A dermatologist has a new patient that he has never seen before and does not know anything about. The patient is a 35 year old white male who presents with an acute case of pustular psoriasis, etiology unknown. What would be his initial work up plan and treatment plan?
Case Summary: A previously unknown 35-year-old white male presents to a dermatologist with an acute episode of pustular psoriasis of unknown trigger. The dermatologist needs to establish an initial diagnostic workup to confirm the diagnosis, identify potential systemic involvement and precipitating factors (e.g., infection, medications, steroid withdrawal, metabolic abnormalities), and formulate an urgent treatment plan focused on stabilizing the patient, controlling inflammation, and preventing complications.
Assessment Summary
35-year-old man with an acute pustular eruption clinically consistent with pustular psoriasis, trigger unknown. Given the acute onset, potential systemic involvement, and overlap with life-threatening mimickers (AGEP, severe infection, SJS/TEN), this should be managed as a dermatologic emergency until stabilized and diagnosis clarified.
Immediate priorities:
- Rapid assessment and stabilization of vitals, fluids, and electrolytes.
- Urgent evaluation for systemic involvement and infection.
- Early dermatology‑led systemic therapy once GPP/pustular psoriasis is strongly suspected and dangerous mimickers reasonably excluded.
- Identification/removal of possible triggers (medications, infection, steroid withdrawal, metabolic disturbances).
Differential Diagnosis / Working Diagnosis
Working diagnosis (provisional):
- Acute generalized pustular psoriasis (GPP) / acute pustular psoriasis flare.
Key differentials to actively evaluate and exclude:
- Acute generalized exanthematous pustulosis (AGEP)
- Often drug-induced (antibiotics, calcium-channel blockers, antimalarials).
- Rapid onset after new drug (hours–days), more prominent facial edema; pustules typically non-follicular, superficial.
- Severe bacterial infection / impetiginized pustulosis
- Staphylococcal or streptococcal infection; can mimic GPP.
- May have focal crusting, purulence, systemic sepsis; positive cultures.
- SJS/TEN
- Mucosal involvement, epidermal necrosis, positive Nikolsky sign, targetoid lesions.
- Strong drug association; life-threatening.
- Other pustular dermatoses
- Subcorneal pustular dermatosis, IgA pemphigus, pustular drug reactions.
- Pustular psoriasis variants
- Localized (palmoplantar) vs generalized; may have history of plaque psoriasis.
Workup Plan
1. Initial Triage and Bedside Assessment (first encounter, 0–2 hours)
- Vital signs: BP, HR, RR, temperature, SpO₂.
- General status: Level of consciousness, oral intake, urine output.
- Skin exam:
- Distribution and extent of pustules (generalized vs localized).
- Erythema, tenderness, edema.
- Presence/absence of skin detachment or erosions.
- Nikolsky sign.
- Signs of secondary infection (honey-colored crusts, purulence, foul odor).
- Mucosal exam: Oral, ocular, genital involvement (to evaluate SJS/TEN).
- Lymph nodes: Cervical, axillary, inguinal.
- Joint exam: Look for arthritis (psoriatic arthritis).
Disposition decision:
- Admit (preferably to monitored bed or ICU step‑down) if:
- Fever, tachycardia, hypotension, or systemic toxicity.
- Extensive body surface area involved (e.g., >10–15% with pustules/erythroderma).
- Dehydration, inability to maintain oral intake.
- Renal impairment, electrolyte derangements.
- Concern for SJS/TEN or sepsis.
- Outpatient management only if:
- Stable vitals, limited skin involvement, no systemic symptoms, strong outpatient support, and quick follow-up possible.
Given “acute case of pustular psoriasis” and unknown status, plan as inpatient or urgent admission unless clearly mild.
2. Laboratory Studies
Order STAT baseline labs to assess systemic involvement, triggers, and therapy safety:
General / systemic:
- CBC with differential
- Look for leukocytosis (common in GPP, AGEP, infection), anemia, thrombocytopenia.
- CMP (electrolytes, BUN, creatinine, liver enzymes, albumin, glucose)
- Assess dehydration, renal injury, hepatic function.
- CRP and/or ESR
- Inflammatory burden; help follow response to therapy.
- Serum calcium, magnesium, phosphate
- Hypocalcemia can trigger or worsen pustular psoriasis.
- Serum lactate (if septic appearance).
- Blood cultures (×2 sets) if fever, rigors, or hypotension.
Infection-focused:
- Throat swab for Group A Streptococcus (rapid test ± culture) if sore throat or suggestive history.
- Urinalysis ± urine culture if urinary symptoms.
- Chest X-ray (see imaging below) if respiratory signs or systemic sepsis suspicion.
- Swab cultures from pustules/erosions:
- Bacterial culture and gram stain.
- Consider fungal culture if atypical features.
Medication / trigger workup:
- Detailed medication history (within past 4–6 weeks):
- New drugs: antibiotics (beta‑lactams), antimalarials, lithium, terbinafine, NSAIDs, ACE‑inhibitors, calcium-channel blockers, biologics.
- Recent systemic corticosteroid use and abrupt withdrawal.
- Changes in antipsoriatic agents or biologics.
- Alcohol and substance history.
Autoimmune/other (as indicated):
- ANA, serum protein electrophoresis (if atypical or chronic recurrent).
- HIV test if risk factors or unclear immunosuppression status.
Baseline for systemic therapy selection/safety:
- Viral hepatitis panel (HBsAg, anti‑HBc, anti‑HBs, HCV Ab) if considering immunosuppressants or biologics.
- Quantiferon-TB Gold or T-spot (if possible before biologic) – may proceed later if urgent but start planning.
- Fasting lipids (for acitretin, if time allows).
- G6PD level (if thinking of dapsone, less typical first-line but occasionally used in other pustular conditions).
3. Imaging
- Chest X-ray if:
- Fever, cough, dyspnea, systemic toxicity (rule out pneumonia, pulmonary edema).
- Echocardiogram / further imaging only if clinically indicated by sepsis or organ involvement.
4. Procedures
Skin biopsy (urgent):
- Perform at least one punch biopsy (4 mm ideally) from a fresh pustule on erythematous skin.
- Request H&E and direct immunofluorescence (DIF) if SJS/TEN, IgA dermatosis, or autoimmune etiology is a concern.
- Optional second biopsy from edge of involved area if needed to sample different morphology.
Cultures:
- Swab pustules before they are significantly treated:
- Bacterial culture and gram stain.
- Fungal culture if morphology atypical or immunosuppressed.
These will help distinguish GPP from AGEP, infections, and other neutrophilic dermatoses.
Treatment Plan
1. General Stabilization and Supportive Care (0–24 hours)
-
Setting: Inpatient (dermatology + internal medicine; consider ICU step‑down for unstable patients).
-
Fluids & electrolytes:
- Start IV fluids if oral intake inadequate or signs of dehydration (use isotonic saline or balanced crystalloid).
- Correct electrolyte abnormalities, particularly hypocalcemia, potassium, and magnesium.
-
Temperature and environment:
- Maintain neutral thermal environment; avoid overheating.
- Active cooling if febrile and uncomfortable (cool compresses, antipyretics).
-
Skin barrier and wound care:
- Liberal application of bland emollients (e.g., petrolatum, Aquaphor, thick fragrance-free ointments) to all involved skin multiple times daily.
- Non-adherent dressings for erosive or exudative areas to reduce pain and fluid loss.
-
Pain and pruritus control:
- Oral acetaminophen for pain/fever (avoid high-dose NSAIDs if renal risk).
- Systemic antihistamines (e.g., cetirizine 10 mg daily ± hydroxyzine 25 mg at night) for itch and sleep.
-
Infection prophylaxis/management:
- Strict skin hygiene; avoid unnecessary invasive lines.
- Start empiric antibiotics only if there is strong clinical suspicion of secondary infection or sepsis while awaiting cultures (e.g., IV anti‑staphylococcal agent ± broader coverage as per local guidelines).
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Medication review and trigger removal (same day):
- Immediately stop potential culprit drugs, especially:
- Recent antibiotics (e.g., beta‑lactams) if AGEP suspected.
- Lithium, terbinafine, antimalarials, or any highly suspect recent new medication.
- Do NOT abruptly stop corticosteroids if the patient is currently on a significant systemic dose; abrupt withdrawal is a known trigger. If on steroids, plan a careful taper once disease is controlled and alternative therapy in place.
- Document timing of each medication relative to eruption onset.
- Immediately stop potential culprit drugs, especially:
2. Systemic Therapy for Acute Pustular Psoriasis (once life-threatening mimickers reasonably excluded or in parallel if high suspicion)
Choice depends on severity, comorbidities, and availability. In a previously healthy 35-year-old, aim for rapid-acting systemic therapy.
A. First-line options (acute severe GPP):
-
Cyclosporine (rapid onset)
- Dose: 3–5 mg/kg/day PO divided BID (e.g., 75–150 mg BID depending on weight).
- Indications: Severe, generalized pustular eruption with systemic symptoms, need for rapid control, normal baseline kidney function and BP.
- Monitoring:
- Baseline and twice-weekly in acute phase: BP, BUN/Cr, potassium, magnesium.
- Watch for hypertension, nephrotoxicity, infection.
- Duration:
- Expect clinical improvement within days to 1–2 weeks.
- Once controlled, plan a gradual taper over several weeks while transitioning to maintenance therapy (e.g., biologic, acitretin, or conventional systemic).
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Acitretin
- Dose: 0.5–1 mg/kg/day PO (e.g., 25–50 mg daily, adjust by weight and tolerance).
- Pros: Effective for pustular psoriasis, non-immunosuppressive.
- Cons: Slower onset than cyclosporine; teratogenic (not relevant for male patient in terms of pregnancy but still long-term blood donation precautions).
- Monitoring:
- Baseline and periodic: LFTs, fasting lipids.
- Often used as either:
- Monotherapy in less acute/systemic cases, or
- Combined with cyclosporine initially then maintained alone after cyclosporine taper.
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Biologic therapy targeting IL‑36R (if available and confirmed GPP):
- Spesolimab (where approved; GPP‑specific).
- Dosing regimen per local approvals (often single IV dose with potential re-dosing).
- Typically initiated in specialized centers; ensure infection screening (TB, hepatitis) as feasible.
B. Alternative acute options (if above unavailable/contraindicated):
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Methotrexate
- Dose: 10–25 mg once weekly PO or SC, plus folic acid 1 mg daily (excluding methotrexate day).
- Slower onset than cyclosporine, but reasonable for concurrent or maintenance therapy.
- Monitoring: CBC, LFTs, creatinine at baseline, then every 1–2 weeks initially.
-
Systemic corticosteroids (caution; generally avoided as primary therapy in pustular psoriasis because of risk of rebound/worsening on withdrawal).
- Consider only in select situations (e.g., GPP overlap with other steroid-responsive disease or no immediate access to other systemic therapies) and with a clear plan for slow taper and bridging to another agent (e.g., cyclosporine, methotrexate).
- If used: e.g., prednisone 0.5–1 mg/kg/day with early addition of another systemic and tapered over weeks.
Avoid:
- Abrupt cessation of existing systemic steroids.
- Monotherapy with topical steroids alone in severe generalized disease (insufficient).
3. Topical Treatments (adjunctive)
Use in addition to systemic therapy:
-
High-potency topical corticosteroids (e.g., clobetasol 0.05% ointment/cream):
- Apply BID to localized, very inflamed plaques or hands/feet.
- In widespread disease, consider short-term use under supervision; avoid occlusion over very large areas to reduce systemic absorption.
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Topical calcipotriene (calcipotriol) or combination calcipotriene/betamethasone:
- More for transition to maintenance after acute pustules subside, especially if residual plaque psoriasis is present.
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Antiseptic washes:
- Chlorhexidine wash once daily to reduce bacterial colonization (avoid in case of known allergy).
4. Lifestyle and Supportive Measures
- Encourage adequate oral hydration (if able) and balanced nutrition with sufficient protein.
- Avoid alcohol and unnecessary OTC medications or supplements.
- Gentle skin care:
- Lukewarm showers, fragrance-free cleansers.
- Avoid scrubbing, hot baths, or harsh soaps.
- If smoker: discuss cessation as part of long-term psoriasis control.
- Screen for and manage psychological stress; offer support or referral if distress is high.
Patient Education
Key points to explain in simple terms:
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Nature and seriousness of the condition
- “You have a severe type of psoriasis where the skin suddenly forms many tiny blisters filled with white cells. This can affect the whole body and stress your organs, so we treat it urgently, similar to a medical emergency, until you are stable.”
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Why hospitalization/urgent care is important
- To monitor temperature, blood pressure, kidneys, and fluids.
- To give strong treatments and watch for side effects.
- To protect against dehydration, infection, and other complications.
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Triggers and medication review
- Emphasize:
- Possible role of recent medications (antibiotics, mood stabilizers, antifungals, antimalarials).
- Risk of flares with sudden stopping of oral steroids: “We avoid suddenly stopping steroids because it can trigger or worsen this condition.”
- Emphasize:
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Treatment expectations
- Systemic medication (e.g., cyclosporine ± acitretin or other) aims to quickly stop new pustules and reduce redness and pain.
- Improvement expected over days to a week, not instantly, but new pustules should slow within 24–72 hours.
- Need for regular blood tests to watch kidney, liver, and blood counts.
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Long-term outlook
- This may be a chronic condition with risk of future flares.
- After this acute episode, a longer-term maintenance plan will be needed (which may involve pills, injections, and/or topicals).
- Importance of regular follow-up with dermatology.
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Self-care at home (when discharged)
- Gentle moisturization several times daily.
- Avoid picking or scratching pustules/crusts.
- Take medications exactly as prescribed; do not stop systemic medications abruptly without talking to the dermatologist.
Follow-up Schedule
Inpatient phase:
- Daily dermatology and internal medicine review.
- Labs:
- CBC, CMP (incl. creatinine, electrolytes), CRP: every 1–2 days initially or more often if unstable.
- BP and urine output: at least every shift.
- Assess:
- Number of new pustules, extent of erythema.
- Pain, pruritus, sleep.
- Signs of infection or organ dysfunction.
Transition to outpatient:
- Discharge when:
- Hemodynamically stable; no fever or minimal low-grade only.
- New pustule formation significantly reduced or stopped.
- Able to maintain oral intake and skin care at home.
- Clear systemic regimen and lab plan established.
Post-discharge follow-up:
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1st visit: within 1 week of discharge.
- Review clinical progress, adjust systemic therapy/tapering.
- Repeat CBC, CMP, and specific drug-monitoring labs (e.g., creatinine for cyclosporine, LFTs/lipids for acitretin).
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Subsequent visits:
- Every 2–4 weeks initially while adjusting therapy.
- Then every 3 months once stable and on maintenance therapy.
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Discuss and plan maintenance strategy at early follow-ups:
- Options: acitretin, methotrexate, biologics targeting IL‑17/23 or IL‑36 (depending on regulatory status and phenotype).
- Integrate any plaque psoriasis or psoriatic arthritis management.
Red Flags / When to Seek Immediate Care
Advise patient (and caregivers) to seek urgent/emergency medical attention if any of the following occur after discharge or during outpatient care:
- High fever (>38.5°C / 101.3°F) or shaking chills.
- Rapid increase in redness, warmth, or tenderness of skin; spreading areas of raw or weeping skin.
- Signs of sepsis: confusion, dizziness, rapid heartbeat, trouble breathing, very low urine output, or feeling faint.
- Sudden or severe eye pain, redness, or vision changes.
- New mouth, eye, or genital ulcers, or widespread skin peeling.
- Shortness of breath, chest pain, or severe cough.
- Severe abdominal pain, persistent vomiting, or diarrhea.
- Dark or greatly reduced urine, new leg swelling, or unexplained weight gain (possible kidney or heart issues).
- Severe headache, visual changes, or very high blood pressure.
- Any new rash or reaction shortly after starting a new medication.
This integrated plan addresses: immediate stabilization, thorough diagnostic workup (including trigger identification and exclusion of mimickers), rapid initiation of appropriate systemic therapy for acute pustular psoriasis, and structured follow-up with clear red-flag education.