- 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
- 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
- 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.
- 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.
- History of psoriasis, particularly pustular variant
- Digital trauma
- Local infection
- Smoking may contribute to exacerbations.
- Defined by clinical evolution and histopathologic features
- Frequently missed, often a diagnosis of exclusion
- 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
- 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.
- 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 & InterpretationInitial 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.
- 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
- Superficial perivascular inflammatory infiltrate composed of lymphocytes, histiocytes, and neutrophils
- Tortuous, dilated vessels displaying erythrocyte extravasation
- Edema of papillary dermis
- 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.
If cultures show secondary infection, treat with appropriate antibiotics.
- Application under occlusion enhances absorption. Topical agents generally have favorable side effect profiles but may be ineffective, especially as monotherapy:
- 5-Fluorouracil (4)
- Tacrolimus ointment, with or without occlusion (4)
- Anthralin (3)
- 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
- Cyclosporine (4)
- Acitretin: possible synergistic effect with topical calcipotriol (4)
- Sulfones, namely dapsone
- 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.
Long-term management by a dermatologist
- Generally persistent, chronically relapsing course
- May lead to severe disability or complications warranting hospitalization
- 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
- L40.2 Acrodermatitis continua
- 696.1 Other psoriasis
- 200976008 Acrodermatitis continua (disorder)
- 58143000 Localized acrodermatitis continua of Hallopeau (disorder)
- 83839005 Acrodermatitis continua of Hallopeau
- 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.
Amulya D. Amirneni, MD
Sudeep K. Aulakh, MD, FACP, FRCPC
- 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]
- 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]
- Razera F, Olm GS, Bonamigo RR. Neutrophilic dermatoses: part II. An Bras Dermatol. 2011;86(2):195–211. [PMID:21603801]
- 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]
- 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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