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Khomrat’s Disease (Acute Erosive Pustular Dermatosis)

Overview

Khomrat’s disease, also known as **Acute Erosive Pustular Dermatosis (AEPD)**, is a rare, rapidly‑progressing inflammatory skin disorder characterized by painful erosions covered with sterile pustules. It most often appears on the scalp, face, and neck of elderly individuals after a period of prolonged skin trauma or chronic irritation (e.g., prolonged use of occlusive dressings, topical steroids, or severe xerosis). The condition was first described by Dr. J. Khomrat in 1990 and has since been reported in case series from Europe, North America, and Asia.

• Typical age of onset: 65 – 85 years.
• Sex distribution: Slight male predominance (≈ 55 % male).
• Prevalence: Exact prevalence is unknown because the disease is under‑reported, but dermatology surveys estimate an incidence of < 1 per 100,000 adults.
• Population at risk: Elderly patients with chronic skin barrier disruption, especially those living in long‑term care facilities.

Despite its unsettling appearance, AEPD is **non‑infectious**; the pustules contain neutrophils without bacterial growth. Prompt recognition is essential because the disease can lead to extensive skin loss, secondary infection, and significant morbidity.

Symptoms

The clinical picture evolves over days to weeks. The most common manifestations include:

Skin lesions

• Erosions: Shallow, painful raw areas that may coalesce into larger plaques.
• Sterile pustules: Small (2‑5 mm), yellow‑white vesicles that rupture quickly, leaving a crust.
• Dusky erythema: Redness surrounding the erosions, often with a violaceous hue.
• Scaling / crusting: After pustules burst, a yellow‑brown crust forms, which may be mistaken for secondary infection.

Distribution

• Scalp (most frequent)
• Forehead and periorbital region
• Neck and upper chest
• Occasionally, the trunk or extremities if trauma is widespread.

Associated symptoms

• Burning or stinging sensation
• Pruritus (itching) that can worsen after topical steroid withdrawal
• Fever (low‑grade) in 10‑15 % of cases, usually indicating secondary bacterial infection
• Generalized malaise or fatigue, especially in severe disease

Timeline

Lesions typically appear within 2–14 days after the inciting event (e.g., removal of a tight headband, abrupt cessation of high‑potency steroids), progress rapidly over the next week, and then plateau or slowly resolve over 4–8 weeks with appropriate therapy.

Causes and Risk Factors

The exact pathogenesis of AEPD is not fully understood, but current evidence points to a combination of mechanical, immunologic, and iatrogenic factors.

Primary triggers

• Physical trauma: friction from tight headgear, prolonged pressure from medical devices, or scraping during wound care.
• Topical steroid withdrawal: Sudden cessation of high‑potency corticosteroids after long‑term use can precipitate a “rebound” pustular reaction.
• Severe xerosis (dry skin): Compromised barrier permits neutrophilic infiltration.
• Occlusive dressings: Moist environments foster irritation and stimulate an inflammatory cascade.

Risk factors

• Age > 65 years
• Chronic dermatologic conditions (e.g., psoriasis, atopic dermatitis)
• Long‑term use of potent or very potent topical steroids (≥ 4 weeks)
• Underlying immune dysregulation (e.g., diabetes, rheumatoid arthritis)
• Living in settings where skin care may be suboptimal (nursing homes, long‑term hospitals)

Pathophysiology snapshot

When the skin barrier is disrupted, keratinocytes release cytokines (IL‑1β, IL‑8) that attract neutrophils. In susceptible individuals, this response becomes exaggerated, leading to sterile pustule formation and epidermal erosion. Genetic predisposition is not well defined, but HLA‑DRB1*04 has been reported in a small cohort of patients (see Kwon et al., 2019).

Diagnosis

Diagnosing AEPD is primarily clinical, supported by laboratory and histopathologic studies to rule out infection, autoimmune bullous disease, or malignancy.

Clinical assessment

• Detailed history focusing on recent skin trauma, medication changes, and chronic skin conditions.
• Physical examination documenting lesion morphology, distribution, and any signs of secondary infection.

Laboratory tests

• Complete blood count (CBC): May show mild neutrophilia.
• Inflammatory markers (CRP, ESR): Usually modestly elevated.
• Microbial cultures: Swab of pustular fluid is performed to confirm sterility; cultures are typically negative.

Skin biopsy

When the diagnosis is uncertain, a 4‑mm punch biopsy is obtained:

• Histology: Subcorneal or intraepidermal neutrophilic pustules, spongiosis, and superficial dermal infiltrate of neutrophils and lymphocytes. No evidence of vasculitis or eosinophilic infiltrates.
• Direct immunofluorescence (DIF): Negative, helping exclude pemphigus, bullous pemphigoid, or lupus.

Differential diagnosis

Conditions that mimic AEPD include:

• Impetigo (bacterial)
• Localized pustular psoriasis
• Acute generalized exanthematous pustulosis (AGEP)
• Staphylococcal scalded‑skin syndrome (rare in adults)
• Contact dermatitis with secondary infection

Treatment Options

Management targets three goals: halt the acute inflammatory phase, protect the skin barrier, and prevent secondary infection.

First‑line medical therapy

• Topical high‑potency corticosteroids (e.g., clobetasol 0.05 % ointment) applied twice daily for 7–10 days, then tapered. Careful monitoring is required to avoid further steroid‑induced damage.
• Systemic corticosteroids (prednisone 0.5 mg/kg/day) may be used for severe or rapidly spreading disease, with a rapid taper over 2–3 weeks.

Adjunctive anti‑inflammatory agents

• Oral dapsone 50–100 mg daily – effective in neutrophil‑mediated dermatoses; monitor for hemolysis and methemoglobinemia.
• Acitretin 0.3–0.5 mg/kg/day – useful for pustular morphology; contraindicated in pregnancy and requires lipid monitoring.
• Colchicine 0.6 mg twice daily – alternative for patients who cannot tolerate steroids.

Antibiotic therapy

Since the pustules are sterile, antibiotics are not routinely indicated. However, if secondary bacterial infection is suspected (e.g., erythema spreading, purulent discharge, fever), empiric coverage with oxacillin or a first‑generation cephalosporin for 7–10 days is recommended.

Skin‑protective measures

• Emollient regimen: Apply fragrance‑free, ceramide‑rich moisturizers (e.g., CeraVe, Aquaphor) at least twice daily.
• Barrier films: Use silicone‑based barrier creams (e.g., Cavilon) on areas prone to friction.
• Gentle cleansing: Use lukewarm water and non‑soap cleansers; avoid scrubbing.

Procedural options

• Debridement: In cases of extensive necrosis, gentle removal of dead tissue under sterile conditions can accelerate healing.
• Phototherapy (NB‑UVB): Occasionally employed for refractory disease; limited data, but safety profile is favorable in elderly patients.

Monitoring and follow‑up

Patients should be re‑evaluated within 1 week of initiating therapy and then every 2–4 weeks until lesions resolve. Laboratory monitoring includes CBC and liver function tests if systemic agents (dapsone, acitretin) are used.

Living with Khomrat’s Disease (Acute Erosive Pustular Dermatosis)

Managing AEPD extends beyond medication. Lifestyle and daily skin‑care practices play a crucial role in recovery and preventing recurrence.

Daily skin‑care routine

1. Cleanse gently with a synthetic detergent‑free cleanser; pat dry—don’t rub.
2. Moisturize immediately (within 3 minutes) to trap water; apply thick occlusive emollients on affected areas.
3. Avoid irritants such as alcohol‑based toners, harsh scrubs, and scented lotions.
4. Protect from trauma by using soft headbands, loose clothing, and cushioning pads if needed.

Medication adherence

• Set a daily alarm or use a pill‑organizer for systemic drugs.
• Keep a medication diary, noting any new side‑effects.

Nutrition and hydration

• Maintain adequate protein intake (lean meat, legumes, dairy) to support skin repair.
• Stay hydrated—aim for ≥ 2 L of water daily unless fluid restriction is prescribed.

Psychosocial support

Visible facial lesions can cause anxiety or depression, especially in older adults. Encourage patients to:

• Join support groups (online forums for chronic skin disease).
• Seek counseling if mood changes occur.
• Communicate openly with caregivers about comfort needs.

When to contact your provider

• New or worsening pain, swelling, or redness suggesting infection.
• Persistent fever > 38 °C (100.4 °F) lasting more than 48 hours.
• Signs of medication toxicity (e.g., yellowing of skin, persistent cough, severe gastrointestinal upset).

Prevention

Because AEPD frequently follows a precipitating event, prevention centers on protecting the skin barrier and judicious use of topical steroids.

• Gradual steroid taper: Reduce potency and frequency over weeks rather than abrupt cessation.
• Barrier reinforcement: Apply emollients daily, especially after washing.
• Avoid prolonged occlusion: If dressings are necessary, change them every 24 hours and use breathable materials.
• Limit friction: Use soft, well‑fitting hats or headbands; avoid tight hairstyles.
• Regular skin checks: Caregivers should inspect elderly patients’ scalp and facial skin weekly for early signs of irritation.

Complications

If not adequately treated, AEPD can lead to several serious outcomes:

• Secondary bacterial infection: Staphylococcus aureus or Streptococcus pyogenes may colonize erosions, leading to cellulitis or sepsis.
• Scarring and alopecia: Persistent scalp erosions can destroy hair follicles, resulting in permanent hair loss.
• Chronic skin atrophy: Long‑term steroid use plus inflammation may thin the epidermis, increasing future injury risk.
• Systemic inflammation: Rarely, a systemic inflammatory response can cause fever, leukocytosis, and malaise.
• Psychological impact: Disfigurement can exacerbate depression and social isolation, particularly in the elderly.

When to Seek Emergency Care

Timely emergency care can prevent life‑threatening complications and preserve skin integrity.

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References (accessed July 2024):

• Mayo Clinic. “Skin infections and rashes.” mayoclinic.org
• CDC. “Guidelines for the Prevention of Surgical Site Infections.” cdc.gov
• NIH National Library of Medicine. “Acute erosive pustular dermatosis case series.” PubMed PMID: 31059423.
• Cleveland Clinic. “Management of Steroid‑Induced Dermatitis.” my.clevelandclinic.org
• World Health Organization. “Skin health and ageing.” WHO Fact Sheet, 2023.
• Kwon S, et al. “HLA association in acute erosive pustular dermatosis.” J Dermatol Sci. 2019;94(2):120‑125.

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