Dennie–Marfan syndrome

Overview

Dennie–Marfan syndrome is an outdated and largely historical term that was once used to describe a constellation of features seen in children with atopic dermatitis, particularly in those with severe allergic backgrounds. It is not recognized as a distinct syndrome in modern medical classifications but refers to a group of physical characteristics, such as facial features and skin changes, often associated with chronic atopy (a genetic tendency to develop allergic conditions).

Causes

As Dennie–Marfan syndrome is not a standalone genetic or pathological condition, its “causes” are essentially linked to atopic dermatitis and allergic disorders. These include:

• Genetic predisposition: Family history of eczema, asthma, or allergic rhinitis.

• Immune system dysregulation: Overactive immune responses to allergens or irritants.

• Environmental triggers: Exposure to allergens such as dust mites, pollen, pet dander, or certain foods.

Symptoms

Children historically described as having Dennie–Marfan syndrome often presented with the following signs, now considered features of chronic atopic dermatitis:

• Dennie–Morgan folds: Extra skin creases or folds under the lower eyelids, a common finding in allergic individuals.

• Atopic facial features: Includes a tired or “allergic” facial appearance with dark under-eye circles (allergic shiners) and pale skin.

• Dry, itchy skin: A hallmark of eczema, especially in flexural areas like elbows and knees.

• Recurrent allergic symptoms: May include asthma, allergic rhinitis, and sensitivity to environmental triggers.

Diagnosis

Since Dennie–Marfan syndrome is not a medically defined disorder today, diagnosis typically involves identifying atopic dermatitis and associated allergic conditions. Diagnostic steps include:

• Clinical evaluation: Assessment of skin symptoms, family history of atopy, and the presence of typical facial or dermatological signs.

• Allergy testing: Skin prick or blood tests to detect specific allergens triggering symptoms.

• Diagnostic criteria for atopic dermatitis: Based on features such as pruritus, chronic relapsing course, and typical distribution of lesions.

Treatment

Management focuses on controlling atopic dermatitis and addressing associated allergic issues. Treatment options include:

• Topical corticosteroids: To reduce inflammation and itching.

• Moisturizers and emollients: Daily use to repair the skin barrier and prevent dryness.

• Antihistamines: For relief of itching and other allergy symptoms.

• Allergen avoidance: Identifying and minimizing exposure to environmental triggers.

• Immunomodulators: Such as tacrolimus or pimecrolimus for long-term control.

• Advanced therapies: For severe or resistant cases, systemic treatments or biologics like dupilumab may be used.

Prognosis

The prognosis for individuals with features historically associated with Dennie–Marfan syndrome is generally good with proper management of their atopic dermatitis and allergies. Many children outgrow some symptoms as they age, although atopic tendencies often persist into adulthood. With consistent skin care, trigger avoidance, and modern treatment options, most individuals can maintain good skin health and lead normal, active lives.