Acrodermatitis Continua

Basics

  • Classified as a noninfectious neutrophilic dermatosis and variant of pustular psoriasis; acropustular eruption with a predilection for distal digits characterized by sterile pustules; early nail involvement; and a persistent, relapsing course
  • Synonym(s): acrodermatitis continua of Hallopeau; acrodermatitis perstans; dermatitis repens; pustular acrodermatitis

Description

  • Acute manifestations
    • Formation of multiple, sterile, painful pustules that have an erythematous base located surrounding and under the nail(s) that coalesce to form polycyclic “lakes of pus,” which subsequently rupture and crust
  • Chronic sequelae occur secondary to recurrent eruptions.
    • Nail changes: paronychia, onychodystrophy, onycholysis, onychomadesis, and anonychia
    • Scaling of the nail bed and periungual skin
    • Sclerosis or atrophy of soft tissue adjacent and deep to nail bed
    • Osteolysis of underlying bone, particularly distal phalanges
  • Distribution
    • Typically, will affect distal extremities, affecting 1 to 2 digits, most frequently the 1st digits of the hands with a predilection for dorsal surfaces of hands and feet, sparing the central palmar/plantar regions
    • May involve all fingers and toes and can rarely spread proximally to involve feet, ankles, hands, and forearms
    • Isolated proximal psoriatic plaques are rare.

Epidemiology

  • Exceedingly rare: Only case studies and series are available.
  • Female predominance
  • Predominantly in adults but also seen in children: observed in 4.7% cases of infantile psoriasis in one series

Etiology and Pathophysiology

  • Immune dysregulation: Acute-phase reactants such as IL-1 and IL-36 likely play a role in pathogenesis (1).
  • In some instances, digital trauma or infection may be an inciting event.

Genetics
  • Reported associations with mutations in the IL36RN gene, which codes for the IL-36 receptor antagonist and is a cause of familial generalized pustular psoriasis (1,2)
  • No observed relationship with plaque psoriasis-associated alleles HLA-B13, B17, or BW37

Risk Factors

  • History of psoriasis, particularly pustular variant
  • Digital trauma
  • Local infection
  • Smoking may contribute to exacerbations.

Diagnosis

  • Defined by clinical evolution and histopathologic features
  • Frequently missed, often a diagnosis of exclusion

History

  • Onset, including any triggering event
  • Duration of eruption, previous episodes
  • Local trauma or infection
  • Impairment in manual dexterity or ambulation
  • Localized symptoms: pain, pruritus, arthritis
  • Systemic symptoms: fever, malaise, weight loss
  • History of other immune disorder such as psoriasis, arthritis, or celiac disease
  • Smoking history

Physical Exam

  • Examine all skin surfaces, nails, scalp, and mucous membranes.
  • Examine joints, evaluating for swelling, erythema, or effusion.
  • Palpate axillary, epitrochlear, and inguinal lymph nodes to assess for lymphadenopathy.
  • Observe and evaluate gait and manual dexterity.
  • Measure vital signs.

Differential Diagnosis

  • Bacterial infection, especially staphylococcal
  • Herpetic whitlow
  • Cutaneous candidiasis
  • Allergic contact dermatitis
  • Dyshidrotic eczema
  • Parakeratosis pustulosa (in children)
  • Pemphigus vulgaris
  • Squamous cell carcinoma, particularly in advanced disease

Diagnostic Tests & Interpretation

Initial Tests (lab, imaging)
  • The following should be performed on fluid obtained from pustules:
    • Gram stain smear
    • Potassium hydroxide preparation
    • In vitro culture
  • Cultures are sterile, unless secondary infection is present.
  • There are no characteristic serologic findings; however, acute systemic inflammation during flares may result in
    • Increased neutrophil count
    • Increased C-reactive protein
  • X-rays of hands and feet may show acroosteolysis, especially in long-standing disease. Ankle osteolysis may also be observed and is usually unilateral.

Follow-Up Tests & Special Considerations
Additional laboratory tests may be necessary to monitor for side effects of treatment.

Diagnostic Procedures/Other
Full-thickness cutaneous punch biopsy of an active pustule may establish diagnosis and should be performed (3,4).

Test Interpretation
  • Nail bed epithelium and adjacent epidermis
    • Compact hyperkeratosis with parakeratosis with or without neutrophils
    • Focal, subcorneal neutrophilic aggregates
    • Spongiform pustules of Kogoj (leukocyte aggregates between epidermal cells, associated with spongiosis)
    • Psoriasiform epidermal hyperplasia
    • Hemorrhagic foci displaying erythrocytes and hemosiderin
  • Dermis
    • Superficial perivascular inflammatory infiltrate composed of lymphocytes, histiocytes, and neutrophils
    • Tortuous, dilated vessels displaying erythrocyte extravasation
    • Edema of papillary dermis

Treatment

  • Due to the rarity of this disease, only expert opinion and case reports are available to guide treatment recommendations.
  • Notoriously refractory to treatment, including topical and systemic agents used successfully in psoriasis
  • The agents listed below may be more effective when used in combination than as monotherapy.
  • Repeated courses or prolonged treatment are often necessary.

General Measures

If cultures show secondary infection, treat with appropriate antibiotics.

Medication

  • Topicals
    • Application under occlusion enhances absorption. Topical agents generally have favorable side effect profiles but may be ineffective, especially as monotherapy:
      • Vitamin D3 analogs, such as calcipotriol: alone or combined with topical tacrolimus, betamethasone dipropionate, or oral acitretin (4)
      • Topical corticosteroids, with or without occlusion
      • May be combined with an oral antibiotic such as a tetracycline (4)
    • 5-Fluorouracil (4)
    • Tacrolimus ointment, with or without occlusion (4)
    • Anthralin (3)
  • Systemics
    • Systemic medications are generally more effective than topicals, although may be more expensive and carry increased risk of adverse effects.
    • Tumor necrosis factor-α (TNF-α) inhibitors
      • Adalimumab has shown greatest effectiveness at 40 mg every 1 to 2 weeks (3,4).
      • Etanercept 20 to 50 mg twice per week (4)
      • Infliximab 5 mg/kg IV at 0, 2, and 6 weeks and then at 8-week intervals (4)
      • Ustekinumab (5)
      • Sometimes combined with oral acitretin, methotrexate, cyclosporine, or prednisone
    • Cyclosporine (4)
    • Acitretin: possible synergistic effect with topical calcipotriol (4)
    • Antibiotics
      • Sulfones, namely dapsone
      • Tetracyclines
    • Colchicine

Additional Therapies

  • Phototherapy and photochemotherapy have been successful as monotherapies or in conjunction with medications.
  • Targeted narrow-band ultraviolet B phototherapy
    • As monotherapy or in conjunction with systemic medications (4)
  • Psoralen ultraviolet A
    • With selective hand bath psoralen (4)
  • Low-level polarized polychromatic noncoherent light (LPPL) plus 0.1% methylprednisolone aceponate cream

Issues For Referral

If there is suspicion of psoriatic arthritis, refer to a rheumatologist.

Ongoing Care

Follow-up Recommendations

Long-term management by a dermatologist

Prognosis

  • Generally persistent, chronically relapsing course
  • May lead to severe disability or complications warranting hospitalization

Complications

  • Association with or progression to PPP or GPP
  • Psoriatic arthritis (possible association)
  • Pain and resultant loss of manual dexterity may have significant psychosocial impact.
  • Fever and systemic inflammatory response syndrome
  • Treatment-related complications, depending on agent used

Codes

ICD-10

  • L40.2 Acrodermatitis continua

ICD-9

  • 696.1 Other psoriasis

SNOMED

  • 200976008 Acrodermatitis continua (disorder)
  • 58143000 Localized acrodermatitis continua of Hallopeau (disorder)
  • 83839005 Acrodermatitis continua of Hallopeau

Clinical Pearls

  • Rare variant of pustular psoriasis that preferentially affects the fingers and toes, often leading to significant pain and loss of function
  • Diagnosis typically made by punch biopsy, is classically refractory to treatment, although case reports show that a variety of agents may have limited efficacy. Adalimumab may be the most promising option.

Authors


Amulya D. Amirneni, MD
Sudeep K. Aulakh, MD, FACP, FRCPC

Bibliography

  1. Sugiura K. The genetic background of generalized pustular psoriasis: IL36RN mutations and CARD14 gain-of-function variants. J Dermatol Sci. 2014;74(3):187–192.  [PMID:22431771]
  2. Abbas O, Itani S, Ghosn S, et al. Acrodermatitis continua of Hallopeau is a clinical phenotype of DITRA: evidence that it is a variant of pustular psoriasis. Dermatology. 2013;226(1):28–31.  [PMID:24656634]
  3. Razera F, Olm GS, Bonamigo RR. Neutrophilic dermatoses: part II. An Bras Dermatol. 2011;86(2):195–211. [PMID:21603801]
  4. Sehgal VN, Verma P, Sharma S, et al. Acrodermatitis continua of Hallopeau: evolution of treatment options. Int J Dermatol. 2011;50(10):1195–1211. [PMID:21950286]
  5. Di Costanzo L, Napolitano M, Patruno C, et al. Acrodermatitis continua of Hallopeau (ACH): two cases successfully treated with adalimumab. J Dermatolog Treat. 2014;25(6):489–494.  [PMID:17073995]


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TY - ELEC T1 - Acrodermatitis Continua ID - 816294 Y1 - 2019 PB - 5-Minute Clinical Consult, Updating UR - https://im.unboundmedicine.com/medicine/view/5-Minute-Clinical-Consult/816294/all/Acrodermatitis_Continua ER -