No Author (year) Age of onset/sex Clinical features Histopathology DIF Treatment and outcome 1 Schroeter et al. [8 ] (1969) 41 mos/M Crusting plaques on the scalp, neck, back; generalized flaccid blisters on erythematous base sparing only lower extremities Acantholytic cells lying within a vesicle high in the epidermis Intercellular staining of the epidermis with IgG Prednisone started at 140 mg/d + triamcinolone cream wet dressing 2 Kahn and Lewis [7 ] (1971) 18 mos/F Vesicular, crusting, arcuate, and circinate lesions spread over the trunk, upper arms, thighs, and perineum into the entire body Subcorneal acantholysis of the granular layer ND High potency TCS applied without occlusion 6 times/day for 6 months 3 Sotiriou et al. [9 ] (1980) 4 yrs/M Tense bullae on the lower extremities and lower part of the abdomen Intraepidermal bullae with a few acantholytic cells and chronic inflammation IgG, C3, and fibrin in the intercellular epidermis with IgM around some blood vessels Prednisone started at 25 mg/d 4 Levine et al. [10 ] (1982) 14 yrs/F Scaly, erythematous, exudative plaques on face, trunk, and legs Separation of the stratum corneum from the malpighian stratum and acantholysis of the granular cells Deposition of IgG and C3 in the epidermal intercellular space Prednisone started at 240 mg/d and tapered to 10 mg/d with an asymptomatic course 5 Jones et al. [11 ] (1986) 4 yrs/M Extensive crusting on face and scalp; generalized, papular, scaly eruption on trunk and extremities Intraepidermal blister at the level of the granular layer, with a few acantholytic cells Intercellular staining of the high epidermis with IgG Topical fluorinated steroid 6 Yorav et al. [12 ] (1989) 8 yrs/F Widespread flaccid vesicles and bullae with superficial erosions on the trunk, shins, and lower legs Midepidermal cleft containing acantholytic cells Deposition of IgG and C3 in the epidermal intercellular space Dapsone 100 mg/d for a month without any clinical improvement. 7 Goodyear et al. [13 ] (1991) 12 yrs/F Crusted lesions on the lower limbs; blisters in a linear configuration on the arms, legs and lower abdomen Separation in the upper epidermis Intercellular staining of the epidermis with IgG and C3 Prednisolone 80 mg/day showed rapid resolution, which gradually reduced. 8 Walker et al. [5 ] (1995) Neonatal/M Denuded skin on the extremities; intact blisters on the right upper arms Intraepithelial vesicles with eosinophilic infiltrates Intercellular IgG and C3 deposits in the superficial epidermis Polysporin ointment twice a day 9 Rosella et al. [14 ] (1996) 10 yrs/M Erosions and crusted lesions on the trunk, pubis, ears, and scalp Subcorneal bulla containing acantholytic epidermal cells. The lower epidermis showed spongiosis and focal acanthosis Intercellular IgG, IgM, and C3 in the epidermis Deflazacort 45 mg on alternate days 10 Galambrun et al. [15 ] (1997) 8yrs/M Small crusted lesions with raised edges and blisters on the trunk, face, and limbs Splitting of the upper epidermis, filled with acantholytic cells IgG and C3 deposits in the upper half of the epidermis Topical betamethasone 11 Qureshi et al. [16 ] (1997) 16 yrs/M Superficial flaccid bullae and erosions on the upper chest, upper back, and neck Intraepidermal separation in the upper malpighian layers with acantholytic cells Intercellular IgG and C3 in the upper stratum Malpighi epidermis TCS cream + triamcinolone acetonide injection 12 Mehravaran et al. [1 ] (1998) 7 yrs/F Generalized erythema, with superficial erosions on the face, trunk, and extremities; intact superficial blisters on the extremities Subcorneal blister with acantholytic cells without any inflammatory cells in the cleft Intercellular staining for IgG, IgA, and C3 in the epidermis Prednisone 50 mg/d showed slow remission. A prednisone and dapsone combination resulted in complete recovery after a month 13 Metry et al. [4 ] (2002) 3 yrs/M Serpiginous, crusted, erythematous plaques on face and neck Subcorneal pustule with upper epidermal acantholysis Intercellular IgG and C3 in the epidermis Prednisolone 2 mg/kg/d + HCQ 5 mg/kg/d 14 Avalos-Díaz et al. [6 ] (2000) Neonatal/M Erythematous eruption on the trunk and scattered vesicles on his trunk, arms, and legs Subcorneal vesicles Intercellular IgG and C3 in the epidermis Spontaneous resolution within two weeks 15 Hirsch et al. [17 ] (2003) Neonatal/F Erosions on face, ears, chest, and extremities Cleft within the superficial epidermis with a sparse neutrophilic infiltrate Intercellular IgG and C3 in the epidermis Mupirocin 2% + hydrocortisone valerate 16 Narbutt et al. [18 ] (2003) 11 yrs/M Disseminated bullae, vesicles in an annular pattern, and erosions on the extremities, abdomen Acantholysis and intraepidermal, subcorneal blistering Intercellular IgG in the epidermis Cyclophosphamide 1 mg/kg/d + methylprednisolone 1.5 mg/kg/d + HCQ 100 mg/d 17 Connelly et al. [19 ] (2007) 21 mos/F Erythroderma; erosions on hands and feet but no evident vesicles/bullae Subcorneal blister with few acantholytic cells Granular/linear IgG and C3 on the keratinocyte epidermal surfaces Solumedrol 2 mg/kg/d 18 Mlynek et al. [20 ] (2009) 14 yrs/F Sharply demarcated crusted erosions and discrete flaccid vesicles on upper trunk, face, and scalp; alopecia Subcorneal acantholytic clefts within the epidermis Intercellular IgG in the epidermis Prednisolone 1 mg/kg/d + IVIg 19 Fariba et al. [21 ] (2012) 12 yrs/M Erythroderma with scaling and exudation; mild palmoplantar keratoderma and scales covered the scalp Subcorneal cleft in the granular layer Intercellular IgG and C3 in the upper epidermis Prednisolone 30 mg/d + azathioprine 50 mg/d 20 Salazar et al. [22 ] (2012) 16 yrs/F Hyperpigmented patches with hemorrhagic crusting and scale, scattered vesicles, and pustules on the trunk Subcorneal blister with superficial acantholysis Intercellular IgG in the epidermis Prednisone was transitioned to minocycline + nicotinamide 21 Lorente Lavirgen et al. [23 ] (2012) Neonatal/F Flaccid bullae and denuded skin areas on the extremities ND ND Prednisolone 0.5 mg/kg/d for a week + TCS 22 García-Meléndez et al. [24 ] (2013) 11 yrs/F Tense blisters on the face progressing slowly to erythroderma with yellow-greenish crusts Subcorneal bulla with upper epidermal acantholysis Deposition of C3 and IgG in the stratum spinosum with a beehive pattern Prednisone 1 mg/kg/d + dapsone 50 mg/d 23 Adah et al. [25 ] (2014) 13 yrs/F Diffuse, tender, erythematous, desquamating rash on the trunk, face, and extremities; intact flaccid blisters; mild conjunctivitis Subcorneal blister filled with acantholytic cells and scattered neutrophils Intercellular IgG, C3, and fibrinogen in the epidermis Pulse steroids in 3 days (methylprednisolone 500 mg/d) 24 Geller et al. [2 ] (2016) 11 yrs/F Erythematous plaques with yellow-brown crusts on the face, upper trunk, and arms Subcorneal bulla with acantholytic keratinocytes at the base of the blister Intercellular IgG in upper the epidermis; fine granular linear IgM deposits along the dermoepidermal junction Prednisone 1 mg/kg/d 25 Laarman and Horii [26 ] (2017) 15 yrs/F Flaccid vesicles on the inner upper arms and axillae; superficial erosions with crust on the buttocks, back, chest, face, and scalp Subcorneal split with eosinophilic spongiosis Intercellular IgG and C3 in the epidermis, with deposition of C3 along the basement membrane Prednisone + MMF 26 Loh and Paravar [27 ] (2017) 17 yrs/M Follicular pustules on over the body; hyperpigmented, grey, and violaceous crusted papules overlying pink patches on hands, trunk, bilateral cheeks, and nose, sparing the nasolabial folds Epidermal acanthosis and multifocal areas of eosinophilic spongiosis within the epidermis. Lymphocytic infiltrate with occasional eosinophils in the superficial dermis Intercellular IgG and C3 in the epidermis Prednisone 80 mg/d + MMF 2 g/d 27 Evans et al. [3 ] (2019) 8 yrs/M Erythematous annular and polycyclic plaques with central clearing and peripheral scaling on the face, trunk, and proximal extremities Subcorneal vesicle with acantholytic keratinocytes; a few neutrophils in the areas of parakeratosis Intercellular IgG in the epidermis Systemic steroids + rituximab 28 Kianfar et al. [28 ] (2022) 14 yrs/F Not mentioned Not mentioned Not mentioned Systemic steroids + rituximab 29 Our case 42 mos/F Generalized scaling and crusted erythematous patches; flaccid blisters easily ruptured into diffuse crusted erosions on trunk and extremities Splits at the level of the subcorneal layer with superficial acantholysis Intercellular staining of the superficial epidermis with IgG and C3 Prednisolone 20 mg/d
