bubble_chart Overview

Enteropathic Acrodermatitis is a rare chronic skin disease. It was first reported by Brandt in 1936, hence also known as Brandt syndrome. In 1942, Danbolt and Closs provided a detailed description of the condition, leading to its alternative name, Danbolt-Closs syndrome. The main characteristics of this disease include skin erosion, alopecia, nail dystrophy, paronychia, and gastrointestinal dysfunction. It commonly occurs in infants aged 3 weeks to 18 months.

bubble_chart Etiology

The cause of the disease remains unclear. It may be due to insufficient secretion of pancreatic enzymes. Some have also suggested it is inherited recessively. Recent studies indicate that hereditary zinc deficiency may be related to the occurrence of this disease.

bubble_chart Pathogenesis

It may be due to impaired zinc absorption affecting the structure or activity of certain enzymes, leading to metabolic disorders such as tryptophan or fatty acid metabolism, which produce toxic substances and thus contribute to the onset of the disease.

bubble_chart Pathological Changes

Skin biopsy often shows nonspecific changes. Subcorneal blisters, acanthosis, edema, and perivascular infiltration of polymorphonuclear neutrophils and eosinophils can be observed.

bubble_chart Clinical Manifestations

1. Skin lesions The primary eruption is blisters, initially with clear fluid that later becomes turbid due to secondary infection. The lesions coalesce to form patches of varying sizes, surrounded by erythema, symmetrically distributed around skin and mucous membrane orifices and their surroundings. Other common sites include the hands and feet (especially around the nails), flexural areas (such as the anterior neck, inner thighs, etc.), elbows, knees, scalp, eyes, nose, etc. Later, the lesions crust over, with affected areas becoming dry, erythematous, and desquamated, presenting a psoriasis-like appearance with well-defined borders.

2. Gastrointestinal symptoms Approximately 90% of patients experience gastrointestinal symptoms, primarily diarrhea, occurring 3–6 times daily, often with large amounts of grayish-white, mucus-laden, fatty stools. Additionally, symptoms such as loss of appetite, abdominal distension, and fullness may occur.

3. Hair loss The hair becomes sparse or completely lost; eyebrows and eyelashes may also fall out. By adulthood, even axillary, pubic, and vellus hair may fail to grow.

4. Others Some patients may also exhibit growth retardation, intellectual disability, mental apathy, rhinitis, angular cheilitis, stomatitis, glossitis, conjunctivitis, blepharitis, paronychia, nail atrophy, and reduced resistance, making them prone to systemic or local secondary infections.

The above symptoms tend to recur intermittently, with periods of improvement and worsening. Secondary infections with Candida albicans and Staphylococcus are common. Candida may often be cultured from stool and skin lesions, but antifungal treatment does not improve the dermatitis.

bubble_chart Treatment Measures

In terms of treatment, zinc preparations and diiodohydroxyquinoline are effective for this disease. Zinc sulfate is administered at 0.2g per dose for adults, three times daily. Diiodohydroxyquinoline is given at 40mg/kg per day, divided into three oral doses. If the condition improves, treatment can be continued for several months, with gradual dose reduction as symptoms alleviate. In cases of intestinal dysfunction, attention should be paid to feeding and appropriate fluid replacement. Additionally, maintaining skin cleanliness is essential to prevent secondary infections. If bacterial or Candida infections occur, antibiotics and nystatin should be used for treatment.