Acrodermatitis continua of Hallopeau in a 47-year-old female
- Author:
Kirk Llew V. Quijote
1
;
Jen-Christina Lourdes Q. Segovia
1
;
Karla Phoebe B. Castaños
1
;
Lalaine R. Visitacion
1
Author Information
- Publication Type:Journal Article
- From: Southern Philippines Medical Center Journal of Health Care Services 2024;10(1):1-
- CountryPhilippines
- Language:English
- Abstract: A 45-year-old female came to our clinic due to multiple pustules on her fingers. The lesions first appeared when she was 15 years old, starting as a few erythematous macules on her left thumb that eventually developed into painful pustules. The patient claimed that no relief was provided by analgesics and oral antibiotics. Over the next 11 years, the lesions on her left thumb gradually spread to all her fingers including those on her right hand, accompanied by the development of erosions, fissures, and scales, as well as intermittent joint pains and swelling. These symptoms remained unresponsive to multiple topical products, the names of which the patient could not recall. At 24 years old, the patient experienced onycholysis in the first to third digits of her left hand, which progressed to anonychia and eventually affected all the fingers in her left and right hands. When the patient was 38 years old, similar pustules, erosions, fissures, and scaling appeared on several toes. Due to increasing discomfort from lesions spreading to her toes, making it difficult to put on footwear, the patient sought consultation at our dermatology clinic. No symptoms related to pulmonary, gastrointestinal, and genitourinary systems were reported. The patient denied pain or immobility in other joints of her right hand, hair or scalp changes, and oral mucosal lesions. There was no family history of psoriasis, hypertension, peripheral arterial diseases, or other conditions with similar lesions. The patient denied any history of cigarette smoking, chronic alcohol intake, or illicit drug use. On physical examination, the patient appeared comfortable with no signs of distress. We observed multiple erythematous pustules, some coalescing into pus-filled lakes, and thick white hyperkeratotic plaques with scales located on the distal interphalangeal joints, extending to the tips of all digits on both hands. Similar pustules and plaques with scales were seen on the first metatarsophalangeal joint of the right foot and the first digit of the left foot, sparing only the patient’s palms and soles (Figure 1). Anonychia affected all fingers, and there was shortening of all digits on both hands. No hair changes, oral mucosal lesions, or lymphadenopathies noted. The rest of the physical examination findings were unremarkable. Based on the history and clinical findings, we initially assessed the patient as having a form of acropustulosis. Differential diagnoses included infections (e.g., herpetic whitlow, staphylococcal felon, candidal paronychia), which were ruled out due to finger involvement and lack of systemic symptoms. Malignant conditions (e.g., squamous cell carcinoma, acrometastasis) were excluded from the differential diagnoses due to the absence of other typical indicators like trauma, chronic paronychia, exposure to radiation or arsenic fumes, a history of cigarette smoking, or a primary tumor.1 2 Inflammatory conditions (e.g., dyshidrotic eczema, chronic hand contact dermatitis) were also ruled out due to the lack of prominent pruritus, burning sensation, and exposure to common irritants.3 4 Immune-mediated conditions (e.g., palmoplantar pustulosis, palmoplantar psoriasis) were considered but eventually ruled because the patient's lesions did not involve her palms and soles.4 At the time of our evaluation, the patient had normal results in hematology, lipid panel, and liver and kidney function tests. Radiography of the hands showed shortening of the distal phalanges on the first to third digits on both hands, good alignment of osseous structures, intact outlines and trabecular patterns, and normal joint spaces and soft tissue shadows. These findings are consistent with brachydactyly, frequently observed in cases of psoriasis manifesting with dactylitis. Gram stain of the pustules yielded negative results. Histopathology from a skin punch specimen taken from an erythematous plaque on the third digit of the left hand showed focal parakeratosis overlying a spongiotic epidermis with hypogranulosis and psoriasiform hyperplasia. The dermis showed superficial dermal edema and moderately dense perivascular inflammatory infiltrates composed predominantly of lymphocytes and some neutrophils (Figure 1). The final histopathologic impression was psoriasiform dermatitis, ruling out other possible differential diagnoses, including infectious, malignant, and inflammatory conditions. Given the histopathologic consistency with psoriasis, coupled with the clinical presentation of multiple pustules on the tips of the digits and nail changes, the clinicopathologic final diagnosis was acrodermatitis continua of Hallopeau. We initiated oral methotrexate at 10 mg/week for 3 weeks, followed by an increase to 15 mg/week for 7 weeks, reaching a cumulative dose of 135 mg. The patient also received oral folic acid 5 mg daily on days without methotrexate and topical clobetasol propionate 0.05% ointment applied twice daily with occlusion at night. We provided counseling and education about the chronic nature of her condition, emphasizing the need for follow-up every 3 to 6 months. Most pustules resolved, and no new lesions were observed during the tenth week of treatment. No adverse events were reported, and erythema and scaling were significantly lessened. Acrodermatitis continua of Hallopeau (ACH) is a rare form of localized pustular psoriasis characterized by recurrent chronic eruptions of sterile pustules, especially affecting the distal regions of the fingers and toes, and occasionally the nail beds.4 5 The pathophysiology remains poorly understood, but a few authors attribute it to mutations in the interleukin-36RN gene.6 7 8 Diagnosis of ACH can be established based on clinical features. Histopathologic examination and laboratory tests may be helpful in difficult cases, but they are not necessarily performed in all patients.4 6 9 ACH is associated with a wide range of differential diagnoses including infectious paronychia of viral, bacterial, or fungal etiology, dishydrotic eczema, and infected contact dermatitis.4 10 11 Rarely, osteitis and osteolysis of the phalanges may occur in persistent or severe cases.4 6 12 Due to its chronic and relapsing nature, long-term therapeutic control of ACH is necessary to prevent complications.<13 Cirone et al> ACH is recalcitrant to available therapies, with no clear management guidelines or drugs achieving lasting remission.5 Progression to severe disease with irreversible complications is common, and even with treatment, ACH often recurs, affecting patients' physical and psychological well-being.
