A new dermatology case for anlysis by DermModel1 program

 Here is an analysis of an example dermatology case by DermModel1, a program created by Michael Perel, M.D.

A 35-year-old white male presents with [Symptoms (patient-reported):

3-month history of a red rash across both cheeks and the bridge of the nose that worsens within hours to a day after sun exposure; burning and tightness > itch; leaves faint hyperpigmentation as it resolves.
Intermittent, shallow, painless sores inside the mouth over the past 2 months, each lasting about 1–2 weeks before healing without scarring.
Diffuse hair shedding over the last 6 weeks, most noticeable on shower drains and brushing; no discrete bald patches and no scalp tenderness or pruritus.
Morning stiffness of fingers and wrists lasting about 45 minutes with episodic swelling and achiness, worse after sun-exposed weekends; no history of trauma .
Fatigue and low-grade fevers (up to 37.8°C) occurring a few evenings per week; 3-kg unintentional weight loss over 2 months .
Increased sensitivity to sunlight on the chest and forearms with burning and redness after short exposures; denies new skincare products or medications before onset .
Occasional color changes of fingers in the cold (white to blue to red) with tingling on rewarming; denies digital ulcers .
Mild pleuritic chest discomfort with deep breaths on two occasions in the last month; resolves spontaneously within a day .
Signs (examiner-observed):

Vitals: T 37.8°C; HR 92 bpm; BP 110/70 mmHg; RR 16; SpO2 99% on room air [4].
Face: Well-demarcated, symmetric erythematous rash over the malar cheeks and nasal bridge with sparing of the nasolabial folds; fine, nonadherent scale; no comedones or papulopustules [3].
Photo-distributed erythema with faint dyspigmentation and mild atrophy (“poikilodermatous” changes) over the V of the chest and dorsal forearms; no vesiculation or crusting [5].
Oral cavity: Two shallow, 3–5 mm, non-tender erosions with erythematous rims on the hard palate; no hemorrhagic crusts or thrush [6].
Scalp: Diffuse hair density reduction with positive hair-pull test (4–6 telogen hairs per pull) without perifollicular scaling; a single 1.2-cm erythematous plaque with adherent scale and follicular plugging behind the right ear; early dyspigmentation centrally [2].
Nails/capillaries: Mild nailfold capillary dilatation and dropout on dermoscopy; periungual erythema; no splinter hemorrhages [1].
Skin elsewhere: Reticular, violaceous mottling over the posterior thighs (livedo pattern) that lessens with warming; no palpable purpura [3].
Musculoskeletal: Tenderness and mild boggy swelling at bilateral MCP 2–3 and PIP 2–3; full range of motion; no deformities or nodules [4].
Lungs/heart: Normal breath sounds; faint, transient pleural rub at the right posterior base on deep inspiration; regular cardiac rhythm without murmurs [6].
Lymph nodes: Small, mobile, non-tender cervical nodes (<1 cm); no hepatosplenomegaly [5].].

Generating Both Plans

Case Summary: 35-year-old white male with a 3-month history of a photosensitive malar rash sparing the nasolabial folds, photo-distributed poikiloderma on chest/forearms, episodic oral ulcers, diffuse non-scarring alopecia with a focal scaly plaque showing follicular plugging, Raynaud-like color changes, inflammatory small-joint arthralgia with morning stiffness, fatigue, low-grade fevers, mild weight loss, intermittent pleuritic chest pain, livedo-like mottling, and nailfold capillary changes. Exam shows symmetric malar erythema without acneiform lesions, telogen effluvium plus a localized plaque suggestive of discoid lupus, non-tender oral erosions on the hard palate, mild synovitis of MCPs/PIPs, transient pleural rub, and livedo reticularis. Overall picture is highly suggestive of systemic lupus erythematosus with cutaneous (acute and chronic CLE), musculoskeletal, mucosal, possible serositis, and possible vasculopathic involvement. Adequate information is present to outline an initial diagnostic workup (autoantibodies, inflammatory markers, organ screening, biopsy where indicated) and an initial treatment plan (photoprotection, topical therapies, systemic immunomodulation such as hydroxychloroquine, and NSAIDs/short-course steroids as appropriate), with referrals to rheumatology and close monitoring for systemic involvement.

Assessment Summary

35-year-old man with a 3‑month history of:

• Photosensitive malar rash sparing nasolabial folds and photo‑distributed poikiloderma.
• Oral painless ulcers.
• Diffuse non‑scarring telogen effluvium plus a localized retroauricular plaque with follicular plugging suggestive of discoid lupus.
• Inflammatory small‑joint arthralgia with morning stiffness and mild synovitis.
• Raynaud phenomenon, livedo reticularis, and nailfold capillary dropout.
• Intermittent pleuritic chest pain with a transient pleural rub.
• Fatigue, low‑grade fevers, mild weight loss.

Overall, this is highly suspicious for systemic lupus erythematosus (SLE) with cutaneous lupus erythematosus (CLE) (acute cutaneous and chronic/discoid) plus musculoskeletal, mucosal, possible serosal, and vasculopathic involvement.

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Differential Diagnosis / Working Diagnosis

Working diagnosis (high probability):

• Systemic lupus erythematosus (SLE) with:
  • Acute cutaneous lupus (malar rash, photosensitivity).
  • Chronic cutaneous lupus (localized discoid plaque).
  • Non‑scarring lupus alopecia (telogen effluvium pattern).
  • Oral ulcers, inflammatory arthritis, probable serositis, Raynaud phenomenon, livedo reticularis.

Key alternative/overlap diagnoses to keep in scope:

• Dermatomyositis (poikiloderma, photosensitivity; less likely: no heliotrope rash/Gottron papules, no weakness yet).
• Mixed connective tissue disease / limited scleroderma overlap (Raynaud, nailfold changes; anti‑RNP/centromere/Scl‑70 to clarify).
• Antiphospholipid syndrome (APS) or secondary aPL positivity (livedo reticularis; thrombosis risk assessment needed).
• Rosacea / seborrheic dermatitis (much less likely: no papulopustules/comedones, nasolabial fold sparing, strong systemic features).
• Polymorphous light eruption or drug‑induced photosensitivity (again less likely given systemic pattern; review meds).

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Workup Plan

Aim to complete most studies within 1–2 weeks; some are same‑day if possible.

Laboratory Studies

1) Autoimmunity / lupus characterization

• ANA by IFA (titer and pattern)

  • Entry criterion for SLE classification; guides further testing.

• Extractable nuclear antigens and SLE‑specific antibodies:

  • Anti–double‑stranded DNA (quantitative).
  • Anti‑Smith.
  • Anti‑Ro/SSA, Anti‑La/SSB.
  • Anti‑RNP.
  • Consider anti‑centromere and anti–Scl‑70 given Raynaud/nailfold changes.

• Complement levels:

  • C3, C4 (± CH50).
  • Low levels support active SLE and are useful for longitudinal monitoring.

• Antiphospholipid antibody panel:

  • Lupus anticoagulant (e.g., DRVVT‑based testing).
  • Anticardiolipin IgG/IgM.
  • Anti‑β2 glycoprotein I IgG/IgM.
  • Plan to repeat any positives at ≥12 weeks for persistence and APS risk stratification.

2) General inflammation, hematologic, renal, hepatic

• CBC with differential

  • Evaluate for anemia, leukopenia, lymphopenia, thrombocytopenia (common in SLE; also relevant for drug safety).

• CMP:

  • Creatinine/eGFR, BUN, electrolytes.
  • AST, ALT, ALP, bilirubin, albumin.

• Hemolysis workup (if anemia present or suspected):

  • LDH, haptoglobin, bilirubin (total/direct), reticulocyte count.
  • Direct antiglobulin (Coombs) test.

• Inflammatory markers:

  • ESR and CRP.
  • ESR typically elevated in SLE; disproportionately high CRP suggests infection or pronounced serositis.

• Renal screening:

  • Urinalysis with microscopy (look for protein, hematuria, RBC casts).
  • Spot urine protein‑to‑creatinine ratio (UPCR) or 24‑hr protein.
  • If hematuria/proteinuria or casts present → urgent nephrology input.

3) Vasculopathy / Raynaud

• Baseline coagulation profile:
  • PT/INR, aPTT (aPTT may be prolonged with lupus anticoagulant).
• Consider fasting lipid profile (vascular risk) and possibly homocysteine if suspicious for thrombophilia.

4) Pre‑immunomodulatory safety

• Hepatitis B: HBsAg, anti‑HBc (± anti‑HBs if not known).
• Hepatitis C Ab.
• HIV 1/2 Ag/Ab.
• TB screening: IGRA (or TST per local practice) if future systemic immunosuppression or biologics are likely.
• Baseline vitamin D level (high risk of deficiency with strict photoprotection).

Imaging

• Chest X‑ray

  • Evaluate for pleural effusion or parenchymal disease given pleuritic chest pain and pleural rub.

• ECG

  • Screen for pericarditis (diffuse ST changes), arrhythmias, and as a QT baseline if using HCQ and other QT‑prolonging drugs.

• Echocardiogram (if possible within 1–2 weeks or sooner if symptoms recur/worsen)

  • Assess for pericardial effusion, wall motion abnormalities if myocarditis suspected.

Procedures

• Skin biopsy with DIF (very important):

  • 4‑mm punch biopsy of the retroauricular discoid‑like plaque:
    • One core for H&E (lesional).
    • One for direct immunofluorescence (perilesional, non‑sunburned skin if feasible).
  • Expect interface dermatitis with follicular plugging; lupus band (granular IgG/IgM/C3 at dermo‑epidermal junction).

• Consider biopsy of malar rash if:

  • Diagnosis remains uncertain, or
  • Discoid plaque biopsy is non‑diagnostic.

• Optional:

  • Trichoscopy and standardized hair‑pull documentation.
  • Scalp biopsy from any suspicious scarring area if concern for discoid scarring alopecia.

Specialist Referrals

• Rheumatology:

  • For formal SLE classification, systemic staging, treatment co‑management (especially for joint, serosal, renal, and hematologic domains).

• Ophthalmology:

  • Baseline exam within the first year of starting hydroxychloroquine (earlier if high‑risk: renal impairment, high dose, tamoxifen use).

• Consider cardiology/pulmonology:

  • If serositis recurs or if imaging/ECG or symptoms suggest significant effusion or myocarditis.

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Treatment Plan

1) Systemic Medications

A. Disease‑modifying baseline therapy

• Hydroxychloroquine (HCQ)
  • Start immediately, weight‑based:
    • Dose: up to 5 mg/kg/day actual body weight, not exceeding 400 mg/day total.
    • Example: if 80 kg → 400 mg/day (often 200 mg BID).
  • Indications:
    • Cutaneous disease, arthritis, oral ulcers, fatigue, reduction in flares, possible cardiometabolic benefit.
  • Counseling:
    • Onset of benefit typically 4–8 weeks.
    • Take with food to reduce GI upset.
    • Eye toxicity risk is low at recommended dosing; must keep dose ≤5 mg/kg/day and maintain follow‑up with ophthalmology.
    • Review potential drug interactions and cumulative QT risk.

B. Symptomatic anti‑inflammatory control (short‑term)

• NSAID (if no contraindications: normal renal function, low GI/cardiovascular risk):

  • E.g., naproxen 250–500 mg PO twice daily with food, or ibuprofen 400–600 mg PO every 6–8 hours PRN.
  • Add PPI if significant GI risk factors.
  • Aim: control arthralgia and mild pleuritic pain while HCQ takes effect.
  • Avoid or limit in the presence of renal impairment, active GI ulcer disease, or if anticoagulation is needed.

• Oral glucocorticoids (short course, steroid‑sparing strategy)

  • Consider if:
    • Arthralgia/synovitis or serositis significantly impairs function despite NSAIDs and initial HCQ.
  • Example regimen:
    • Prednisone 10–15 mg/day for 5–7 days, then taper over 2–3 weeks aiming to be off or at ≤5 mg/day by 4 weeks.
  • Avoid prolonged or high‑dose steroids whenever possible; plan taper from the outset.

C. Raynaud management

• Non‑pharmacologic first‑line (see Lifestyle below).
• If functionally significant episodes persist:
  • Long‑acting dihydropyridine calcium‑channel blocker:
    • E.g., nifedipine ER 30 mg PO daily, titrate up as tolerated (BP‑dependent).
  • Consider topical nitroglycerin ointment to particularly ischemic digits if needed (warn about headaches, hypotension).

D. Future escalation (if needed, after 6–12 weeks or earlier with organ involvement)

To plan, not to start immediately unless clearly indicated by severity:

• For persistent cutaneous and joint activity despite optimized HCQ, photoprotection, and topicals:
  • Methotrexate (weekly) with folic acid for joint/skin predominance.
  • Mycophenolate mofetil or azathioprine if broader systemic or serosal involvement.
• For refractory mucocutaneous/musculoskeletal disease on conventional agents:
  • Biologics such as belimumab or anifrolumab (rheumatology‑managed).
• Refractory CLE options in specialized centers:
  • Quinacrine addition (where available) to HCQ, thalidomide/lenalidomide, or low‑dose JAK inhibitors with stringent risk controls.

2) Topical and Local Treatments

A. Facial malar rash / photo‑distributed CLE

• First‑line maintenance (steroid‑sparing):

  • Tacrolimus 0.1% ointment thin layer to affected facial areas twice daily.
    • Good for long‑term use on thin skin; transient burning is common initially.

• Short rescue courses of low‑ to mid‑potency topical corticosteroid for flares:

  • Examples:
    • Hydrocortisone 2.5% cream BID to face for up to 7–10 days.
    • Or desonide 0.05% cream BID for similar duration.
  • Avoid prolonged continuous use on the face to minimize atrophy and telangiectasia.

B. Discoid retroauricular plaque

• High‑potency topical corticosteroid:

  • Clobetasol 0.05% ointment or cream once or twice daily for 2–4 weeks, then taper frequency or switch to weekend‑only use.
  • After acute control, transition to tacrolimus 0.1% ointment for maintenance.

• Intralesional corticosteroid for thick/refractory plaque:

  • Triamcinolone acetonide 2.5–5 mg/mL.
  • Inject 0.1–0.2 mL per injection point spaced ~1 cm apart, total ≤1–2 mL per session.
  • Repeat every 4–6 weeks as needed, monitoring for atrophy.

C. Oral ulcers

• Topical corticosteroid paste:
  • Triamcinolone acetonide 0.1% in Orabase applied to ulcers 2–3 times daily after meals and at bedtime.
• Alternative:
  • Dexamethasone elixir 0.5 mg/5 mL swish for 2–3 minutes and spit, 3–4 times daily during flares.
• Avoid spicy, acidic, and abrasive foods during active ulcers.

D. Hair and scalp

• Telogen effluvium pattern:
  • Reassurance; shedding should improve as systemic disease is controlled.
  • Gentle hair care (avoid tight hairstyles, harsh chemicals, frequent heat styling).
• If any area suggests scarring alopecia progression:
  • High‑potency topical steroid (e.g., clobetasol solution) to the patch 1–2 times daily in short courses.
  • Consider intralesional triamcinolone and scalp biopsy.

3) Lifestyle Modifications

Photoprotection (critical)

• Daily broad‑spectrum sunscreen SPF 50+ with strong UVA coverage applied to face, ears, neck, chest, forearms, and hands.
  • Use ~2 mg/cm² (roughly a teaspoon for face/neck) 15–30 minutes before sun exposure.
  • Reapply every 2 hours when outdoors, and after sweating or water exposure.
• Prefer tinted sunscreens containing iron oxides for additional visible‑light protection, especially for pigmentary changes.
• UPF clothing: long sleeves, tightly woven fabrics, UV‑protective shirts, wide‑brim hat.
• Avoid peak sun hours (10 a.m.–4 p.m.) and reflective surfaces.
• Consider UVA‑protective window films for car/home (UVA penetrates glass).

Smoking

• Strongly advise complete smoking cessation (if applicable):
  • Smoking worsens CLE and diminishes antimalarial efficacy; increases vascular and thrombotic risk.

General measures

• Adequate rest, gradual physical activity to reduce fatigue and maintain joint function.
• Balanced diet; consider vitamin D3 supplementation 800–2000 IU/day initially, adjusted to levels.
• Avoid unnecessary photosensitizing medications if alternatives exist (e.g., some thiazides, tetracyclines, sulfonamides), in coordination with other prescribers.
• Avoid estrogen‑containing contraceptives if high‑risk antiphospholipid profile (will depend on aPL results).

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Patient Education

Key topics to cover:

• Nature of disease:

  • Likely autoimmune disease (SLE) with strong skin involvement.
  • Symptoms arise from immune‑mediated inflammation; not contagious.

• Role of treatment:

  • HCQ is a cornerstone medication to reduce flares and control skin, joint, and systemic symptoms; it works slowly, so adherence is crucial even if you don’t feel better immediately.
  • Topicals help directly calm skin and prevent scarring in discoid lesions.
  • Steroids (oral and topical) are “rescue” medications, not long‑term solutions—goal is minimal effective use.

• Sun protection:

  • Even incidental sun exposure can significantly worsen rash and systemic symptoms; strict daily protection is essential, not optional.

• Raynaud care:

  • Keep entire body warm, especially hands/feet.
  • Avoid sudden cold exposure, vibrating tools, and smoking.
  • Move fingers/toes and warm them at first sign of color change.

• Thrombosis risk and APS:

  • Livedo and possible antiphospholipid antibodies may increase blood clot risk.
  • Importance of follow‑up to interpret aPL tests and possibly using low‑dose aspirin or anticoagulation if indicated.

• Medication toxicity monitoring:

  • HCQ eye risk is small with proper dosing; ophthalmology exams are preventive.
  • Report vision changes (blurring, missing spots, difficulty seeing at night) promptly.
  • If on NSAIDs, report black stools, severe stomach pain, or reduced urine output.

• When to seek urgent care (also see Red Flags section below).

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Follow-up Schedule

Initial period (first 3 months):

• 4–6 weeks after starting HCQ and initial regimen:

  • Clinical review:
    • Skin: rash, discoid plaque, photosensitivity, oral ulcers.
    • Joints: pain, swelling, morning stiffness.
    • Raynaud episodes.
    • Chest symptoms, fatigue, fevers, weight changes.
  • Labs:
    • CBC, CMP (renal/hepatic), urinalysis, UPCR.
    • ESR, CRP.
    • C3, C4, anti‑dsDNA (if initially positive).
  • Adjust NSAIDs, taper steroids if started; reinforce photoprotection.

• Every 8–12 weeks in first year (or more often if unstable):

  • Repeat above labs.
  • Assess clinical disease activity (consider CLASI for skin, simple joint counts).
  • Monitor for side effects of medications.
  • Reassess Raynaud and APS risk periodical based on aPL results.

• Antiphospholipid antibodies:

  • If any are positive initially → repeat aPL panel at ≥12 weeks to confirm persistence and guide vascular prophylaxis decisions.

• Ophthalmology:

  • Baseline exam within the first year of HCQ (earlier if high‑risk: renal disease, high dose, tamoxifen, or other retina‑toxic drugs).
  • Annual exams beginning at 5 years of continuous use (earlier if high‑risk or if dosing/duration exceed recommendations).

• Rheumatology/dermatology visits:

  • Coordinate ongoing systemic management, particularly if escalation to methotrexate, mycophenolate, biologics, or advanced CLE therapies is needed.

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Red Flags / When to Return Sooner

Advise immediate medical attention (ER or urgent clinic) if any of the following occur:

• Renal or severe systemic signs:

  • New dark, red, or cola‑colored urine.
  • New or worsening leg/ankle swelling.
  • Marked decrease in urine output.

• Cardiopulmonary symptoms:

  • New or worsening chest pain, especially if at rest or with breathing.
  • Shortness of breath at rest or with minimal exertion.
  • Rapid, irregular heartbeat or fainting.

• Neurologic/vascular events:

  • Sudden severe headache unlike prior headaches.
  • New weakness or numbness on one side of the body, facial droop, difficulty speaking, confusion, or vision loss.
  • New, persistent, or worsening severe Raynaud with pale/blue digits that do not re‑perfuse, or development of digital ulcers.

• Hematologic/infectious:

  • Unexplained easy bruising, spontaneous nosebleeds, gum bleeding, or petechiae.
  • Fever ≥38.0°C (100.4°F), especially with chills, sore throat, or if on steroids or immunosuppressants.

• Dermatologic escalation:

  • Rapidly progressive or painful skin ulcers.
  • Widespread blistering or necrotic lesions.
  • Sudden dramatic increase in hair loss with scalp redness and crusting.

Prompt reporting of these symptoms allows early adjustment of therapy and can prevent serious complications.

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This plan integrates the diagnostic workup, initial therapy, and monitoring strategy for suspected SLE with cutaneous involvement in this patient, with a strong emphasis on early HCQ use, strict photoprotection, conservative steroid strategy, and close coordination with rheumatology.