Acrodermatitis continua suppurativa L40.2

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 08.10.2021

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Synonym(s)

Acrodermatitis continua of Hallopeau; Acrodermatitis continua suppurativa; Acrodermatitis perstans; Dermatitis infectious eczematoid; dermatitis repens; Hallopeau-Leredde syndrome; Hallopeau's disease; impetigo; pustular dermatitis; vegetarian dermatitis

History
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Crocker 1888; Hallopeau 1889

Definition
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Eminently chronic, sterile, usually painful, pustular dermatitis of the finger (less commonly: toe) end-limbs, leading to skin and nail atrophy, with an exudative course lasting many years. May also lead to osteolytic changes of the acras.

Etiopathogenesis
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Unexplained; the pustular variant of an acral localized psoriasis pustulosa palmaris et plantaris is postulated. In larger collectives, however, only a weak association with psoriasis is found. In this respect, an independence of the disease must also be discussed.

An association with HLA-B27 is described.

Further associations exist to variants of the IL-36RN gene which have been shown to be involved in acropustulosis as well as pustulosa generalisata psoriasis.

Furthermore, associations were found to a mutation in the AP1S3 gene (Adaptor related protein-1 complex subunit delta1C), a gene coding for a subunit of the adaptor protein complex 1 (AP-1). The detected mutations lead to an autoreactive inflammatory response via an overexpression of IL 36 (Setta-Kaffetzi N et al. 2014). AP1S3 mutations are also detected in pustulosa generalisata psoriasis and palmoplantar pustulosis (Setta-Kaffetzi N et al. 2014). Furthermore, IL36RN mutations have also been identified (IL36RN is a protein-coding gene that forms a cytokine gene cluster with 8 other genes of the interleukin-1 family on chromosome 2. Cytokines of this family play an essential role in epithelial barrier function as well as in inflammatory processes)

Manifestation
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Age at first manifestation > 40 years

Localization
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Dorsal sides and tips of the end phalanges of fingers and/or toes. Usually severe affection of the nail organ with nail dystrophy or complete loss of the entire nail(anonychia). More rarely, blisters or pustules on the soles of the feet or palms of the hands are observed. A transition to pustular psoriasis generalisata is not excluded in principle, but is the exception.

Clinical features
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Sharply proximal, red, painful plaque with superficial, mostly grouped, often confluent, 0.2-0.5 cm large yellowish or white pustules. Next to it scales and scaly crusts. Pustules often also form subungually and on the lateral nail walls.

The characteristic pustules may be absent (e.g. due to pre-treatment), so that at the first diagnosis the clinical picture is misjudged as chronic paronychia.

Typical is an eminently chronic, intermittent course lasting for years.

As the disease progresses, dystrophy or loss of nails, acral bone atrophy, stiffening of the distal finger end joints (DIP) may occur.

The minus variant, which occurs mainly in children < 8 years of age, can be called parakeratosis Hjorth-Sabouraud.

Histology
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Hyperkeratoses, acanthosis, unilocular, intraepithelial pustule with neutrophil leukocytes, spongiform degeneration of the epidermal cells in the peripheral area of the pustule.

External therapy
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In the acute phase of relapse glucocorticoids under occlusion. Suitable are 0.1% triamcinolone cream or halogenated glucocorticoids such as 0.05-0.1% betamethasone cream/ointment or mometasone furoate ointment (Ecural).

Glucocorticoid crystal suspensions injected intralesionally by Dermojet are indicated for very persistent lesions. Note: Injections are very painful due to localization!

In addition, astringent hand baths with tanning agents (e.g. Tannosynt liquid) can be performed.

Radiation therapy
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The local PUVA therapy as PUVA bath therapy or PUVA cream therapy is the first choice for moderate and severe forms of acrodermatitis continua suppurativa therapy.

Internal therapy
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The disease is a classic indication for a systemic therapy.

  • Acitretin:
    • Adults: Initially 0.5 mg/kg bw neotigasone/day, over 2-4 weeks, then depending on the effect increase to a maximum of 1 mg/kg bw/day. Maintenance dose: Usually 0.1-0.5 mg/kg bw/day for a further 6-8 weeks, possibly longer.
    • Children: Very strict indication, careful benefit-risk assessment. Initial: 0.5 mg/kg bw/day. Maintenance dose: 0.1 mg/kg bw/day, under no circumstances > 0.2 mg/kg bw/day or > 35 mg/day.

Remember! Systemic therapy should be combined with external treatment. If necessary combination of acitretin with PUVA bath therapy or PUVA cream therapy as RePUVA therapy.

  • Alternative:
    • Fumaric acid ester (e.g. Fumaderm): In severe cases, try increasing the dosage, e.g. Fumaderm initial 1 tablet/day in week 1 up to a maximum of 6 tablets/day of Fumaderm in week 6.
    • Methotrexate: initial 10-15 mg/week p.o. or s.c. Depending on the response, the dose can be slowly reduced week by week. Maintenance dose: 2.5-5.0 mg/week.
    • Ciclosporin A: reserve therapeutic agent! Initial: 2.5 mg/kg bw/day p.o., increase to 5 mg/kg bw possible, dose reduction according to skin findings, aim for the lowest possible maintenance dose.
    • Biologics: The use of biologicals is obvious. However, it is limited to individual case reports.
      • Etanercept: S.a.u. Psoriasis vulgaris. Standard dose (patient > 18 years): 2 times/week 25 mg s.c. Alternatively: 2 times/week 50 mg s.c. for up to 12 weeks, then 2 times/week 25 mg s.c.
      • Adalimumab: 40 mg s.c. every 4 weeks (standard dose)

Case report(s)
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A 43-year-old patient with pronounced, therapy-refractory acrodermatitis continua suppurativa at several fingers of both hands received adalimumab as off-label-use in a dosage of 40 mg every 2 weeks for a total duration of 32 weeks after the application of multiple, less successful topical agents as well as an only moderately successful system therapy with methotrexate (15 mg/week p.o.) as well as Ciclosporin A (3x100 mg/day). Thereunder clear improvement. Except for the occasional appearance of "dyshidrotic vesicles" in the lesional area, the patient is free of symptoms.

Literature
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  1. Ashurst PJC (1964) Relapsing pustular eruptions of the hands and feet. Brit J Derm 76: 169-180
  2. Buder V et al (2015) Successful therapy with adalimumab in acrodermatitis continua suppurativa. JDDG 13 (Suppl 1) 80
  3. Crocker HR (1888) Diseases of the skin. Lewis, London
  4. Hallopeau FH (1889) Sur une nouvelle forme de dermatite pustuleuse chronique en foyer à progression excentrique. Congr intern Derm Syph Compt rend, Paris: 344
  5. Hallopeau M (1887) Sur un quatrié fait d'acrodermatite suppurative continuu. Ann Dermatol syphiligr 8: 1277-1281
  6. Iijima S (2014) Case of acrodermatitis continua of Hallopeau following psoriasis with atypical clinical presentation. J Dermatol 41:1006-1008
  7. Piraccini BM et al (1994) Hallopeaus acrodermatitis continua of the nail apparatus. Acta Derm Vener 74: 65-67
  8. Sahin MT et al (2003) Acrodermatitis continua of Hallopeau: was it an outcome of a surgical trauma or initially misdiagnosed as onychomycosis? J Eur Acad Dermatol Venereol 17: 236-238
  9. Setta-Kaffetzi N et al (2014) AP1S3 mutations are associated with pustular psoriasis and impaired Toll-like receptor 3 trafficking. At J Hum Genet 94: 790-797
  10. Török L et al (2003) Pemphigus vegetans presenting as acrodermatitis continua suppurativa. Eur J Dermatol 13:579-581
  11. Weisshaar E et al (2007) Successful etanercept therapy in therapy refractory
  12. acrodermatitis continua suppurativa Hallopeau. J Dtsch Dermatol Ges 5:489-492
  13. Wilsmann-Theis D et al (2004) Successful treatment of acrodermatitis continua suppurativa with topical tacrolimus 0.1% ointment. Br J Dermatol 150:1194-1197

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Please ask your physician for a reliable diagnosis. This website is only meant as a reference.

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Last updated on: 08.10.2021