Clinical and Experimental Dermatology 1992; 17: 354-356.
Bullous and haemorrhagic lichen sclerosus with scalp involvement P..MARREN. n . D E BF.RKER, P.MILLARD* AND F.WOJNAROWSKA Departments of Dermatohf^y and *Histopathitlogj, The John Radcliffe and The Slade Hospitals, O.vford Accepted for publication 4 November 1991
Summary We describe a patient who developed a generalized blistering eruption due to lichen sclerosus and who was observed to have scalp involvement. Both are unusual manifestations of this di.sease which merit consideration. Lichen sclernsus is an uncommon disease that most frequently affects the external genitalia of perimenopausal women.' 'I'he aetiology is unknown. Approximately 20"o of affected patients have extragenital lesions-' that present as small, ivory, shiny round macules or papules that later become atrophic; extragenital lesions are generally asymptomatic. Bullous and haemorrhajjic forms may occur but these are generally loealized and reports of extensive or generalized involvement are rare.* ** We describe an elderly woman with generalized bullous lichen sclerosus. As an incidental finding, she was observed to have lichen sclerosus affecting her scalp. This has rarely been described and it would appear that she is the third reported case of scalp involvement.
Case report A healthy 82-year-old lady with pruritus vulvae was referred to the skin clinic by a gynaecologist. He diagnosed multiple skin problems including a vulval dystrophy, vitiligo and possibly scabies. She gave a 2-year history of generalized pruritus and pruritus vulvae had been troublesome for one year. On examination she had generalized lichen sclerosus with multiple round and oval patches of white shiny parchment-like skin affecting her limbs and trunk (Fig. 1). There was typical vulval lichen sclenwus and this extended to the perianal skin. There were no bullac. The diagnosis was confirmed histnlogically and the pruritus vulvae was suppressed by nightly applications of lopical clt)betasol propionate for 3 months. She discontinued all treatment at that time. No comment was made regarding tbe condition of ber scalp at presentation. Correspondrnce: Dr P.Marren, I!>epannient of Derinatolog)'. The Sbdc 1 Iwpital. I ludington, Oxford OX3 7JH, UK.
354
Figure I. Oval and round macules of white shiny atrophit skit) of upper back.
She returned 1 year later with a 4-day history of a blistering eruption which had gradually become generalized (Fig. 2). Some of these blisters were .small and intact and were surrounded by purpura (Fig. 3). Others were larger and overlapped to produce extensive superficial ulcerations. .Many lesions bad become necrotic and secondarily infected. Tbe oral mucosa was normal. Her bair was noted to be sparse and fine textured but was not involved in tbe generalized blistering. Sbe had used no topical or systemic therapy during tbe previous year. 'I'be following investigations were normal or unremarkable: full blood count, ESR, pla.sma urea, creatinine, electrolytes, and liver function tests. Sbe had positive gastric parietal cell antibodies 11160 and her serum Bj^ was low 6.S ng/l (normal range 150-750) consistent witb a diagnosis of pernicious anaemia. The remainder of ber aulo-antibody .screen was negative. Skin swabs grew Staphylococcus aureus.
Histology of intact skin showed the typical features of licben sclerosus with a flattened epidermis, and a clear homogenized area within the papillary dermis associated with a moderate inflammatory infiltrate. There was some basal-cell vacuolation more typieal of bullous licben sclerosus. In some areas tbere was early separation ofthe
RUI.LOUS AND HAEMORRHAGIC LICHEN SCLEROSUS
355
Ml'
Figure 4. Histology ofan intact blister showed a subepidermal bulla cuntaining numerous red cells, fibriti and inflammatory cells.
4
Figure 2. Kxiensiic purpurj and ilat haemorrhage heralded the onset of a generalized blistering eruption.
epidermis and dermis. Riopsy ofan intact blister (Fig. 4) revealed a subepidermal bulla with some epidermal regeneration. There were numerous red cells within the blister together with fibrin and inflammatory cells. Direct immunofluorescence and indirect immunofluorescence on intact and split skin was negative. She was admitted and treated with systemic antibiotics and topical ciobetasol propionate. Her pernicious anaemia was treated with parenteral B|2 injections. The
Figured. C^nst-up of purpuric area in Figure 2 (arrow intact blister formation.
blistered and necrotic areas gradually healed and she has remained blister-free 9 months after discharge. Six months after discharge, at routine follow-up, she complained for tbe first time ofan intensely itcby scalp. Clinical examination revealed diffuse alopecia with a focal area of atropby and shiny pallor over tbe occiput. A biopsy ofthe scalp revealed basket-weave byperkeratosis with basal layer degeneration and occasional apoptotic cells consistent with a diagnosis of lichen sclerosus. The underlying inflammatory infiltrate, bowever, was sparse and, whilst tbis is unusual, it is a well deseribed histological variant.''"' Discussion Lichen sctcrosus presenting as a bullous eruption was first described in 1936 by Myers" and Schubert.'' In 1944 Anderson published a further case" and discussed the relationship of tbis entity to scleroderma. He clearly felt that the two conditions were distinct and although .some controversy persists, it is generally accepted that many lesions thought in tbe past to be *builous morpboea' are in facr a bullous form of Iieben sclerosus.^ Generalized haemorrhagic blistering in lichen sclerosus however is uncommon. Tbere are only a handful of similar cases in the literature.^ " Other reports describe the condition in perimenopausal females (50-68 years) and our patient's presentation at the age of 82 years is unique. There is a single report of an extensive bullous eruption in a 64year-old man. All the patients described had extensive ano-genital involvement in addition to extragenital lesions. In the earlier reports. Vitamin A and K preparations, topical and systemic conjugated oestrogens, and chloroquine therapy bave been recommended bur these were of doubtful value. More recently topical or intralesionai steroids have been shown to suppress blister formation in some. Systemic corticosteroids are not
356
P.MARREN et al.
recommended but there is a single report describing the beneficial effects of ACTH. There are no reports suggesting a beneficial response of this clinical variant to retinoids Our patient, was found coincidentally to have pernicious anaemia and the link between autoimmunity and hchen sclerosus is well established.^ The largest study investigating 300 women with lichen sclerosus found that just 2% of affected patients have pernicious anaemia, although 9-5% have positive anti-gastric parietal cell antibodies. This study also found that there were no significant differences in the natural history of the condition in those patients with and without autoimmune phenomena so the role, if any, of autoimmunity in the pathogenesis of lichen sclerosus remains unknown. From a histological standpoint many authors have expressed surprise that bullous and haemorrhagic elements are not more frequent. Oedema is a prominent feature in the papillary dermis. Blood vessels extend into the upper cutis and end freely there, where there is a lack of connective tissue support. These factors could account for the haemorrhage. Blistering is the result of vacuolar degeneration of the basal layer. In common with other reports, our patient's lesions were particularly prominent on her back and flexures so perhaps pressure and occlusion are contributory factors. Finally scalp involvement is exceedingly uncommon.^ The original case was described in the English literature in 1980''* and consisted of an extensive hyperkeratotic lesion affecting 80% of the scalp in a patient with generalized lichen sclerosus. Since then there has been just one reported case of cicatricial alopecia in a patient with lichen sclerosus and unusual extracutaneous manifestations.'^ In conclusion, this lady with widespread lichen sclerosus presented with a generalized blistering eruption wbich has rarely been described. She is particularly
unusual because of her age at presentation, the severity and extent of her blistering eruption, and the finding of lichen sclerosus of the scalp. References 1. Ridley CM. Dermatoses ofthe vulva. In: Rook A. consulting ed. The Vulva, vol 5 in series Major Problems in Dermatology. London: WB Saunders & Co Ltd, 1975; 172-179. 2. Ridley CM, Ridley CM ed. The Vulva; London: Churchill Livingstone, 1988; 172-188. 3. Meyrick Thomas RH, Ridley CM, McGibbon DH, Black MM, Lichen sclerosus et atrophicus and autoimmunity. British Journal of Dermatology 1988; 118: 41-46. 4. Gottschalk HR, Cooper ZK. Lichen sclerosus et atrophicus with bullous lesions and extensive involvement. Archives of Dermatology and Syphilis 1947; 55: 433, 5. Glickman FS, Silvers SH. Generalised bullous and hemorrhagic lichen sclerosus et atrophicus. Cutis 1972; 10: 501-503. 6. Di Silverio A, Serri F. Generalised bullous and haemorrhagic lichen sclerosus et atrophicus. British Journal of Dermatology 1975; 93: 215-217. 7. Brodey A. Society Transactions. Archives of Dermatology 1967; 95: 328-329. 8. DeFeo CP. Bullous lichen sclerosus et atrophicus vs scleroderma. Archives of Dermatology 1967; 97: 238-239. 9. Lever EF. Histopathology of the skin, 6th edn. Philadelphia: JB Lippincott Company, 1983: 252. 10. Montgomery H. Dermatopathology, Vol. 2. London: Hoebner Medical Division. 1967: 754, 11. Myers WK, Lichen Sclerosus case report. British Journal of Dermatology 1936; 48: 658. 12. Schubert M. Zur kenntnis des lichen sclerosus atrophicans. Derm. IVschr. 1936; 103: 1653. 13. Anderson CR, Bullous lichen sclerosus et atrophicans, it's relation to bullous scleroderma. Archives of Dermatology and Syphilis 1944; 49: 423. 14. Foulds IS. Lichen sclerosus et atrophicus ofthe scalp. British Journal of Dermatology 1980. 103: 197-200. 15. Dalziel K, Reynolds AJ, Holt PJA. Lichen Sclerosus et atrophicus with ocular and maxillary complications. British Journal of Dermatology. 1987. 116: 735-737.
Bullous and haemorrhagic lichen sclerosus with scalp involvement P..MARREN. n . D E BF.RKER, P.MILLARD* AND F.WOJNAROWSKA Departments of Dermatohf^y and *Histopathitlogj, The John Radcliffe and The Slade Hospitals, O.vford Accepted for publication 4 November 1991
Summary We describe a patient who developed a generalized blistering eruption due to lichen sclerosus and who was observed to have scalp involvement. Both are unusual manifestations of this di.sease which merit consideration. Lichen sclernsus is an uncommon disease that most frequently affects the external genitalia of perimenopausal women.' 'I'he aetiology is unknown. Approximately 20"o of affected patients have extragenital lesions-' that present as small, ivory, shiny round macules or papules that later become atrophic; extragenital lesions are generally asymptomatic. Bullous and haemorrhajjic forms may occur but these are generally loealized and reports of extensive or generalized involvement are rare.* ** We describe an elderly woman with generalized bullous lichen sclerosus. As an incidental finding, she was observed to have lichen sclerosus affecting her scalp. This has rarely been described and it would appear that she is the third reported case of scalp involvement.
Case report A healthy 82-year-old lady with pruritus vulvae was referred to the skin clinic by a gynaecologist. He diagnosed multiple skin problems including a vulval dystrophy, vitiligo and possibly scabies. She gave a 2-year history of generalized pruritus and pruritus vulvae had been troublesome for one year. On examination she had generalized lichen sclerosus with multiple round and oval patches of white shiny parchment-like skin affecting her limbs and trunk (Fig. 1). There was typical vulval lichen sclenwus and this extended to the perianal skin. There were no bullac. The diagnosis was confirmed histnlogically and the pruritus vulvae was suppressed by nightly applications of lopical clt)betasol propionate for 3 months. She discontinued all treatment at that time. No comment was made regarding tbe condition of ber scalp at presentation. Correspondrnce: Dr P.Marren, I!>epannient of Derinatolog)'. The Sbdc 1 Iwpital. I ludington, Oxford OX3 7JH, UK.
354
Figure I. Oval and round macules of white shiny atrophit skit) of upper back.
She returned 1 year later with a 4-day history of a blistering eruption which had gradually become generalized (Fig. 2). Some of these blisters were .small and intact and were surrounded by purpura (Fig. 3). Others were larger and overlapped to produce extensive superficial ulcerations. .Many lesions bad become necrotic and secondarily infected. Tbe oral mucosa was normal. Her bair was noted to be sparse and fine textured but was not involved in tbe generalized blistering. Sbe had used no topical or systemic therapy during tbe previous year. 'I'be following investigations were normal or unremarkable: full blood count, ESR, pla.sma urea, creatinine, electrolytes, and liver function tests. Sbe had positive gastric parietal cell antibodies 11160 and her serum Bj^ was low 6.S ng/l (normal range 150-750) consistent witb a diagnosis of pernicious anaemia. The remainder of ber aulo-antibody .screen was negative. Skin swabs grew Staphylococcus aureus.
Histology of intact skin showed the typical features of licben sclerosus with a flattened epidermis, and a clear homogenized area within the papillary dermis associated with a moderate inflammatory infiltrate. There was some basal-cell vacuolation more typieal of bullous licben sclerosus. In some areas tbere was early separation ofthe
RUI.LOUS AND HAEMORRHAGIC LICHEN SCLEROSUS
355
Ml'
Figure 4. Histology ofan intact blister showed a subepidermal bulla cuntaining numerous red cells, fibriti and inflammatory cells.
4
Figure 2. Kxiensiic purpurj and ilat haemorrhage heralded the onset of a generalized blistering eruption.
epidermis and dermis. Riopsy ofan intact blister (Fig. 4) revealed a subepidermal bulla with some epidermal regeneration. There were numerous red cells within the blister together with fibrin and inflammatory cells. Direct immunofluorescence and indirect immunofluorescence on intact and split skin was negative. She was admitted and treated with systemic antibiotics and topical ciobetasol propionate. Her pernicious anaemia was treated with parenteral B|2 injections. The
Figured. C^nst-up of purpuric area in Figure 2 (arrow intact blister formation.
blistered and necrotic areas gradually healed and she has remained blister-free 9 months after discharge. Six months after discharge, at routine follow-up, she complained for tbe first time ofan intensely itcby scalp. Clinical examination revealed diffuse alopecia with a focal area of atropby and shiny pallor over tbe occiput. A biopsy ofthe scalp revealed basket-weave byperkeratosis with basal layer degeneration and occasional apoptotic cells consistent with a diagnosis of lichen sclerosus. The underlying inflammatory infiltrate, bowever, was sparse and, whilst tbis is unusual, it is a well deseribed histological variant.''"' Discussion Lichen sctcrosus presenting as a bullous eruption was first described in 1936 by Myers" and Schubert.'' In 1944 Anderson published a further case" and discussed the relationship of tbis entity to scleroderma. He clearly felt that the two conditions were distinct and although .some controversy persists, it is generally accepted that many lesions thought in tbe past to be *builous morpboea' are in facr a bullous form of Iieben sclerosus.^ Generalized haemorrhagic blistering in lichen sclerosus however is uncommon. Tbere are only a handful of similar cases in the literature.^ " Other reports describe the condition in perimenopausal females (50-68 years) and our patient's presentation at the age of 82 years is unique. There is a single report of an extensive bullous eruption in a 64year-old man. All the patients described had extensive ano-genital involvement in addition to extragenital lesions. In the earlier reports. Vitamin A and K preparations, topical and systemic conjugated oestrogens, and chloroquine therapy bave been recommended bur these were of doubtful value. More recently topical or intralesionai steroids have been shown to suppress blister formation in some. Systemic corticosteroids are not
356
P.MARREN et al.
recommended but there is a single report describing the beneficial effects of ACTH. There are no reports suggesting a beneficial response of this clinical variant to retinoids Our patient, was found coincidentally to have pernicious anaemia and the link between autoimmunity and hchen sclerosus is well established.^ The largest study investigating 300 women with lichen sclerosus found that just 2% of affected patients have pernicious anaemia, although 9-5% have positive anti-gastric parietal cell antibodies. This study also found that there were no significant differences in the natural history of the condition in those patients with and without autoimmune phenomena so the role, if any, of autoimmunity in the pathogenesis of lichen sclerosus remains unknown. From a histological standpoint many authors have expressed surprise that bullous and haemorrhagic elements are not more frequent. Oedema is a prominent feature in the papillary dermis. Blood vessels extend into the upper cutis and end freely there, where there is a lack of connective tissue support. These factors could account for the haemorrhage. Blistering is the result of vacuolar degeneration of the basal layer. In common with other reports, our patient's lesions were particularly prominent on her back and flexures so perhaps pressure and occlusion are contributory factors. Finally scalp involvement is exceedingly uncommon.^ The original case was described in the English literature in 1980''* and consisted of an extensive hyperkeratotic lesion affecting 80% of the scalp in a patient with generalized lichen sclerosus. Since then there has been just one reported case of cicatricial alopecia in a patient with lichen sclerosus and unusual extracutaneous manifestations.'^ In conclusion, this lady with widespread lichen sclerosus presented with a generalized blistering eruption wbich has rarely been described. She is particularly
unusual because of her age at presentation, the severity and extent of her blistering eruption, and the finding of lichen sclerosus of the scalp. References 1. Ridley CM. Dermatoses ofthe vulva. In: Rook A. consulting ed. The Vulva, vol 5 in series Major Problems in Dermatology. London: WB Saunders & Co Ltd, 1975; 172-179. 2. Ridley CM, Ridley CM ed. The Vulva; London: Churchill Livingstone, 1988; 172-188. 3. Meyrick Thomas RH, Ridley CM, McGibbon DH, Black MM, Lichen sclerosus et atrophicus and autoimmunity. British Journal of Dermatology 1988; 118: 41-46. 4. Gottschalk HR, Cooper ZK. Lichen sclerosus et atrophicus with bullous lesions and extensive involvement. Archives of Dermatology and Syphilis 1947; 55: 433, 5. Glickman FS, Silvers SH. Generalised bullous and hemorrhagic lichen sclerosus et atrophicus. Cutis 1972; 10: 501-503. 6. Di Silverio A, Serri F. Generalised bullous and haemorrhagic lichen sclerosus et atrophicus. British Journal of Dermatology 1975; 93: 215-217. 7. Brodey A. Society Transactions. Archives of Dermatology 1967; 95: 328-329. 8. DeFeo CP. Bullous lichen sclerosus et atrophicus vs scleroderma. Archives of Dermatology 1967; 97: 238-239. 9. Lever EF. Histopathology of the skin, 6th edn. Philadelphia: JB Lippincott Company, 1983: 252. 10. Montgomery H. Dermatopathology, Vol. 2. London: Hoebner Medical Division. 1967: 754, 11. Myers WK, Lichen Sclerosus case report. British Journal of Dermatology 1936; 48: 658. 12. Schubert M. Zur kenntnis des lichen sclerosus atrophicans. Derm. IVschr. 1936; 103: 1653. 13. Anderson CR, Bullous lichen sclerosus et atrophicans, it's relation to bullous scleroderma. Archives of Dermatology and Syphilis 1944; 49: 423. 14. Foulds IS. Lichen sclerosus et atrophicus ofthe scalp. British Journal of Dermatology 1980. 103: 197-200. 15. Dalziel K, Reynolds AJ, Holt PJA. Lichen Sclerosus et atrophicus with ocular and maxillary complications. British Journal of Dermatology. 1987. 116: 735-737.
