Clinical and Experimental Dermatology 1992; 17: 182-185.
Unilateral linear eapillaritis C.A.RIORDAN, C.DARLEY*, A.C.MARKEYf, G . M U R P H Y : AND J.D.WILKINSON Department of Dermatology, Wycombe General Hospital, High Wycombe, ^Brighton General Hospital, Brighton, Sussex, t Department of Dermatology, Guy's Hospital, London and % Department of Dermatology, Beaumont Hospital, Dublin Accepted for publication \9 July 1991
tbe following 3 montbs be developed more pigmented maeules over tbe postero-lateral aspect of tbe rigbt We present four cases of a distinctive type of pigmented buttock, right tbigb and rigbt lower leg in a patcby linear purpuric eruption occurring in a striking linear and distribution (Fig. 2b). No predisposing factors or assopseudo-dermatomal distribution in young males. Whilst ciated features were present and be was otberwise well. tbese cases sbare some of tbe clinical and bistological Histology is not available as tbe patient refused biopsy. features of otber pigmented purpuric dermatoses., tbey are He was, bowever,, seen in consultation by a vascular not readily classified with any of tbe entities so far defined surgeon wbo felt tbere was no evidence of venous in tbis group of disorders. We believe tbese cases bypertension and no varicose veins or venous telangiectarepresent a distinct group not previously described. sia present in tbe leg. Tbe eruption reached maximal extent after 3 montbs. Four patients witb an unusual pigmented purpuric Ten montbs after tbe onset, the lesions became more eruption are presented. yellow-brown in colour and started to resolve spontaneously. He was last seen 14 montbs after tbe onset and tbe lesions had significantly regressed. Only a few pale Case reports brown patches remained on tbe skin. Summary
Case 1
A healtby, 13-year-old boy noted a pigmented area Case 3 bebind his left knee 18 montbs prior to presentation. Over An otberwise bealthy 38-year-old male developed brown the following 6-12 montbs tbis slowly extended to involve pigmented patcbes on tbe left buttock. Tbere was gradual tbe posterior aspect of tbe left buttock, left thigh and tbe extension of similar patches down tbe postero-lateral left lower leg. Tbe rash was completely asymptomatic and aspect of the left leg onto the left foot over a 12-month tbere were no predisposing factors or associated features. period. Examination revealed yellow-brown maeules in a Tbe eruption bad not changed in tbe 6 montbs immedi- patcby linear distribution over the postero-lateral aspect ately prior to presentation. Examination revealed a of the left leg and involving tbe left foot. Some scattered macular pigmented eruption wbicb extended from the punctate purpura was evident in tbe pigmented maeules. dorsum of tbe left foot up tbe posterior aspect ofthe left He noted a very sligbt itcb in some ofthe lesions. Tbere leg in a patchy linear distribution (Fig. la). Punctate were no predisposing factors or associated symptoms. purpuric lesions were scattered tbrougbout some of tbe Histology sbowed a moderate perivascular infiltrate pigmented maeules. Histology sbowed a mild eapillaritis confined to tbe dermis and composed of lympbocytes and witb occasional baemosiderin-laden macropbages present macropbages witbout leucocytociasis. A few extravasated in tbe dermis (Fig. lb). Tbirty montbs after tbe onset, tbe red blood cells were present togetber witb foci of baemosiderin. Tbe appearances were cbaracteristic of a eruption started to regress spontaneously. mild non-specific eapillaritis. On review 18 montbs after tbe onset, tbe eruption bad faded from tbe buttock area, Case 2 but was still present on tbe tbighs and lower legs. A 13-year-old boy developed an asymptomatic pigmented purpuric patcb on tbe rigbt foot (Fig. 2a). Over Case 4
Correspondence: Dr C.A.Riordan, Skin & Cancer Foundation, 277 Bourke Street, Darlinghurst 2010, Sydney, Australia.
182
A bealtby 25-year-old male presented with a 1-year bistory of a progressive pigmented eruption whicb commenced around tbe right ankle and spread to involve
UNILATERAL LINEAR CAPILLARITIS
183
Figure 1. (a) Macular pigmented eruption on tbe left leg of Patient 1. (b) Histology of lesion showing a mild eapillaritis.
Figure 2. (a) Asymptomatic pigmented purpuric patch on right foot of Patient 2. (b) Three months later, more pigmented maeules develop.
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Figure 3. Histology ol" Patient 4 showing low-grade capillaritis.
tbe popliteal fossa and rigbt buttock. Examination revealed pigmented maeules extending in a patcby linear distribution from tbe rigbt ankle up the postero-lateral aspect ofthe leg and onto tbe right buttock. The eruption was asymptomatic and tbere were no predisposing factors or associated features. Histology sbowed a low-grade capillaritis (Fig. 3). He was treated for a sbort time witb topical Dermovate but tbere was no clinical improvement. Wben reviewed 3 years after tbe onset of tbis eruption, there had been almost complete resolution of tbe pigmented eruption. Discussion Tbere arefiverecognized sub-groups whicb comprise the pigmented purpuric dermatoses. Tbey are, for the most part, distinguisbed on morpbological grounds as tbey all share a similar pathology, namely a benign chronic capillaritis. There is a male predominance in all of these conditions except in Majocchi's disease which is more common in females.'"^ The lesions in progressive pigmented purpuric dermatosis or Schamberg's Disease are irregular red-brown pigmented patches or maeules with satellite punctate purpura' described as 'cayenne pepper' spots. They are usually symmetrically distributed in the tibial region'' and the majority of cases are asymptomatic. The condition runs a chronic course, an average duration of 4-5 years being reported in one series.^ Purpura annularis telangiectoides or Majocchi's disease is seen in a slightly younger age group^ than that of Schamberg's disease, where the average age of onset is in the fifth decade. The individual lesions are annular maeules^ which are smaller and more uniform than those seen in Schamberg's disease. There is often mild atrophy and clearing in the centre ofthe lesions and telangiectasia frequently predominate.^ The site and symmetrical
distribution resembles Schamberg's disease but Majocchi's disease is almost always bilateral. Its course is sligbtly sborter, lasting montbs ratber tban years. In pigmented purpuric licbenoid dermatosis of Gougerot and Blum, the lesions are orange-red and purpuric lichenoid papules or plaques. Again the site and pattern of distribution are similar. Erythema, scaling and pruritus are often distinguishing features of this condition.*• The histology shows more marked epidermal involvement with basal liquefaction, spongiosis, byperkeratosis and parakeratosis often present.'' Tbe clinical course also is usually fairly chronic ranging from 2 months to 2 years in one series.' The individual lesions in itching purpura (eczematidelike purpura or disseminated pruriginous angiodermatitis) closely resemble those of Schamberg's disease as does the site and pattern ofthe eruption except that it may be more widespread and may involve the lower body.^ As the name suggests, pruritus is a prominent feature often from the outset. Scaling and epidermal change are often present clinically and histology reflects this. It usually runs a shorter course than the other pigmented purpuric dermatoses. Lichen aureus is a rare, localized^ variant of the pigmented purpuric dermatoses with a more distinctive histology, which some believe justify its separate classification. The essential pathology, however, like the other pigmented purpuric dermatoses is still that of a chronic capillaritis but it is characterized by a denser 'band-like' lymphocytic infiltrate in the dermis with a thin grenz zone of normal tissue separating the epidermis and the infiltrate.'' Golden brown or purple, discrete or confluent lichenoid papules or maeules occur most commonly over tbe lower limb. Tbe distribution is unilateral and localized in 93% of cases in one series.' Lichen aureus is slow to evolve and runs a very chronic course with complete and spontaneous resolution being seen only occasionally. Our cases share some of the features of the entities discussed. As with most ofthe other pigmented purpuric dermatoses there is a male predominance. Similarly, there do not appear to be any predisposing factors or associated features and the lesions are, for the most part, asymptomatic. Mild itch was reported by only one patient. Tbe average age of 22 years is similar to tbat seen in licben aureus wbich bas a peak incidence in the second and tbird decade and tbe lower limb distribution is a feature of all tbe pigmented purpuric dermatoses. Our cases, bowever, are unusual because of their extensive, unilateral, Unear patterns. A unilateral distribution has sometimes been reported in patients with lichen aureus, but the lesions are usually localized and not as extensive as in our cases. There have been three reported cases of segmental lichen aureus. In all three the lesions were quite localized and present on the upper trunk and upper
UNILATERAL LINEAR CAPILLARITIS limbs.^ The striking linear pattern in our cases is atypical for any ofthe groups of pigmented purpuric dermatoses but is perhaps more compatible witb licben aureus wbere linear and zosteriform patterns, wbilst uncommon, bave been described. Histology in our patients, however, was not tbat of licben aureus. Despite tbe striking clinical appearance, tbe bistology was relatively unremarkable sbowing only a mild patcby, predominantly perivascular, lymphocytic infiltrate with variable amounts of haemosiderosis consistent with capillaritis. In all our cases, spontaneous regression commenced approximately 2 years after the onset and this also represents a far shorter duration than most cases of lichen aureus. Our cases, therefore, seem to represent a distinct sub-group of pigmented purpuric dermatoses, namely unilateral linear capillaritis. Whilst the clinical picture of unilateral linear capillaritis is florid, the histology is comparatively banal and tbe prognosis appears quite favourable witb spontaneous regression commencing witbin 2 years of tbe onset. A number of dermatological entities are well known to occur in linear or zosteriform patterns. Tbe best recognized of these are lichen planus, lichen striatus, vitiligo.
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herpes zoster and Darier's disease. Pigmented purpuric dermatoses now need to be included in tbis list. References 1. Randall SJ, Kierland RR, Montgomery H. Pigmented purpuric eruptions. Archives of Dermatotogy and Syphil's 1951; 64: 177182. 2. Farrokhzad S, Champion RH. Pigmented purpuric dermatoses. Dermatologtca 1970; 140: 4.S-53, 3. Newton RC, Raimer SS. Pigmented purpurie eruptions. Dermatologie Cltmcs \9%S, i: 165-169, 4. Graham RM, English JS, Emmenson RW. Lichen aureus—a study of twelve cases. Clinicat and Experimental Dermatology 1984; 9: 39.3-401. 5. Rook A, Wilkinson DS, Ebling FJ et al. Textbook of Dermatology, 4th edn; Oxford: Blackwell Scientific Publications, 1116-1119. 6. Lever WF. Histology of the Skm, 6th Edn, pp, 17.V174. J.B. Lippino & Co., Philadelphia. 7. Price ML, Wilson-Jones E, Calnan CD, Macdonald DM. Lichen aureus: a localised persistent form of pigmented purpuric dermatitis, British Journal of Dermatotogy 1985; 112: 307-314. 8. Ruiz-Esmenjaud J, Dahl MV. Segmental lichen aureus: onset associated with trauma and puberty. Archives of Dermatology 1988; 124: 1572-1474.
Unilateral linear eapillaritis C.A.RIORDAN, C.DARLEY*, A.C.MARKEYf, G . M U R P H Y : AND J.D.WILKINSON Department of Dermatology, Wycombe General Hospital, High Wycombe, ^Brighton General Hospital, Brighton, Sussex, t Department of Dermatology, Guy's Hospital, London and % Department of Dermatology, Beaumont Hospital, Dublin Accepted for publication \9 July 1991
tbe following 3 montbs be developed more pigmented maeules over tbe postero-lateral aspect of tbe rigbt We present four cases of a distinctive type of pigmented buttock, right tbigb and rigbt lower leg in a patcby linear purpuric eruption occurring in a striking linear and distribution (Fig. 2b). No predisposing factors or assopseudo-dermatomal distribution in young males. Whilst ciated features were present and be was otberwise well. tbese cases sbare some of tbe clinical and bistological Histology is not available as tbe patient refused biopsy. features of otber pigmented purpuric dermatoses., tbey are He was, bowever,, seen in consultation by a vascular not readily classified with any of tbe entities so far defined surgeon wbo felt tbere was no evidence of venous in tbis group of disorders. We believe tbese cases bypertension and no varicose veins or venous telangiectarepresent a distinct group not previously described. sia present in tbe leg. Tbe eruption reached maximal extent after 3 montbs. Four patients witb an unusual pigmented purpuric Ten montbs after tbe onset, the lesions became more eruption are presented. yellow-brown in colour and started to resolve spontaneously. He was last seen 14 montbs after tbe onset and tbe lesions had significantly regressed. Only a few pale Case reports brown patches remained on tbe skin. Summary
Case 1
A healtby, 13-year-old boy noted a pigmented area Case 3 bebind his left knee 18 montbs prior to presentation. Over An otberwise bealthy 38-year-old male developed brown the following 6-12 montbs tbis slowly extended to involve pigmented patcbes on tbe left buttock. Tbere was gradual tbe posterior aspect of tbe left buttock, left thigh and tbe extension of similar patches down tbe postero-lateral left lower leg. Tbe rash was completely asymptomatic and aspect of the left leg onto the left foot over a 12-month tbere were no predisposing factors or associated features. period. Examination revealed yellow-brown maeules in a Tbe eruption bad not changed in tbe 6 montbs immedi- patcby linear distribution over the postero-lateral aspect ately prior to presentation. Examination revealed a of the left leg and involving tbe left foot. Some scattered macular pigmented eruption wbicb extended from the punctate purpura was evident in tbe pigmented maeules. dorsum of tbe left foot up tbe posterior aspect ofthe left He noted a very sligbt itcb in some ofthe lesions. Tbere leg in a patchy linear distribution (Fig. la). Punctate were no predisposing factors or associated symptoms. purpuric lesions were scattered tbrougbout some of tbe Histology sbowed a moderate perivascular infiltrate pigmented maeules. Histology sbowed a mild eapillaritis confined to tbe dermis and composed of lympbocytes and witb occasional baemosiderin-laden macropbages present macropbages witbout leucocytociasis. A few extravasated in tbe dermis (Fig. lb). Tbirty montbs after tbe onset, tbe red blood cells were present togetber witb foci of baemosiderin. Tbe appearances were cbaracteristic of a eruption started to regress spontaneously. mild non-specific eapillaritis. On review 18 montbs after tbe onset, tbe eruption bad faded from tbe buttock area, Case 2 but was still present on tbe tbighs and lower legs. A 13-year-old boy developed an asymptomatic pigmented purpuric patcb on tbe rigbt foot (Fig. 2a). Over Case 4
Correspondence: Dr C.A.Riordan, Skin & Cancer Foundation, 277 Bourke Street, Darlinghurst 2010, Sydney, Australia.
182
A bealtby 25-year-old male presented with a 1-year bistory of a progressive pigmented eruption whicb commenced around tbe right ankle and spread to involve
UNILATERAL LINEAR CAPILLARITIS
183
Figure 1. (a) Macular pigmented eruption on tbe left leg of Patient 1. (b) Histology of lesion showing a mild eapillaritis.
Figure 2. (a) Asymptomatic pigmented purpuric patch on right foot of Patient 2. (b) Three months later, more pigmented maeules develop.
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Figure 3. Histology ol" Patient 4 showing low-grade capillaritis.
tbe popliteal fossa and rigbt buttock. Examination revealed pigmented maeules extending in a patcby linear distribution from tbe rigbt ankle up the postero-lateral aspect ofthe leg and onto tbe right buttock. The eruption was asymptomatic and tbere were no predisposing factors or associated features. Histology sbowed a low-grade capillaritis (Fig. 3). He was treated for a sbort time witb topical Dermovate but tbere was no clinical improvement. Wben reviewed 3 years after tbe onset of tbis eruption, there had been almost complete resolution of tbe pigmented eruption. Discussion Tbere arefiverecognized sub-groups whicb comprise the pigmented purpuric dermatoses. Tbey are, for the most part, distinguisbed on morpbological grounds as tbey all share a similar pathology, namely a benign chronic capillaritis. There is a male predominance in all of these conditions except in Majocchi's disease which is more common in females.'"^ The lesions in progressive pigmented purpuric dermatosis or Schamberg's Disease are irregular red-brown pigmented patches or maeules with satellite punctate purpura' described as 'cayenne pepper' spots. They are usually symmetrically distributed in the tibial region'' and the majority of cases are asymptomatic. The condition runs a chronic course, an average duration of 4-5 years being reported in one series.^ Purpura annularis telangiectoides or Majocchi's disease is seen in a slightly younger age group^ than that of Schamberg's disease, where the average age of onset is in the fifth decade. The individual lesions are annular maeules^ which are smaller and more uniform than those seen in Schamberg's disease. There is often mild atrophy and clearing in the centre ofthe lesions and telangiectasia frequently predominate.^ The site and symmetrical
distribution resembles Schamberg's disease but Majocchi's disease is almost always bilateral. Its course is sligbtly sborter, lasting montbs ratber tban years. In pigmented purpuric licbenoid dermatosis of Gougerot and Blum, the lesions are orange-red and purpuric lichenoid papules or plaques. Again the site and pattern of distribution are similar. Erythema, scaling and pruritus are often distinguishing features of this condition.*• The histology shows more marked epidermal involvement with basal liquefaction, spongiosis, byperkeratosis and parakeratosis often present.'' Tbe clinical course also is usually fairly chronic ranging from 2 months to 2 years in one series.' The individual lesions in itching purpura (eczematidelike purpura or disseminated pruriginous angiodermatitis) closely resemble those of Schamberg's disease as does the site and pattern ofthe eruption except that it may be more widespread and may involve the lower body.^ As the name suggests, pruritus is a prominent feature often from the outset. Scaling and epidermal change are often present clinically and histology reflects this. It usually runs a shorter course than the other pigmented purpuric dermatoses. Lichen aureus is a rare, localized^ variant of the pigmented purpuric dermatoses with a more distinctive histology, which some believe justify its separate classification. The essential pathology, however, like the other pigmented purpuric dermatoses is still that of a chronic capillaritis but it is characterized by a denser 'band-like' lymphocytic infiltrate in the dermis with a thin grenz zone of normal tissue separating the epidermis and the infiltrate.'' Golden brown or purple, discrete or confluent lichenoid papules or maeules occur most commonly over tbe lower limb. Tbe distribution is unilateral and localized in 93% of cases in one series.' Lichen aureus is slow to evolve and runs a very chronic course with complete and spontaneous resolution being seen only occasionally. Our cases share some of the features of the entities discussed. As with most ofthe other pigmented purpuric dermatoses there is a male predominance. Similarly, there do not appear to be any predisposing factors or associated features and the lesions are, for the most part, asymptomatic. Mild itch was reported by only one patient. Tbe average age of 22 years is similar to tbat seen in licben aureus wbich bas a peak incidence in the second and tbird decade and tbe lower limb distribution is a feature of all tbe pigmented purpuric dermatoses. Our cases, bowever, are unusual because of their extensive, unilateral, Unear patterns. A unilateral distribution has sometimes been reported in patients with lichen aureus, but the lesions are usually localized and not as extensive as in our cases. There have been three reported cases of segmental lichen aureus. In all three the lesions were quite localized and present on the upper trunk and upper
UNILATERAL LINEAR CAPILLARITIS limbs.^ The striking linear pattern in our cases is atypical for any ofthe groups of pigmented purpuric dermatoses but is perhaps more compatible witb licben aureus wbere linear and zosteriform patterns, wbilst uncommon, bave been described. Histology in our patients, however, was not tbat of licben aureus. Despite tbe striking clinical appearance, tbe bistology was relatively unremarkable sbowing only a mild patcby, predominantly perivascular, lymphocytic infiltrate with variable amounts of haemosiderosis consistent with capillaritis. In all our cases, spontaneous regression commenced approximately 2 years after the onset and this also represents a far shorter duration than most cases of lichen aureus. Our cases, therefore, seem to represent a distinct sub-group of pigmented purpuric dermatoses, namely unilateral linear capillaritis. Whilst the clinical picture of unilateral linear capillaritis is florid, the histology is comparatively banal and tbe prognosis appears quite favourable witb spontaneous regression commencing witbin 2 years of tbe onset. A number of dermatological entities are well known to occur in linear or zosteriform patterns. Tbe best recognized of these are lichen planus, lichen striatus, vitiligo.
185
herpes zoster and Darier's disease. Pigmented purpuric dermatoses now need to be included in tbis list. References 1. Randall SJ, Kierland RR, Montgomery H. Pigmented purpuric eruptions. Archives of Dermatotogy and Syphil's 1951; 64: 177182. 2. Farrokhzad S, Champion RH. Pigmented purpuric dermatoses. Dermatologtca 1970; 140: 4.S-53, 3. Newton RC, Raimer SS. Pigmented purpurie eruptions. Dermatologie Cltmcs \9%S, i: 165-169, 4. Graham RM, English JS, Emmenson RW. Lichen aureus—a study of twelve cases. Clinicat and Experimental Dermatology 1984; 9: 39.3-401. 5. Rook A, Wilkinson DS, Ebling FJ et al. Textbook of Dermatology, 4th edn; Oxford: Blackwell Scientific Publications, 1116-1119. 6. Lever WF. Histology of the Skm, 6th Edn, pp, 17.V174. J.B. Lippino & Co., Philadelphia. 7. Price ML, Wilson-Jones E, Calnan CD, Macdonald DM. Lichen aureus: a localised persistent form of pigmented purpuric dermatitis, British Journal of Dermatotogy 1985; 112: 307-314. 8. Ruiz-Esmenjaud J, Dahl MV. Segmental lichen aureus: onset associated with trauma and puberty. Archives of Dermatology 1988; 124: 1572-1474.
