. Boll Soc Ital Biol Sper 1982: 58: 1425-31, 9 Mulley G. Axillary temperature differences in hemipiegia. Postgrad Med I 1 980: 56: 248-9, H) Alveraz AS, McNair I), WildmanJ.HewsonlW, Unilateral clubbing of the tingcrnails In patients with hemiplegia, Geromo! Clm ] 975: 17: 1-6, 1 1 Denham M|, Hodkinson HM, Wright BM. Unilateral clubbing in hemiplegia. C.erontol Clin I97S: 17: 7-12, 12 Exton-Smitb AN, Crockett D], Nature of oedema In paralysed limbs of hemiplegic patients, Br Med } 1957: 2: 1280-3. 1 } Burton \L. Cunliffe W|, Saunders IGG. Shuster S, The effect of facial nerve paresis on sebum excretion, Hr j Dermatol 1971:84: 1 J5-8. ! 4 Thomas SIi, Conway ]. Ebting l'\G. Harrington Cl, Measurement of sebum excretion rate and skin temperature above and below the neurological lesion In paraplegic patients. Hr / Dermato! 1985; 112: 569-7J, 15 Adams WC, Imms h'\. Resting bkK)d flow in the paretic and nonparetic lower legs of hemiplegic persons: relation to local skin temperature. Arch Phys Med Rehabil 198 J: 64: 423-8,
616
C.C.LONG et al
16 Takebe K. Narayan M("l. Kukulka C. Busmajian ]V. Slowing of nerve conduction velocity in hemiplegia: possible factors. Arch Phys Med Rehabil 1975; 56: 285-9, 17 Chokroverty S. Medina J. Electrophysiological study of hemiplegia. Motor nerve conduction velocity, brachia! plexus latency and electromyography. Arch Neurol 1978: 35: 360-3. 18 Bork K, Korting C,W. Vasculitis racemosa hemiplegica (Ger) (Eng Abstr), Dermatohgica 1979; 158: 275-8, 19 Bettley FR. Marten RH, Unilateral seborrheic dermatitis following a nerve lesion, Areh Dermatol 1956; 73: 110-15. 20 Troilius A. Moller H, Unilateral eruption of endogenous eczema after hemiparesis, Acta Derm Venereol iStockh) 1989; 69: 256-8. 21 Thomsen K. Unilateral skin conditions after hemiparesis. Acta Derm Venereol (Storkh) 1989: 69: 544,
22 Sethi S, Sequeira W. Sparing effect of hemipiegia on scleroderma. Ann Rheum /)isl99(): 49: 999-1000, 2 3 Thomason M, Bywaters RC, Unilateral rheumatoid arthritis following hemiplegia, Ann Rheum Dis 1962; 2 1 : 370-7. 24 Click EN, Asymmetrical rheumatoid arthritis after poliomyelitis. BrMedl 1967: ii: 26-8, 25 Glyn JJ. Clayton ML, Sparing effect of hemiplegia on tophaceous gout. Ann Rheum Dis 1976; 35: 534-5, 26 Winter S, Unilateral Heberden's nodes in a case of hemiplegia. NY State I Merf 1952; 52; 349-50. 27 Goldberg DJ, Sabolinski M, Bystyrn J-C, Regional variation in the expression of bulious pemphigoid antigen and location of lesions in bulious pemphigoid, / Invest Dermatol 1984: 82: J26-8.
