ACROPUSTULOSIS REPENS ()

Inserida em: 29/08/2018

(*) Published in (International Journal of Dermatology 2006, 45, 389-393)

Nelson Guimarães Proença

From the Faculty of Medical Sciences, Santa Casa de São Paulo, SP, Brazil

 

ABSTRACT

BACKGROUND: Pustular eruptions of the extremities of fingers and toes (acropustulosis) have been grouped under the single term of Hallopeau’s acrodermatitis continua, which is a severe disease, with uninterrupted course, and successive eruptions that may become generalized. However, there is a form of acropustulosis with few lesions, evolving with remissions and relapses, of a benign course. It is necessary to separate these two forms of acropustulosis.

METHODS: I had the opportunity to observe six patients with a mitigated form of acropustulosis that does not fit into the diagnostic criteria of Hallopeau’s acrodermatitis continua, which should be considered an independent clinical entity.

RESULTS: Sometimes, there is only one lesion in each eruption. The pustules are sterile and appear in small crops, located on the hyponychium or on the nail bed. The result is partial onycholysis or nail detachment. After each eruption there is complete recovery. Histopathology shows a non-spongiform pustule filled with neutrophils, with subcorneal localization. Four patients had follow-up at least for two years, and one patient  presented  psoriasis lesions on the scalp after 11 years.

 

CONCLUSIONS: Acropustulosis as I described it can be differentiated from Halopeau’s acrodermatitis continua using the following diagnostic criteria: (1) it is a benign condition; (2) the pustules are located on the hyponychium or nail bed; (3) pustules can be single or occur in small groups; (4) they reccur in flares, with restitutio ad integrum during the intervals; (5) the pustules are sterile; (6) microscopic study  shows a subcorneal pustule (spongiform aspect is rare). A similar condition to our cases was described by Radcliff-Crocker under the denomination of dermatitis repens (H. Radcliff-Crocker, Diseases of the Skin, London: H.K.Lewis,1888), termed “dermatitis repens”. 

INTRODUCTION

The chapter in Dermatology about pustular eruptions of the hands and feet is one in which the current concepts are not definitively fixed neither universally accepted. Nowadays only the two following entities have been included in the group: pustular psoriasis and Hallopeau’s acrodermatitis continua,  with exclusion of the relapsing Radcliff-Crocker type. The Andrews’s “pustular bacterid” , whose independence is no longer accepted, also nowadays has been excluded of the group. Recently a recurring condition was added to the chapter, which affects children during the first 2 years of life, and is characterized by pustular eruptions on the hands and feet (infantile acropustulosis recidivans).

The oldest account is given by Radcliffe-Crocker (1) that, in 1888, included in his textbook his personal observations of patients with repetitive pustular eruptions on  the hands and feet. Two years later, in 1890, Hallopeau (2) published his first case entitled “polydactylite suppurative chronique et poussées éphéméres des dermatite pustuleuse”. The latter author returned to the theme seven years later (3), adding new cases, keeping the denomination “acrodermatites continués (polydactylites récidivantes)”. The clinical picture and the evolution of these two conditions are not exactly the same. Radcliff-Crocker made reference to the benign course of his cases, with isolated lesions evolving by crops and recovering completely between eruptions. The patients treated by Hallopeau always presented lesions on the same location, and evolved with intermittent eruptions in a more aggressive form, without periods of complete remission. In Hallopeau’s disease progressive worsening of the clinical conditions occurs and it may become generalized.

Even considering these differences, prestigious textbooks prefer to consider the two conditions as manifestations of a single morbid entity, both grouped under the denomination Hallopeau’s “acrodermatitis continua” (4,5).

The author of the present study observed six patients with acropustulosis that does not fit the description of acrodermatitis continua, with the lesions being more similar to the variety described by Radcliff-Crocker. Four of these cases have been previously communicated (6,7), but there are two new cases. Hoping to contribute to the clarification of this subject, we deciced to publish the complete series.

 

METHODS

Observations were done in a private dermatological clinic, between 1978 and 2003. Table I summarizes the data regarding the six cases.

RESULTS

The pustules appeared in an isolated fashion, on the finger extremity (fig 1a and 1b), or on the toes. The location could be on the nail bed, beeing observed by transparency (figure 2), and causing partial detachment of the nail (fig 3). When located on the nail bed, healing of the eruption led to complete recovery of the nail. Anonychia was not observed. The eruptions usually affected only one finger at a time. In one case, four fingers were simultaneously involved. In successive relapses, pustules could affect previously affected locations or not.

Pathology was done only in three cases (the remaining patients did not agree to undergo biopsies). The microscopic analysis identified subcorneal pustules filled with neutrophils (fig 4). The spongiform aspect described by Kogoj was seen, in one field or another, but not in a convincing manner.

With reference to the location, the pustules were distributed as follows: (a) On the right hand: thumb (1 case), index (3 cases), medial (1 case), minimum (3 cases); (b) On the left hand: index (3 cases), medial (1 case), ring finger (1 case), minimum (2 cases). On the foot only the right halux was affected (2 cases).

 

Four of our six patients were followed for more than three years. For these patients, the results of treatment were dubious and ephemeral. Because of the spontaneous healing of the pustules, it was difficult to evaluate the results of the effect of therapies. Even after the remission of the pustules, it was not possible to prevent the recurrence of eruption with any of the proposed treatments. The following therapies were used: topical corticoids (5 cases), oral corticoids (1), corticoid infiltration (1), colchicine (2), dapsone (2), methotrexate (1), and calcipotriol (1).

In spite of the recurrence, the evolution was always very benign both in the period prior to the first consultation as well as during follow-up. Case 1 presented spontaneous resolution, with clinical cure, and this remission persists for 21 years. In case 2, after five years, pitting appeared in several nails, suggesting an association to psoriasis, which was confirmed after another six years, with psoriasis vulgaris plaques appearing on the scalp (eleven years after the original condition!). Patient 5 is currently in follow-up, always manifesting eruptions with scarce pustules, in intervals ranging between 2 to 6 months. Patient 4 died after three years of follow-up from myocardial infarction. In cases 3 and 6, the follow-up was done for few months, with information lacking about the posterior course.

DISCUSSION

An important cause of disagreement arises from the relationship between pustules of the extremities and psoriasis. In 1930, on the same issue of the British Journal of Dermatology, the works of Ingram (8) and of Barber (9) were published, both calling attention to the many cases in which pustules of the extremities were associated with psoriasis. For the history of Dermatology, palmoplantar pustular psoriasis, as it was described, became known as “Barber type”. As a matter of fact this denomination seems incorrect, because it should be “Ingram-Barber type”.

For some authors, all pustulous conditions of the extremities would be varieties of pustular psoriasis of acral localization (4,5,10). In support of this point of view, there are two arguments. One refers to the clinical aspect of the pustules, which is practically the same on the palms and soles. The other argument concerns the microscopic features of the lesions, characterized by the frequent presence of subcorneal pustule replete with neutrophils, with the margins having a spongiform aspect as described by Kogoj.

There are opposing opinions to the concept of a single entity, showing preference in the separation of two forms of pustulosis of the palms and soles, palmoplantar pustular psoriasis and pustulosis palmaris et plantaris (PPP). They are: (a) only about 7.7% of the patients with PPP show association to familial psoriasis, against 33% of the patients with psoriasis (all forms) that report familial cases (11); (b) the HLA study in patients with  PPP was not able to demonstrate the presence of HLA-B13 and HLA-B17 antigens, which are frequently found in psoriasis cases (12-14); (c) PPP frequently presents association with skeletical alterations (chronic recurrent multifocal osteomyelitis), particularly on the sternocostoclavicular bones (15-17); (d) the response to the treatment with methotrexate is better in Ingram-Barber pustular psoriasis than PPP (personal experience).

A series of 272 patients with PPP was followed-up during 10 years in Sweden (18). Only 6.1% presented the open form of psoriasis, prior or during follow-up, always in very discrete forms. Adding the 18.3% of cases that presented only nail pitting, or areas of scaling on the scalp, which could be considered probable mitigated forms of psoriasis, this percentage increases to 24.4%.

Although recognizing that there is a relationship between both of these conditions, the arguments presented above suggest to maintain separated the two forms of clinical presentation: one which has association with psoriasis on other sites (psoriasis pustulosa palmaris et plantaris, type Ingram-Barber) and another one which does not have this association (named only pustulosis palmaris et plantaris).

The pustular bacterid, described by Andrews & Machacek as a separate entity, with the presentation of 15 patients (19) in the original study, is no longer recognized as such. All of the cases with this diagnosis are today classified under pustulosis palmaris et plantaris. The previous argument to maintain pustular bacterid as an independent condition was its association to tonsillitis or infectious foci of the teeth, which is presently considered insufficient to justify its individuality.

 

Now we will comment on pustular eruptions with acral localization, on fingers and toes, which was the purpose of this paper. It is necessary to remember that almost all authors recognize only one entity on this site, the Hallopeau’s acrodermatitis continua, not accepting the individuality of the disease previously described by Radcliff-Crocker, called dermatitis repens. This is due to the fact that this last author grouped more severe clinical conditions (like the ones of Hallopeau), with more benign clinical conditions. In opposition to this unitary concept we are convinced that a more benign form of the disease really exists, having nothing in common with Hallopeau’s disease. Exactly these more benign acropustulosis cases comprise the series of six patients presented in this paper. Our cases showing a very benign clinical picture and course, need to have their diagnostic individuality maintained separate from the form described by Hallopeau. We understand that our cases, to a certain point, coincide with the description of Radcliff-Crocker. We propose that their individuality should be preserved and to reinforce our proposal, we present the following criteria for its diagnosis:

 

a. the entity is benign, having always localized lesions, without any tendency of generalization;

b. the lesion is represented by a localized pustule on the finger (or toe), on the nail bed  or on the hyponychium;

c. the pustule presents itself as a solitary lesion or a few pustules may be present simultaneously;

d. the evolution occurs through crops, with intervals, and ends with recuperation ad integrum;

e. the pustules are subcorneal and filled with neutrophils, and the spongiform aspect is infrequent;

f. the pustules are sterile.

 

These criteria establish the difference between the disease observed on our series of patients and the Hallopeau’s acrodermatitis continua.There is no reason to consider the  acropustulosis by us described as a benign variant of acrodermatitis continua. They are different entities and should be accepted as such.

 

The denomination proposed by Radcliff-Crocker, of dermatitis repens of the extremities, does not seem entirely appropriate. Such denomination would include, for example, dyshidrosis, which is typically a dermatitis of the extremities and is recurrent. It seems more appropriate to use Acropustulosis repens, denomination that we adopted for our cases.

 

A new condition has been recently added to the group of pustulosis of hands and feet: Infantile acropustulosis. It is characterized by a pruriginous pustular eruption, which affect hands and feet in successive crops. In the cases of infantile acropustulosis that we accompanied personally, frequently succeeded scabies, which makes us believe that it may be a hypersensitive reaction. Its evolution is always favorable because it always regresses completely within approximately six months, after a sequence of relapses.

 

To end our comments we propose a reasonable classification of pustulosis of the extremities, which would be more in agreement with the current knowledge (Table II).

 

REFERENCES

1- Radcliff-Crocker H – Diseases of the skin, London, HK Lewis, 1888.

2- Hallopeau H – Sur une asphyxie locale des extrémités avec polydactylite suppurative chronique et poussées éphéméres des dermatite pustuleuse disséminées et symmétriques. Ann Dermatol Syphiligr 1890; 1: 420.

3- Hallopeau H – Sur les acrodermatites continues (polydactylites récidivantes). Ann Dermatol Syphilogr 1897; 8: 473. 

4- Camp RD - Pustular forms of psoriasis. In Rook ’s Textbook of Dermatology, Blackwell Science Ltd, 1998; 1633-1937.

5- Christophers E, Mrowietz U – Pustular Eruptions of Palms and Soles. In Fitzpatrick ‘s Dermatology in General Medicine, McGraw – Hill, 1999; 723-727.

6- Proença NG – Pustulosis palmaris et plantaris with minimal lesions (type dermatite repens of Radcliff-Crocker). An Bras Dermatol 1990; 65: 256-260.

7- Proença NG – Palmar and plantar pustulosis Radcliff-Crocker”s type, a variety of pustular psoriasis? (Letter). An Bras Dermatol 1995; 70: 70-71.

8- Ingram JT – Acrodermatitis perstans and its relation to psoriasis. Br J Dermatol 1930; 42: 489.

9- Barber HW – Acrodermatitis continua vel. Perstans (Dermatitis repens) and psoriasis pustulosa. Br J Dermatol 1930; 42: 500.

10- Kumar B, Saraswat A, Kaur I. Palmoplantar lesions in psoriasis: a study of 3065 patients. Acta Derm Venereol 2002; 82: 192-195.

11- Hellgren L, Mobacken H. Pustulosis palmaris et plantaris. Prevalence, clinical observations and prognosis. Acta Derm Venereol 1971; 51: 284-288.

12- Karvonen J, Tiilikainen A, Larssus A – HLA antigens in psoriasis. In Psoriasis, Proceedingsof the 2nd International Symposium, New York. Yorke Medical Books 1977; 405-408.

13- Zachariae H. Significance of the pustular reaction in psoriasis. In Psoriasis, Procceedings of the 2nd International Symposium, New York. Yorke Medical Books 1977; 163-170.

14- Ward JM, Barnes RM. HLA antigens in persistent palmoplantar pustulosis and its relationship to psoriasis. Br J Dermatol 1978; 99: 477-483.

15- Jurik AG, Ternowitz T. Frequency of skeletal disease, arthro-osteitis, in patients with pustulosis palmoplantaris. J Am Acad Dermatol 1988; 18: 666-671.

16- Hradil E, Gentz CF, Matilainen T, Moller H, Sanzen L, Uden A. Skeletal involvement in pustulosis palmo-plantaris with special reference to the sterno-costo-clavicular joints. Acta Derm Venereol 1988; 68: 65-73.

17- Sonazaki H, Mitsui H, Miyanaga Y, Okitsu K, Igarashi M, Hayashi Y, Matsuura M, Azuma A, Okai K, Kawashima M. Clinical features of 53 cases with pustulotic arthro-osteitis. Ann Rheum Dis 1981; 40: 547-553.

18- Enfors W, Molin L. Pustulosis palmaris et plantaris. A follow-up study of a ten-year material. Acta Derm Venereol 1971; 51: 289-294.

19- Andrews GC, Machacek GF. Pustular bacterids of the hands and feet. AMA Arch Derm Syphilol 1935; 32: 837.

 

 

 

Table 1 – Acropustulosis repens – presentation of 6 cases

 

Case 1

Case 2

Case 3

Case 4

Case 5

Case 6

1st. Cons

02/23/1978

07/28/1981

11/22/1983

04/12/1984

10/11/1996

10/15/2002

Last Cons.

11/30/2003

08/16/1994

03/13/1984

06/10/1986

12/11/2003

11/28/2002

Follow-up

25 years

13 years

04 months

02 years

07 years

01 month

 

 

 

 

 

 

 

Age Beginning

44 years

15 years

11 years

72 years

17 years

31 years

Age  1st.  Cons.

47 years

20 years

32 years

73 years

18 years

32 years

Age last Cons.

72 years

28 years

32 years

74 years

25 years

32 years

 

 

 

 

 

 

 

Sex/Race

fem/white

fem/white

male/white

male/white

fem/white

fem/white

Fingers/Toes

Great toe

Middle fing.

Index fing.

Great toe

Index fing.

Little fing.

Localization

Nail bed

Nail bed

Hyponych.

Nail bed

N.bed/Hypon

Nail bed

 

 

 

 

 

 

 

Interval/Relapse

1to 6 months

1to 2 months

1 to 2 months

1 to 6 months

2 to 6 months

1 to 2 months

 

 

 

 

 

 

 

Mycol. KOH

negative

negative

negative

negative

negative

negative

Culture

S.epiderm.

negativo

negative

negative

negative

negative

 

 

 

 

 

 

 

Histopathology

SCP

Campos do Jordão

Rua Monsenhor José Vita, 225 - Abernessia
CEP: 12460-000 - Campos do Jordão - SP - Brasil
Telefone: 12 3662-2864 / 12 3664-5300 / 12 3664-3705

SÃO PAULO

Rua Professor Arthur Ramos, 241 - 10º andar - CJ 104
CEP: 01454-011 - São Paulo - SP - Brasil
Telefone: 11 3032-4633