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Table 2 Individual case results of photodistributed Stevens–Johnson Syndrome and toxic epidermal necrolysis

From: Photodistributed Stevens–Johnson syndrome and toxic epidermal necrolysis: a systematic review and proposal for a new diagnostic classification

Reference to case

[28]

[22]

[29]

[30]

[31]

[32]

[33]

[34]

[35]

[36]

[37]

[38]

[39]

Study year

1989

1996

2000

2003

2005

2008

2010

2011

2014

2016

2018

2021

2021

Study type: case report (C), abstract (A)

C

C

C

C

C

C

C

C

C

C

A

(poster abstract)

C

C

Demographics (age, gender)

12, M

23, F

16, F

34, M

66, M

29, F

30, M

22, F

19, F

48, F

65, F

22, F

18, F

SJS or SJS/TEN or TEN

SJS

TEN

SJS

SJS

TEN

TEN

TEN

TEN

SJS

SJS

SJS

TEN

SJS

Causal drug

3 weeks chloroquine and sulfadoxine-pyrimethamine

14 days clobazam

3 weeks of carbamazepine, increased doses from 200 mg/day to 600 mg/day

2 months sulfasalazine

One dose Naproxen Sodium

(Rash not present until 3 days after drug exposure. Experienced similar eruption three times after intake of naproxen, in the last 5 years

3 years of hydroxychloroquine

(Also used gabapentin for 2 years for

postherpetic neuralgia)

19 days of lamotrigine and increased dose of chlorpromazine after 10 years of use

(Also used alprazolam, zolpidem, lithium)

One dose (200 mg) of

Ibuprofen, not first-time use

(1 day after drug use, rash started, stable 2 days, then sever rash after tanning bed)

10 days ciprofloxacin/ 1-day fluconazole

(Rash presented day after drug course)

3 days itraconazole

(Rash presented day after drug course)

Lamotrigine (duration of use not specified)

10 days of lamotrigine

One dose of tramadol

(Only drug taken 7–10 prior to rash onset)

Diagnosis associated with drug

Prophylactic malaria

Alopecia areata on the scalp

Epilepsy

(Additional history of allergic rhino-conjunctivitis)

Seronegative symmetrical polyarthritis

Keratitis of the right eye

Sjogren’s syndrome

Bipolar 1

Menstrual discomfort

Vaginal infection

Vaginal yeast infection

Epilepsy

Bipolar disorder

Hip pain

UVR exposure place and amount

Rash presented after first day of sun exposure at the seashore in Thailand

1 day at beach

45 min of sunlight, explained as an intense exposure

N/A

N/A

Sunburnt on Mediterranean cruise

19 days in psychiatric ward, often lay in sun during day

Eight-minute exposure in tanning bed

1 day at beach

Several hours of sun exposure all three days of itraconazole use

N/A

Daily tanning bed use all 10 days of lamotrigine use

1 day at a lake

Reported photodistributed rash (Y/N)

Y

Y

Y

Y

Y

Y

Y

Y

Y

Y

Y

Y

Y

Rash extension to clothing covered skin (Y/N) (lack of linear demarcation, satellite lesions)

Y

Lack of linear demarcation, satellite lesions

Y

Lack of linear demarcation, satellite lesions

N/A

N/A

Y

Lack of linear demarcation, satellite lesions

Y

Lack of linear demarcation, satellite lesions

Y

Lack of linear demarcation, satellite lesions

Y

Lack of linear demarcation, satellite lesions

Y

Lack of linear demarcation, satellite lesions

Y

Satellite lesions under areola

N/A

Y

Lack of linear demarcation, satellite lesions

Y

Lack of linear demarcation, satellite lesions

Time from UVR exposure to rash onset

N/A

36 h

1 day

3 days

N/A

N/A

N/A

1 day (to change from baseline rash)

2 days

1 day

N/A

N/a

1 day

Timeline of progression/

worsening of rash

Reported worsening rash at 3 days

4 days until progression of rash stopped

Progressed past 48 h

On day 12 of hospitalization, skin lesions were improving

Eruption of new lesions ceased on 12th day of admission

14 days

(7 days to hospital admission, then 7 days later, skin detachment extended to involve at least 60% of the body surface area)

5 days of

gradual progression to painful bullous eruptions and 30% total body surface area

N/A

N/A

Reported worsening rash at 48 h, Presented to ED 5 days

N/A

N/A – Hospitalized for 12 days

Day 5 of rash presented to hospital from continued worsening symptoms

Mucus membrane involvement

Conjunctivitis, erosions of the buccal mucosa and lips, and erosive balanitis

Bilateral conjunctival, oral, and genital mucosa

Oral, ocular, and genital mucosa

Oral and genital mucosa

Buccal and palatal mucosa

Hemorrhagic crusting of the lips, buccal mucosa and tongue. Eyelids were eroded with intense conjunctival

Injection. Vulvar and vaginal lesions

Oral mucosa, conjunctivitis

Oral, ocular, and vaginal mucosa

White vesicles and erosions on the lips. Ulcers of the oral mucosa. Erythematous patches within the labia

Irritation and redness of the conjunctivas. Necrotic crusts on the lips. Oral mucosal inflammation and ulcerations

N/A

Conjunctival injection. Bullae involving the vermillion and mucosal lips, buccal mucosa, and tongue. Swelling of genitalia

Cheilitis, bleeding oral ulcers, and bilateral conjunctival hyperemia with purulent discharge

Palmar and plantar rash (Y/N)

Y

Iris lesions on palms and soles

N/A

N/A

Y

Annular iris lesions on palms of the hands and the soles of the feet

N/A

N/A

N/A

N/A

N

Y

After 48 h the palms and soles were erythematous and painful with some blisters between the toes

N/A

Y

Pruritic eruption including palms and soles

Y

Maculopapular rash of palms of hands and soles of feet

Nikolsky sign ( ±)

N/A

 + 

N/A

 + 

 + 

 + 

N/A

N/A

N/A

-

N/A

N/A

 + 

Skin biopsy

N/A

Hydropic and vacuolar degeneration of the basal cells, with exocytosis of mono-nuclear cells in the epidermis and a subepidermal bulla with festooning of the underlying papillary dermis

Direct immunofluorescence for immunoglobulins and complement was negative

Presence of a subepidermal blister with necrosis of the epidermal keratinocytes and intense chronic inflammatory infiltrate with some eosinophils around the vessels and hair follicles in the dermisa

Full-thickness epidermal necrosis. The cytoplasmic limits of the cells had been lost

Extensive epidermal necrosis; areas around the necrosis showed vacuolar

degeneration of the basal layer, edema of the superficial dermis, melanophages and a mild perivascular

lymphocytic infiltration

Subepidermal,

cell-poor bulla formation with full thickness epidermal

necrosis, consistent with TEN

Direct immunofluorescence

for immunoglobulins and complement was negative

N/A

Lymphocytic infiltrate at the dermal–epidermal junction with some apoptotic keratinocytes consistent with TEN

Intact stratum corneum with interface dermatitis and full thickness epidermal necrosis compatible with SJS

Necrotic epidermis and interface dermatitis with vacuolization

Direct immunofluorescence was negative

N/A

Interface dermatitis with scattered dyskeratosis, consistent with SJS/TEN

Direct immunofluorescence was negative

Findings consistent with SJS

Reported flu-like prodrome (Y/N)

Reported initial signs/symptoms

N

Severe erythema confined to sun-exposed areas. Within the next 3 days fever and malaise accompanied the concomitant development of round, annular, concentric typical iris lesions in sun-protected areas

N

Pruriginous and erythematous eruptions

N

General malaise, fever, conjunctival injection, erythematous and infiltrated lesions

(Quadro de malestar general, fiebre, inyeccion conjunctival, lesiones eritematosas e infiltadas)

N

Severe cutaneous eruption that had started 3 days after sun exposure, No abdominal pain, nausea, vomiting, or fever was present

N

An erythematous and bullous rash had started on his face and neck, which rapidly spread to his arms and legs

N

Facial swelling and a painful erythematous eruption with lethargy and anorexia

N

Whole body itched and he developed restlessness, then wheal-like erythematous, itching skin rashes

N

Four hours following the tanning bed exposure the patient noted increased itchiness of her tanning bed exposed skin. The next morning the patient experienced severe redness, pain, and the beginning of blister formation on her tanning bed exposed abdomen, back, face, and proximal arms

N

A red rash concentrated on her chest developed

N

Without any prodromes, developed rash on upper trunk

No initial symptoms reported

N

Diffusely pruritic eruption involving her upper back and chest that later extended to the face and extremities, including the palms and soles

N

Onset of an erythematous burning rash on her shoulders

Treatment

Systemic corticosteroids and antibiotics (4 weeks to recovery)

Pentoxifylline and prophylactic systemic antimicrobials, supportive measures

methyl-prednisolone

N/A

Methylprednisolone

Benzyl penicillin,

Flucloxacillin, prophylactic low molecular weight

heparin, eye steroid and lubricant

Antibiotics (4 weeks to recovery)

IM Corticosteroid and vancomycin, 2 days I.V. cyclosporine, 3 days of intravenous immunoglobulin G

N/A

3-week course of oral steroids and betamethasone dipropionate ointment

N/A

5-day regimen of oral cyclosporine 5 mg/kg/d

Prophylactic oral doxycycline and topical oral and ocular medications

  1. aOriginal version prior to translation to English: “la presencia de una ampollo subepidérmica con necrosis de los queratinocitos del techo de la misma e intenso infiltrado inflamatorio crónico con algunos eosinófilos alredador de los vasos y folículos pilosos en la dermis”
  2. SJS  Stevens–Johnson syndrome, TEN  Toxic epidermal necrolysis, UVR   Ultraviolet radiation