British Journal of Dermatology (1991) 124. 470-474.
ADONIS 0007096391001122
Chronic subcorneal pustulosis with vasculitis: a variant of generalized pustular psoriasis in black South Africans R.I.BARLOW AND E.J.SCHULZ* Medical llmversity of Southern Africa, Pretoria. South Africa 'University ofthe Witwatersrand. Johannesburg, South Africa Accepted for publication 3 December 1990
Summary
A rare, hut distinctive chronic eruption in six female hlack South Africans is reported. The original diagnosis of subcorneal pustular dermatosis of Sneddon and Wilkinson in these patients was refuted by the subsequent histological observation of both spongiform pustules and an underlying vasculitis. This may represent a previously undocumented form of generalized pustular psoriasis.
Pustular psoriasis may run an acute or chronic course with or without evidence of previous plaque psoriasis. Skin lesions may be localized, often to the hands and feet, or generalized. Annular forms of generalized pustular psoriasis tend to be subacute or chronic and clinically resemble subcorneal pustular dermatosis of Sneddon and Wilkinson.' A suhcorneal pustule occurs in both conditions but the spongiform pustule, characteristic of pustular psoriasis, is not found in suhcorneal pustular dermatosis.^ This is a retrospective study of a rare chronic pustular eruption seen in six black South Africans over the 12year period 1975-1989. The clinical features and initial histological findings suggested subcorneal pustular dermatosis. hut later histological findings included spongiform pustules and evidence of a vasculitis. We have been unable to find vasculitis documented In association with pustular psoriasis (i.e. with spongiform pustules), but it has been reported in patients with acute eruptions precipitated by infection and described as 'bacterids'.'"'' pustulosis acuta generalisata'*' and "acute acral pustulosis*.''
Case reports All six patients were black South Africans seen as outpatients in the Transvaal between 1975 and 1989. Clinical details are summarized in Table 1. All were Correspondence: Professor E.J.Schulz. Division of Dennatology. Area 553, Johannesburg Hospital and tJniversity of the Witwatersrand. Pdrktown, Johannesburg 2193. South Africa.
470
female and their ages ranged from 11 to S5 years. Two patients were related, being mother and daughter (Cases 3 and 4). and a third (Case 1) reported that her sister had a similar rash. The duration of the disease varied from 1 to 31 years and the age of onset from 4 to 29 (mean 18) years. Case 4 had never been free of lesions for more than a few weeks at a time and Case (S had been free of lesions only during periods of treatment with dapsone and prednisone. It is not known whether the rash was continuous or recurrent in the remaining four patients. The lesions were recorded as being itchy in two patients (Cases 2 and 4). not itchy in one (Case 1) and irritating in Case 5. In the remaining two (Cases 3 and 6) the presence or absence of symptoms was not recorded. No patient reported any associated fever, malaise, arthralgia or other systemic upset and all were otherwise healthy with no history of significant previous illnesses. The eruption affected the trunk and limbs in varying degrees. There was a tendency for involvement of the sides of the neck (Fig. 1) and trunk and the shins. The head, palms and soles were spared in each case. In all patients the skin lesions were at various stages of development. They were diffusely scattered without any grouped, annular or serpiginous arrangement. The primary (esion was a superficial rather flaccid off-white pustule. 2-4 mm in diameter set on a mildly erythematous base. The pustule dried to form a dark crust which separated to reveal a scaling rim around a small white depigmented and slightly atrophic scar. This, together with the surrounding skin, later became hyperpigmented. with some mild residual scaling in areas (Fig, 1). In two patients (Cases 2 and 5) secondary infection was
SUBCORNEAL PUSTULOSIS WITH VASCULITIS
471
Table t. Clinical and histological findings in patients with chronic subcorncal pustulosis Histoiogii-iil features Pattent 1
Sex/age (years)
Duration (years) 1
F/11
Epidermal
Distribution of rash Trunk, limbs especially shins
Three biopsies: (a) Long flat SCP with basal spongiform pustule and parakeratosis Mild spongiosis (b) Flat flaccid SPC Small spongiform pustule Little parakeratosis Mild spongiosis (c) Round tense intraepidermal SCP Small lateral spongiform pustule No parakeratosis Spongiosis + +
Dermal
Perivascular oedema + Mild vascuiitis Papillary otxlema + + RBC + -(- H- in papillae Vascuiitis Miltl papillary oedema Mild mixed perivascular inflltrate
2
F/25
2
Trunk, mainly lateral: proximal limbs
Tense dome-shaped SCP with PMN and cellular debris Large basal spongiform pustule and parakeratosis
Mild papillary' oedema l-'ew papillary RBC Mild mixed perivascular infiltrate
3
F/43
25
Sides of neck, axillae
long tense SCP with PMN and cellular debris Long basal spongiform pustule and parakeratosis
Marked papillary oedema Papillary RBC + + + Vascuiitis
4
F/15
11
Lower legs mainly shins, wrists
Tense round SCP with few PMN Small lateral spongiform pustule Very little parakeratosis
Mild papillary oedema Mild vascuiitis
5
F/31
2
Sides of neck, trunk. axillae, groins
Long tiaccid SCP Spongiform pustule and parakeratosis in base
Papillary oedema + Perivascutar RBC + 4^ Vascuiitis + +
F/55
31
Sides of neck, trunk. axillae, limbs, shins
Flaccid SCP Long ill-defined basal spongiform pustule No parakeratosis
Papillary oedema Papillary RBC Mild vascuiitis
SCP. subcomeai pustule: RBC. red blood cells: PMN. polymorphonuclear leucocytes.
present in some areas resulting in additional frankly purulent pustular and exudative erosions (Fig. 2). On general examination all the patients looked fit and none showed evidence of systemic disease. It is not certain whether septic foci were specifically sought in each patient, but it was noted that the ears, nose and throat were normal in Cases 2. 5 and 6. Ixiboratory
investigations
Abnormal findings were as follows. In Case 2 the erythrocyte sedimentation rate was raised to 78 ram/h (Westergren). Streptococcus pifogenes and Staphylococcus aureus were isolated from lesions which were obviously secondarily infected in Case 2 and S. aureus was isolated from an infected lesion in Case 5.
Skin biopsies
Histological examination revealed similar changes in all patients, although it was usually necessary to examine serial sections. The findings in each patient are summarized in Table 1. A constant feature was a subcorneal pustule, either densely packed with polymorphonuclear leucocytes and cellular debris or flaccid and containing relatively few cells. There was a moderate degree of acanthosis and mild spongiosis of the epidermis in most cases. There was evidence of a spongiform pustule in each patient. This was either small and insignificant, situated in the side of a round subcorneal pustule, or large and well developed forming part of the base of a flat blister. A parakeratotic layer was usually seen to form part of the base of the subcorneal pustule (Fig. 3).
472
R.J.BARLOW AND E.J.SCHIILZ
Dermal changes included papillary oedema which was seen in all paiients except Case 4. lixtravasalitHi of erythrocytes in the tips of dermal papillae was a feature in some patients and occurred mainly in areas where papillary oedema was intense (Fig. 41. In Case S the extravasation was perivascular, situated deeper in the papilla. Some papillae contained polymorphonuclear leucocytes that could be seen migrating through the overlying epidermis towards the subcomeai pustule. Capillaries and larger vessels showed perivascular oedema and a perivascular infiltrate of mononuclear cells and polymorphs showing varying amounts of leukocytoclasis. In Cases 5 and h fragments of neulrophils and erythrocytes were seen in the same papillae. Fibrinoid change and disruption of vessel walls were seen in Cases 1, 4, S and ft. Figure 1. Lesions involving mainly sides of neck In Case i.
Gram stains of pustules in Cases 4 and 6 demonstrated no bacteria. Immunofluorescence studies on frozen sections in Cases 2, 5 and 6 gave negative or non-specific results. In Case 2 there was a moderately strong, thin linear band of fluorescence at the basement membrane zone under the pustule with Cj and strong fluorescence of vessels in the mid-dermis with fibrin. Treatment In three patients (Cases 1, 2 and S) the rash responded rapidly to dapsone in a dose of 50-1 (K) mg/day. In Case 6, who was followed up for 2 years, a combination of dapsone 100-1 SO mg/day and prednisone 5 or 10 mg on alternate days was necessary. The remaining two patients (Cases 3 and 4) showed a rapid temporary improvement on treatment with topical and oral tetracycline. the latter given in a dose of 250 mg twice daily for a few weeks at a time.
Discussion
Rgurc 2. Diffuse involvemeni ol trunk and neck with secondary infection in Case 5.
Our patients were initially diagnosed as having subcorneal pustular dermatosis (Sneddon-WilkinsonI because of the chronic recurrent nature of the eruption and the initial histological finding of subcorneal pustules without other obvious epidermal change. However the finding of a spongiform pustule in one of the later patients in the series prompted us to re-examine serial sections of the original biopsies. In addition to spongiform pustules, we found evidence of capillaritis in the papillary dermis and vascuiitis in the mtd-dermis. We seemed to be dealing with a specific entity wilh a characteristic clinical picture in which recurrent wide-
SUBCORNEAL PUSTULOSIS WITH VASCULITIS
473
''i V
•
(•igure J. Histology of flat Hactid suhcorneal pustule in Case I showing spongiform pustule in hase with parakeratosis on the right.
. * .
>••
%
. .
'^^^:l'-iS^^^ •
. '
•
•••%
^•,1-'.
Figure 4. Oedema, erythrocyte extravasation and polymorphs in papillae in Case 5.
spread subcorneal pustules were often maximal in the flexures but were never annular or gyrate and which showed on biopsy a spongiform pustule and underlying vascuiitis. Additional features were that all patients were female and that there was a familial occurrence in some. The infection present in some areas in two of the patients was regarded as a secondary phenomenon and not part of the actual disease. Subcorneal pustular dermatosis was regarded by Sneddon and Wilkinson as a specific entity.-** However, with continued observation many cases have later been diagnosed as pustular psoriasis because they developed spongiform pustules.•* A spongiform pustule is regarded as the single definitive criterion for distinguishing
between subcorneal pustular dermatosis and pustular psoriasis.- •* For this reason we consider our patients to have a chronic recurrent form of generalized pustular psoriasis. In our patients both the spongiform and subcorneal pustules varied in size and shape according to the age of the lesion and the level of sectioning. Subcorneal pustules were round and tense with small lateral spongiform pustules in some biopsies while in others they were flat and flaccid with large basal spongiform pustules. In the dermis the presence of marked papillary oedema with erythrocyte extravasation and leucocytoclasis were regarded as evidence of vascular damage. We have been unable to find vascuiitis reported in
474
R-l-BARLOW AND E.J.SCHIILZ
pustular psoriasis but it has been described in acute pustular eruptions not regarded to be psoriasis. I^eucocytoclastic vascuiitis with a subcorneal (but not spongiform) pustule was found in an 'acute generalized pustular bacterid' ascribed to a streptococcal throat Infection by Tan.' The clinical presentation in this case differed from ours in Ihat the pustules were larger. involved mainly the extremities, including palms and soles, and (x:curred as a single attack of short duration. Miyachi et «/."' described a similar patient with an acute rash in whom histological examination showed spongiform pustules with an underlying leucocytoclastic vascuiitis. In addition, there have been three reports of patients who have had upper respiratory tract infections and subsequently develcjped acute intraepidermal pustular eruptions maximal on their hands and feet."* " These were accompanied by an underlying leucocytoclastic'•*• or lymphocytic" vascuiitis and are likely to have been due to circulating Immune complexes. Circulating immune complexes are an expected finding in acute pustular eruptions due to infections but they have also been demonstrated in patients with psoriasis vulgaris where it is uncertain whether they play a primary or secondary role in the pathogenesis of the disease.'" Depositis of C, and immunoglobulins at the dermo-epidermal junctitin and in vessel walls in patients with pustular and other forms of psoriasis also suggest that immune complexes may be important in the development of lesions.'' In our patients immune complex deposition \s suggested by the observation of a leucocytoclastic vascuiitis. The lack of clinical evidence of underlying infection and the chronic recurrent course makes it unlikely that infection played a role. Our immunofluorescence studies serve mainly to confirm the absence of deposits of fgA which have recently been described in other intraepidermal pustular dermatoses.' In our view, these patients represent a variant of pustular psoriasis in which there is a recognizable
clinical presentation and in which histological examination reveals evidence of vascular damage in addition to both a subcorneal and spongiform pustule. Since making this observation, we have found this histological combination In biopsy specimens of three patients clinically diagnosed as having pustular psoriasis. Our experience has shown that serial sections and. if necessary, multiple biopsies are needed in order to classify the subcomeai pustular dermatoses properly.
References 1 Baiter H. Hyan TI, Cieneraliscd pustular psoriasis. A clinlcaf and epidemiologk-al sludy of 1 IM cases. Hr / t)rrmaiol 1968: 80: 771 ti. 2 Sneddon IB. Wilkinson DS. Subcorneal pustular dermatosis. Hr / Dermatol 1979: 100: 61-8, i Tan RS'H. Acute generalLsed puitlular bacterid. An unusual niEiniri'station of Icukocytoclastic vascuiitis. Br / Dermatol 1974: 91:209 IS. 4 Miyachi Y. Danno K. Vanase K. Imamura S. Acute gencraliacd pustular bacterid and Immune complexes, .^lia Derm Vetierrol (Stockh) 1980:6(>:(>6-H. 5 Ishikawa II. Nameki H. Hattori A, Akutcs generalisiertes pustuloses Baktcrid: Klne Abart dcs pustuliisen Buktcrid Andrews. Hautarzt 1979: 10: 144-8. 6 Braun-Faico 0. lAiderschmidt C, Maclelewskl W. Scherer R. Pusiulnsis in ulH genera I isata. Kine ungcwohnlicht' l-lrschelnunRSfomi von irukozytoklHsticher Vaskulitls. Hauiurzt 1978; 29: 1717. 7 Hoffman T). Keltler A, Bruce S. Acute iicrnl pustulosis. Hr / Dermatol 19S9: 120: 107-11. 8 Sneddon IB. Wilkinson DS. Subcomeai pu.stular dermatosis. Hr / Dfrrwaio/ I9SI>:6S: 185-94, 9 Sanchez NP. IVrry HO. Muller SA. WInkelmann RK. Subcomeai pustuiar dcrmaUisis and pustular psoriasis, ,Arcb Uermatol 198J; 119:715-21. 10 Kapp A. Kempcr A. Scho[rfE. Deicher H. Detection oT circulating immune complexes In patients with atopic dermatitis and psoriasis. Aita Derm Venereol (Siockh) 1986: 66: 121-6. 11 llllman S, 1 ialberg P. Hentzer B. IJeposlLs of Im muni Jglobu tin and complement in psoriatic lesions. I Cutan I'athol 1980: 7: 2 7 t - 5 . 12 Wright S. I'hlllips T. Ryan |. Leigh IM, Intraepldemial neutrophilic IgA demiatosls with colitis. Br J Dermatol 1989: 120: I D - 1 9 .
ADONIS 0007096391001122
Chronic subcorneal pustulosis with vasculitis: a variant of generalized pustular psoriasis in black South Africans R.I.BARLOW AND E.J.SCHULZ* Medical llmversity of Southern Africa, Pretoria. South Africa 'University ofthe Witwatersrand. Johannesburg, South Africa Accepted for publication 3 December 1990
Summary
A rare, hut distinctive chronic eruption in six female hlack South Africans is reported. The original diagnosis of subcorneal pustular dermatosis of Sneddon and Wilkinson in these patients was refuted by the subsequent histological observation of both spongiform pustules and an underlying vasculitis. This may represent a previously undocumented form of generalized pustular psoriasis.
Pustular psoriasis may run an acute or chronic course with or without evidence of previous plaque psoriasis. Skin lesions may be localized, often to the hands and feet, or generalized. Annular forms of generalized pustular psoriasis tend to be subacute or chronic and clinically resemble subcorneal pustular dermatosis of Sneddon and Wilkinson.' A suhcorneal pustule occurs in both conditions but the spongiform pustule, characteristic of pustular psoriasis, is not found in suhcorneal pustular dermatosis.^ This is a retrospective study of a rare chronic pustular eruption seen in six black South Africans over the 12year period 1975-1989. The clinical features and initial histological findings suggested subcorneal pustular dermatosis. hut later histological findings included spongiform pustules and evidence of a vasculitis. We have been unable to find vasculitis documented In association with pustular psoriasis (i.e. with spongiform pustules), but it has been reported in patients with acute eruptions precipitated by infection and described as 'bacterids'.'"'' pustulosis acuta generalisata'*' and "acute acral pustulosis*.''
Case reports All six patients were black South Africans seen as outpatients in the Transvaal between 1975 and 1989. Clinical details are summarized in Table 1. All were Correspondence: Professor E.J.Schulz. Division of Dennatology. Area 553, Johannesburg Hospital and tJniversity of the Witwatersrand. Pdrktown, Johannesburg 2193. South Africa.
470
female and their ages ranged from 11 to S5 years. Two patients were related, being mother and daughter (Cases 3 and 4). and a third (Case 1) reported that her sister had a similar rash. The duration of the disease varied from 1 to 31 years and the age of onset from 4 to 29 (mean 18) years. Case 4 had never been free of lesions for more than a few weeks at a time and Case (S had been free of lesions only during periods of treatment with dapsone and prednisone. It is not known whether the rash was continuous or recurrent in the remaining four patients. The lesions were recorded as being itchy in two patients (Cases 2 and 4). not itchy in one (Case 1) and irritating in Case 5. In the remaining two (Cases 3 and 6) the presence or absence of symptoms was not recorded. No patient reported any associated fever, malaise, arthralgia or other systemic upset and all were otherwise healthy with no history of significant previous illnesses. The eruption affected the trunk and limbs in varying degrees. There was a tendency for involvement of the sides of the neck (Fig. 1) and trunk and the shins. The head, palms and soles were spared in each case. In all patients the skin lesions were at various stages of development. They were diffusely scattered without any grouped, annular or serpiginous arrangement. The primary (esion was a superficial rather flaccid off-white pustule. 2-4 mm in diameter set on a mildly erythematous base. The pustule dried to form a dark crust which separated to reveal a scaling rim around a small white depigmented and slightly atrophic scar. This, together with the surrounding skin, later became hyperpigmented. with some mild residual scaling in areas (Fig, 1). In two patients (Cases 2 and 5) secondary infection was
SUBCORNEAL PUSTULOSIS WITH VASCULITIS
471
Table t. Clinical and histological findings in patients with chronic subcorncal pustulosis Histoiogii-iil features Pattent 1
Sex/age (years)
Duration (years) 1
F/11
Epidermal
Distribution of rash Trunk, limbs especially shins
Three biopsies: (a) Long flat SCP with basal spongiform pustule and parakeratosis Mild spongiosis (b) Flat flaccid SPC Small spongiform pustule Little parakeratosis Mild spongiosis (c) Round tense intraepidermal SCP Small lateral spongiform pustule No parakeratosis Spongiosis + +
Dermal
Perivascular oedema + Mild vascuiitis Papillary otxlema + + RBC + -(- H- in papillae Vascuiitis Miltl papillary oedema Mild mixed perivascular inflltrate
2
F/25
2
Trunk, mainly lateral: proximal limbs
Tense dome-shaped SCP with PMN and cellular debris Large basal spongiform pustule and parakeratosis
Mild papillary' oedema l-'ew papillary RBC Mild mixed perivascular infiltrate
3
F/43
25
Sides of neck, axillae
long tense SCP with PMN and cellular debris Long basal spongiform pustule and parakeratosis
Marked papillary oedema Papillary RBC + + + Vascuiitis
4
F/15
11
Lower legs mainly shins, wrists
Tense round SCP with few PMN Small lateral spongiform pustule Very little parakeratosis
Mild papillary oedema Mild vascuiitis
5
F/31
2
Sides of neck, trunk. axillae, groins
Long tiaccid SCP Spongiform pustule and parakeratosis in base
Papillary oedema + Perivascutar RBC + 4^ Vascuiitis + +
F/55
31
Sides of neck, trunk. axillae, limbs, shins
Flaccid SCP Long ill-defined basal spongiform pustule No parakeratosis
Papillary oedema Papillary RBC Mild vascuiitis
SCP. subcomeai pustule: RBC. red blood cells: PMN. polymorphonuclear leucocytes.
present in some areas resulting in additional frankly purulent pustular and exudative erosions (Fig. 2). On general examination all the patients looked fit and none showed evidence of systemic disease. It is not certain whether septic foci were specifically sought in each patient, but it was noted that the ears, nose and throat were normal in Cases 2. 5 and 6. Ixiboratory
investigations
Abnormal findings were as follows. In Case 2 the erythrocyte sedimentation rate was raised to 78 ram/h (Westergren). Streptococcus pifogenes and Staphylococcus aureus were isolated from lesions which were obviously secondarily infected in Case 2 and S. aureus was isolated from an infected lesion in Case 5.
Skin biopsies
Histological examination revealed similar changes in all patients, although it was usually necessary to examine serial sections. The findings in each patient are summarized in Table 1. A constant feature was a subcorneal pustule, either densely packed with polymorphonuclear leucocytes and cellular debris or flaccid and containing relatively few cells. There was a moderate degree of acanthosis and mild spongiosis of the epidermis in most cases. There was evidence of a spongiform pustule in each patient. This was either small and insignificant, situated in the side of a round subcorneal pustule, or large and well developed forming part of the base of a flat blister. A parakeratotic layer was usually seen to form part of the base of the subcorneal pustule (Fig. 3).
472
R.J.BARLOW AND E.J.SCHIILZ
Dermal changes included papillary oedema which was seen in all paiients except Case 4. lixtravasalitHi of erythrocytes in the tips of dermal papillae was a feature in some patients and occurred mainly in areas where papillary oedema was intense (Fig. 41. In Case S the extravasation was perivascular, situated deeper in the papilla. Some papillae contained polymorphonuclear leucocytes that could be seen migrating through the overlying epidermis towards the subcomeai pustule. Capillaries and larger vessels showed perivascular oedema and a perivascular infiltrate of mononuclear cells and polymorphs showing varying amounts of leukocytoclasis. In Cases 5 and h fragments of neulrophils and erythrocytes were seen in the same papillae. Fibrinoid change and disruption of vessel walls were seen in Cases 1, 4, S and ft. Figure 1. Lesions involving mainly sides of neck In Case i.
Gram stains of pustules in Cases 4 and 6 demonstrated no bacteria. Immunofluorescence studies on frozen sections in Cases 2, 5 and 6 gave negative or non-specific results. In Case 2 there was a moderately strong, thin linear band of fluorescence at the basement membrane zone under the pustule with Cj and strong fluorescence of vessels in the mid-dermis with fibrin. Treatment In three patients (Cases 1, 2 and S) the rash responded rapidly to dapsone in a dose of 50-1 (K) mg/day. In Case 6, who was followed up for 2 years, a combination of dapsone 100-1 SO mg/day and prednisone 5 or 10 mg on alternate days was necessary. The remaining two patients (Cases 3 and 4) showed a rapid temporary improvement on treatment with topical and oral tetracycline. the latter given in a dose of 250 mg twice daily for a few weeks at a time.
Discussion
Rgurc 2. Diffuse involvemeni ol trunk and neck with secondary infection in Case 5.
Our patients were initially diagnosed as having subcorneal pustular dermatosis (Sneddon-WilkinsonI because of the chronic recurrent nature of the eruption and the initial histological finding of subcorneal pustules without other obvious epidermal change. However the finding of a spongiform pustule in one of the later patients in the series prompted us to re-examine serial sections of the original biopsies. In addition to spongiform pustules, we found evidence of capillaritis in the papillary dermis and vascuiitis in the mtd-dermis. We seemed to be dealing with a specific entity wilh a characteristic clinical picture in which recurrent wide-
SUBCORNEAL PUSTULOSIS WITH VASCULITIS
473
''i V
•
(•igure J. Histology of flat Hactid suhcorneal pustule in Case I showing spongiform pustule in hase with parakeratosis on the right.
. * .
>••
%
. .
'^^^:l'-iS^^^ •
. '
•
•••%
^•,1-'.
Figure 4. Oedema, erythrocyte extravasation and polymorphs in papillae in Case 5.
spread subcorneal pustules were often maximal in the flexures but were never annular or gyrate and which showed on biopsy a spongiform pustule and underlying vascuiitis. Additional features were that all patients were female and that there was a familial occurrence in some. The infection present in some areas in two of the patients was regarded as a secondary phenomenon and not part of the actual disease. Subcorneal pustular dermatosis was regarded by Sneddon and Wilkinson as a specific entity.-** However, with continued observation many cases have later been diagnosed as pustular psoriasis because they developed spongiform pustules.•* A spongiform pustule is regarded as the single definitive criterion for distinguishing
between subcorneal pustular dermatosis and pustular psoriasis.- •* For this reason we consider our patients to have a chronic recurrent form of generalized pustular psoriasis. In our patients both the spongiform and subcorneal pustules varied in size and shape according to the age of the lesion and the level of sectioning. Subcorneal pustules were round and tense with small lateral spongiform pustules in some biopsies while in others they were flat and flaccid with large basal spongiform pustules. In the dermis the presence of marked papillary oedema with erythrocyte extravasation and leucocytoclasis were regarded as evidence of vascular damage. We have been unable to find vascuiitis reported in
474
R-l-BARLOW AND E.J.SCHIILZ
pustular psoriasis but it has been described in acute pustular eruptions not regarded to be psoriasis. I^eucocytoclastic vascuiitis with a subcorneal (but not spongiform) pustule was found in an 'acute generalized pustular bacterid' ascribed to a streptococcal throat Infection by Tan.' The clinical presentation in this case differed from ours in Ihat the pustules were larger. involved mainly the extremities, including palms and soles, and (x:curred as a single attack of short duration. Miyachi et «/."' described a similar patient with an acute rash in whom histological examination showed spongiform pustules with an underlying leucocytoclastic vascuiitis. In addition, there have been three reports of patients who have had upper respiratory tract infections and subsequently develcjped acute intraepidermal pustular eruptions maximal on their hands and feet."* " These were accompanied by an underlying leucocytoclastic'•*• or lymphocytic" vascuiitis and are likely to have been due to circulating Immune complexes. Circulating immune complexes are an expected finding in acute pustular eruptions due to infections but they have also been demonstrated in patients with psoriasis vulgaris where it is uncertain whether they play a primary or secondary role in the pathogenesis of the disease.'" Depositis of C, and immunoglobulins at the dermo-epidermal junctitin and in vessel walls in patients with pustular and other forms of psoriasis also suggest that immune complexes may be important in the development of lesions.'' In our patients immune complex deposition \s suggested by the observation of a leucocytoclastic vascuiitis. The lack of clinical evidence of underlying infection and the chronic recurrent course makes it unlikely that infection played a role. Our immunofluorescence studies serve mainly to confirm the absence of deposits of fgA which have recently been described in other intraepidermal pustular dermatoses.' In our view, these patients represent a variant of pustular psoriasis in which there is a recognizable
clinical presentation and in which histological examination reveals evidence of vascular damage in addition to both a subcorneal and spongiform pustule. Since making this observation, we have found this histological combination In biopsy specimens of three patients clinically diagnosed as having pustular psoriasis. Our experience has shown that serial sections and. if necessary, multiple biopsies are needed in order to classify the subcomeai pustular dermatoses properly.
References 1 Baiter H. Hyan TI, Cieneraliscd pustular psoriasis. A clinlcaf and epidemiologk-al sludy of 1 IM cases. Hr / t)rrmaiol 1968: 80: 771 ti. 2 Sneddon IB. Wilkinson DS. Subcorneal pustular dermatosis. Hr / Dermatol 1979: 100: 61-8, i Tan RS'H. Acute generalLsed puitlular bacterid. An unusual niEiniri'station of Icukocytoclastic vascuiitis. Br / Dermatol 1974: 91:209 IS. 4 Miyachi Y. Danno K. Vanase K. Imamura S. Acute gencraliacd pustular bacterid and Immune complexes, .^lia Derm Vetierrol (Stockh) 1980:6(>:(>6-H. 5 Ishikawa II. Nameki H. Hattori A, Akutcs generalisiertes pustuloses Baktcrid: Klne Abart dcs pustuliisen Buktcrid Andrews. Hautarzt 1979: 10: 144-8. 6 Braun-Faico 0. lAiderschmidt C, Maclelewskl W. Scherer R. Pusiulnsis in ulH genera I isata. Kine ungcwohnlicht' l-lrschelnunRSfomi von irukozytoklHsticher Vaskulitls. Hauiurzt 1978; 29: 1717. 7 Hoffman T). Keltler A, Bruce S. Acute iicrnl pustulosis. Hr / Dermatol 19S9: 120: 107-11. 8 Sneddon IB. Wilkinson DS. Subcomeai pu.stular dermatosis. Hr / Dfrrwaio/ I9SI>:6S: 185-94, 9 Sanchez NP. IVrry HO. Muller SA. WInkelmann RK. Subcomeai pustuiar dcrmaUisis and pustular psoriasis, ,Arcb Uermatol 198J; 119:715-21. 10 Kapp A. Kempcr A. Scho[rfE. Deicher H. Detection oT circulating immune complexes In patients with atopic dermatitis and psoriasis. Aita Derm Venereol (Siockh) 1986: 66: 121-6. 11 llllman S, 1 ialberg P. Hentzer B. IJeposlLs of Im muni Jglobu tin and complement in psoriatic lesions. I Cutan I'athol 1980: 7: 2 7 t - 5 . 12 Wright S. I'hlllips T. Ryan |. Leigh IM, Intraepldemial neutrophilic IgA demiatosls with colitis. Br J Dermatol 1989: 120: I D - 1 9 .
