Ring around the rosie. Pocket full of posies & A medical case with ring lesions

 The nursery rhyme is:

 "Ring around the rosie,

A pocket full of posies,

Ashes! Ashes! 

,
We all fall down!"

Medical case:

A patient, who is a 35-year-old white male, has target lesions that look like a ring around a red spot or bump, on his hands and trunk, and also mucosal lesions. There are also plaques and subcutaneous nodules on the trunk. There is also a fever.

Assessment Summary

35-year-old man with acute onset of:

• Target / targetoid lesions on hands and trunk
• Mucosal involvement (oral/other not fully specified)
• Tender plaques and subcutaneous nodules on trunk
• Fever

This constellation is concerning for a serious acute dermatosis. Priority is to:

• Rapidly exclude / treat Stevens–Johnson syndrome (SJS) / toxic epidermal necrolysis (TEN)
• Distinguish erythema multiforme (EM) major from early SJS/TEN
• Evaluate for neutrophilic dermatosis (Sweet syndrome) and panniculitis (including erythema nodosum–like process)
• Consider other systemic drug/infection-related dermatoses (DRESS, vasculitis, etc.)

Immediate in-person assessment and likely hospital-level care are warranted.

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Differential Diagnosis / Working Diagnosis

Top priorities to rule out urgently

1. SJS/TEN spectrum

   • Features in favor: targetoid lesions involving trunk, fever, mucosal involvement, possible pain.
   • Must urgently assess: extent of epidermal detachment, Nikolsky sign, skin pain severity, recent high-risk drug exposures.

2. Erythema multiforme (EM) major

   • Classic target lesions, often acral, may involve trunk; 1+ mucosal site; often HSV or Mycoplasma-triggered.
   • Typically minimal or no widespread skin detachment.

3. Mycoplasma-induced rash and mucositis (MIRM)

   • Prominent mucosal disease with relatively fewer skin lesions; evaluate if respiratory prodrome.

4. Sweet syndrome (acute febrile neutrophilic dermatosis)

   • Fever with tender edematous erythematous plaques or nodules on upper trunk; may be pseudovesicular.
   • Often neutrophilia; may associate with infections, IBD, malignancy, drugs.

5. Panniculitis / erythema nodosum–like process

   • Subcutaneous tender nodules (classically shins but can involve trunk in some panniculitides).
   • Often triggered by infection, IBD, sarcoid, TB, or drugs.

Other important considerations

• DRESS (drug reaction with eosinophilia and systemic symptoms)
• Urticarial vasculitis or small-vessel vasculitis (if purpura/painful lesions, systemic involvement)
• Fixed drug eruption (if localized recurring plaques at same sites)
• Autoimmune bullous disease with targetoid lesions (less likely, but DIF biopsy should exclude)

Working approach:
Treat and triage as possible EM major vs early SJS/TEN, with concurrent evaluation for Sweet syndrome vs panniculitis driving the subcutaneous nodules.

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Workup Plan

1. Immediate Triage (First 0–2 Hours)

• ABCs and Vital Signs

  • Airway, breathing, circulation; full vitals including pain score.
  • If hypotensive, tachycardic, tachypneic, altered, or with extensive skin pain/erosions → ED/ICU.

• Level of care decision

  • ICU/burn unit if:
    • Epidermal detachment >10% BSA OR rapidly progressing painful erosions
    • Positive Nikolsky/Asboe-Hansen signs
    • ≥2 mucosal sites with erosions + systemic toxicity
  • Inpatient ward if:
    • Fever + mucosal involvement but minimal/no detachment; still needs IV fluids, monitoring, biopsy.
  • Outpatient only if:
    • Hemodynamically stable, minimal mucosal disease, classic limited EM pattern, reliable follow-up, no red flags.

• Immediate actions (regardless of location)

  • Stop all non-essential medications; note start/stop dates of all drugs within past 8 weeks.
  • Establish IV access; fluids as needed.
  • Analgesia (e.g., IV/PO acetaminophen ± opioids as appropriate).
  • Non-adherent dressings on eroded areas.
  • Photograph lesions and estimate BSA involvement (rule of nines or Lund–Browder).
  • Check Nikolsky and Asboe–Hansen signs and document.

2. Focused History (Rapid but thorough)

Ask specifically:

• Timeline
  • Onset of rash; progression over hours–days; new lesions in last 24–48 h.
  • Onset of mucosal symptoms (oral, ocular, genital, anal, nasal).
• Medications (past 8 weeks)
  • Sulfonamide antibiotics, penicillins/cephalosporins, allopurinol, anticonvulsants (e.g., lamotrigine, carbamazepine, phenytoin), NSAIDs, antiretrovirals, recent vaccines, G-CSF, others.
• Infections
  • HSV: history of cold sores/genital herpes; current or recent oral/genital vesicles/ulcers.
  • Respiratory: cough, sore throat, atypical pneumonia symptoms → concern for Mycoplasma.
  • Recent strep pharyngitis or upper respiratory infection.
• Systemic symptoms
  • Fever, malaise, arthralgias, myalgias, GI symptoms (diarrhea, abdominal pain), dyspnea, chest pain, urinary symptoms.
• Comorbidities and exposures
  • IBD, sarcoidosis, TB exposure, travel, HIV risk, prior malignancy, hematologic symptoms (weight loss, night sweats, B-symptoms).
• Previous similar episodes
  • Recurrent targetoid eruptions suggesting recurrent EM.

3. Focused Physical Examination

Document in detail:

• Skin morphology and distribution
  • Classic three-zone targets (dusky center, pale ring, outer erythematous halo) vs flat atypical targets/dusky macules.
  • Acral (hands/feet) vs trunk predominance.
  • Presence of purpura, vesicles, bullae, erosions.
• Mucosal involvement
  • Sites: oral, ocular, genital, anal, nasal, urethral.
  • Severity: erosions, hemorrhagic crusts, pain, dysphagia, odynophagia, dysuria, urinary retention.
• Epidermal detachment/BSA
  • Estimate %BSA of frank denudation/erosion.
• Nikolsky/Asboe–Hansen
  • Positive suggests SJS/TEN.
• Ocular screen
  • Conjunctival injection, photophobia, discharge, vision changes.
• Subcutaneous nodules / plaques
  • Number, location (trunk vs extremities), tenderness, depth.
• Other systems
  • Lymphadenopathy, hepatosplenomegaly, joint swelling, lung exam.

4. Laboratory Studies

Order now:

• CBC with differential
  • Neutrophilia → consider Sweet, infection, AGEP.
  • Eosinophilia → consider DRESS, some drug reactions.
• Comprehensive metabolic panel
  • BUN/creatinine, electrolytes, bicarbonate, LFTs (ALT/AST, alk phos, bilirubin).
• Inflammatory markers
  • CRP and/or ESR.
• Glucose and BUN/Cr/bicarbonate (for SCORTEN if SJS/TEN suspected).
• Urinalysis
  • Evaluate for renal involvement (vasculitis, DRESS).
• Blood cultures and lactate
  • If febrile and toxic-appearing or hypotensive.
• Infectious testing
  • HSV PCR/NAAT from active vesicles/erosions (skin/mucosa) if feasible.
  • Mycoplasma pneumoniae PCR/serology.
  • Respiratory viral panel if clinically indicated.
  • Strep testing (throat culture/rapid; ASO titer) if pharyngitis and nodules suggest EN.
• Additional baseline tests
  • HIV Ag/Ab (immunocompromise, drug reaction risk).
  • Hepatitis panel (if DRESS or systemic therapy anticipated).
• If nodules strongly suggest EN/panniculitis
  • TB IGRA.
  • CXR (for sarcoid, TB, Mycoplasma; also part of EN and sarcoid evaluation).

5. Imaging

• Chest X-ray
  • If respiratory symptoms, suspected Mycoplasma, EN workup, or sarcoid.
• Additional imaging only if guided by systemic findings (e.g., CT if malignancy suspected with Sweet).

6. Procedures / Pathology

Arrange within 24 hours (sooner if feasible; do not delay stabilization):

1. Skin biopsies of targetoid lesion
   • Two 4-mm punch biopsies from a fresh representative lesion:
     • One from the active edge for routine H&E (to distinguish EM vs SJS/TEN vs vasculitis, etc.).
     • One perilesional for direct immunofluorescence (to exclude autoimmune bullous disease, characterize interface dermatitis).
2. Deep biopsy of subcutaneous nodule
   • Incisional or large punch (≥4 mm) including subcutis to assess for panniculitis versus neutrophilic dermatosis (Sweet).
   • Request special stains (PAS, GMS, AFB) and tissue cultures if infectious panniculitis is a concern.

7. Severity / Risk Stratification

• If SJS/TEN suspected:
  • Calculate SCORTEN on day 1 and day 3 (age, malignancy, HR, BUN, detached BSA, bicarbonate, glucose).
  • Baseline ECG if considering cyclosporine.
  • Pregnancy test where relevant.

8. Consultations

• Dermatology – urgent for bedside evaluation, biopsy, and treatment guidance.
• Ophthalmology – same day if any ocular symptoms or ocular mucosal involvement.
• Burn/ICU team if SJS/TEN features (significant detachment, positive Nikolsky, systemic toxicity).
• Urology/gynecology if significant genital mucositis or urinary retention.
• Infectious Disease if severe infection suspected or complex antimicrobial decisions.
• Rheumatology if systemic vasculitis or complex panniculitis suspected.
• Oncology/Hematology if Sweet or panniculitis with concerning labs (cytopenias, blasts, etc.).

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Treatment Plan

Management initially is supportive + trigger removal, with condition-specific therapy once diagnosis is clearer.

1. Universal / Initial Measures (Before Biopsy Results)

• Stop all suspect medications

  • Discontinue any non-essential drugs started within the past 1–8 weeks, especially high-risk SJS/TEN agents.
  • Document exact start and stop dates.

• Supportive Care

  • Fluids & Electrolytes: IV fluids as needed; monitor input/output.
  • Wound Care:
    • Non-adherent, sterile dressings on erosions/denudation.
    • Avoid harsh antiseptics; use gentle cleansers; maintain warm environment to reduce heat loss.
  • Pain Control:
    • Systemic: acetaminophen ± opioids; avoid NSAIDs if suspected culprits.
    • Topical: viscous lidocaine mixtures for oral mucosa (avoid swallowing large amounts).
  • Nutritional Support:
    • Soft/liquid diet if oral pain; consider enteral nutrition if intake inadequate.
  • Oral/Mucosal Care:
    • Bland mouthwash (saline/bicarbonate); sucralfate suspension or other barrier rinses as available.
  • Ocular Care:
    • Frequent preservative-free lubricating drops; ophthalmology to decide on topical corticosteroids/cyclosporine.

2. Condition-Specific Therapy (Tailored to Most Likely Diagnosis)

A. If SJS/TEN is suspected (or cannot be excluded and features suggest severity)

• Manage as medical emergency:
  • Admit to burn/ICU-capable unit.
  • Protocolized wound care, temperature regulation, infection prevention, DVT prophylaxis.
  • Close monitoring of fluids, electrolytes, renal and hepatic function.
• Systemic therapy (per local protocol and dermatology guidance)
  • Consider cyclosporine (e.g., 3–5 mg/kg/day divided doses) or TNF-α inhibitor (e.g., etanercept single or repeated dosing) early in course where evidence and institutional experience support them.
  • IVIG and systemic corticosteroids: evidence mixed; use only according to institutional protocols and individualized risk–benefit assessment.
• Avoid empiric high-dose systemic steroids unless clearly recommended by dermatology in this context.

B. If EM Major is favored over SJS/TEN (classic targets, minimal detachment, milder mucosal disease)

• Trigger-directed therapy
  • If HSV-associated EM is likely:
    • Start valacyclovir or acyclovir (dosing per local guideline, e.g., valacyclovir 1 g PO TID for 7–10 days for acute episode, adjusted for renal function).
  • If Mycoplasma likely (respiratory prodrome):
    • Start macrolide (e.g., azithromycin) or doxycycline per local protocol.
• Anti-inflammatory therapy
  • Topical corticosteroids (e.g., medium–high potency for body plaques; low potency for face/genitals).
  • For severe mucosal involvement and once SJS/TEN has been reasonably excluded:
    • Short course systemic corticosteroids (e.g., prednisone 0.5–1 mg/kg/day with brief taper) may be considered under dermatology guidance.
• For recurrent HSV-associated EM (long-term plan):
  • Consider suppressive antivirals (e.g., valacyclovir 500–1000 mg daily) for 6–12 months.

C. If Sweet Syndrome is suspected/confirmed

• After infection is reasonably excluded and biopsy supports neutrophilic dermatosis:
  • Systemic corticosteroids are first line:
    • Prednisone ~0.5–1 mg/kg/day; expect rapid improvement (often within 24–48 h); gradual taper over weeks according to response.
  • Alternatives / steroid-sparing agents:
    • Colchicine, dapsone, potassium iodide depending on comorbidities and tolerance.
  • Evaluate for associated conditions:
    • Malignancy (especially hematologic), IBD, rheumatologic disease, drug-induced causes.

D. If Panniculitis / Erythema Nodosum-like Process is identified

• Treat underlying cause:
  • Strep: antibiotics per guidelines.
  • Sarcoidosis: systemic evaluation and treatment per specialist.
  • IBD: coordinate with gastroenterology.
  • TB: anti-tuberculous therapy.
• Symptomatic treatment:
  • NSAIDs if not contraindicated (and not suspected as culprit).
  • Rest and, if leg involvement, elevation/compression.
  • For severe pain/swelling and infection ruled out:
    • Short course systemic corticosteroids or potassium iodide may be used under specialist guidance.

E. If DRESS, Vasculitis, or Other Drug Eruption is Diagnosed

• DRESS
  • Immediate cessation of culprit drug.
  • Systemic corticosteroids (e.g., prednisone 0.5–1 mg/kg/day) with slow taper over weeks–months.
  • Monitor liver, kidney, and thyroid function for ≥8–12 weeks.
• Urticarial vasculitis / Leukocytoclastic vasculitis
  • Workup: UA, renal function, complements, ANCA, hepatitis serologies.
  • Treatment severity-based: antihistamines/NSAIDs for mild; systemic corticosteroids or other immunosuppressants if organ involvement or hypocomplementemia.

Note: Avoid systemic corticosteroids until SJS/TEN is reasonably excluded or therapy is under dermatology/ICU guidance, as inappropriate steroids can affect infection risk and complicate SJS/TEN course.

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Patient Education (Once Stable)

Explain in clear language:

• The rash is potentially serious and may be related to a medication or infection.
• We are doing:
  • Blood tests, swabs, and skin biopsies to clarify the exact diagnosis.
  • Immediate stopping of possibly responsible drugs.
  • Supportive care to protect skin, mouth, and eyes and to prevent complications.
• Emphasize:
  • Importance of reporting any eye pain, difficulty swallowing, breathing problems, or new blisters/skin pain immediately.
  • Need to avoid any medication identified as the culprit in the future; this will be placed in the medical record and allergy list.
• If drug-related cause confirmed:
  • Provide a written list of drugs to avoid and possible cross-reacting medications.
  • Consider medical alert information (card/bracelet).
• For HSV-associated EM or other recurrent patterns:
  • Discuss potential long-term antiviral suppression and early treatment plans for flares.

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Follow-up Schedule

Inpatient

• Daily dermatology review (or equivalent consulting team).
• Daily reassessment of:
  • BSA involved, new lesions, mucosal involvement.
  • Vitals, fluid status, pain control, nutritional status.
  • Labs (CBC, CMP, CRP/ESR) as indicated.
• Ophthalmology
  • Within 24 hours if any ocular involvement; follow every 1–2 days in SJS/TEN.

Post-Discharge

• Dermatology clinic: within 48–72 hours of discharge, then weekly or as clinically indicated until clear diagnosis and stable/improving course.
• Ophthalmology: at 1–2 weeks after resolution for SJS/TEN or if any eye involvement.
• If DRESS:
  • LFTs, renal function, and thyroid function every 1–2 weeks for 8–12 weeks.
• If EN/panniculitis or Sweet:
  • Follow-up as needed to monitor underlying condition (IBD, sarcoid, malignancy, etc.) and recurrence.

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Red Flags / When to Seek Immediate Care

Advise the patient (and nursing staff if inpatient) to report/act immediately for:

• Rapid spread of rash or sudden increase in skin pain
• Development of blisters, skin sloughing, or any areas of raw, denuded skin
• New or worsening mucosal erosions, especially if:
  • Eye pain, redness, discharge, blurred vision, or photophobia
  • Trouble swallowing, speaking, or drooling
  • Shortness of breath, chest tightness, or noisy breathing
  • Severe genital pain, difficulty urinating, or urinary retention
• Fever >38.5°C (101.3°F) with chills or feeling very unwell
• Signs of organ involvement:
  • Dark urine or markedly decreased urine output
  • Jaundice, severe abdominal pain, persistent nausea/vomiting
  • New confusion or severe headache
• Any hemodynamic instability: dizziness, fainting, palpitations, chest pain, or low blood pressure.

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This unified plan emphasizes rapid triage and stabilization, early exclusion of SJS/TEN, systematic diagnostic workup (labs + biopsies), and targeted therapy while monitoring for serious complications and planning for long-term prevention of recurrence.