Case Report

Sex

Age at Presentation

Described Morphology

Distribution

Duration

Outcome

Kapur et al⁽²⁾

7F, 4M

10 at birth, 1 at 1 mo

7 had solitary lesions. 4 had multiple lesions

T, E, P

 Unknown

Brazzola et al⁽³⁾

M

Birth

5 mm red-brown papulonodular lesions, some with crusts

H, T, E

3 mo

Anetoderma-like lesions, multiple milia

Ersoy-Evans et al⁽⁴⁾

2M Monozygotic twins

Birth

Hemorrhagic bullae at birth. Progression into crusted papules the following day

E/ T

2 mo

Hashimoto et al⁽⁶⁾

F

Birth

Yellowish-brown papules, some with central ulceration and crusting

H, G, E

1 mo

Ganga-Zandzou et al⁽⁷⁾

M

Birth

Bullous skin lesions that became ulcerated after 10 days

H, E, T

4 mo

Hyperpigmentation

Inuzuka et al⁽⁸⁾

M

Birth

2-6 mm hemorrhagic vesicles or bullae with the appearance of vascular proliferation that became crusted and flat-topped

H, T, E, G

4 mo

Butler et al⁽⁹⁾

M

Birth

Dry, scaly patch on the left foot,  0.2–0.5 cm orange-brown papules

T, E

3 mo

Shaffer et al⁽¹⁰⁾

F

Birth

2-5 mm non-blanching macules, "blueberry muffin baby"

T, E, P

3 mo

6 mo after resolution lytic bone lesions seen, with progressive improvement

Sankilampi et al⁽¹¹⁾

M

Birth

1-10 mm brownish-red or purplish papulo-nodular, macular, some were firm and violacious-reddish. "blueberry muffin baby". Formed crusted  papules during involution

H, T, E, P

8 mo

Atrophic scars

Divaris et al⁽¹²⁾

M/M

Birth

2-5 mm purpleish-brown macular papular lesions resembling "blue berry muffin baby", some with crusting/ solitary 1 cm ulcerated brown nodule with central crust

H, T, E

6 w/ 11 days

Hyperpigmentation

Edwards et al⁽¹³⁾

M

2 mo

multiple 1- to 5-mm flesh-colored and erythematous papules, some with central umbilication and crust

H, T, G

5 mo

Bernstein et al⁽¹⁴⁾

M

Birth

1 cm solitary, firm, centrally crusted nodule

E

3 mo

Atrophic, hypopigmented scar

Weiss et al⁽¹⁵⁾

F

Birth

Small papule that progressed to a 5 mm yellow-red, solid nodule with superficial telangiectases by 7 mo

H

Excised

Masouyé et al⁽¹⁶⁾

F

2 mo

3 mm brownish, firm nodule  that grew to 9 mm in one mo

E

Excised

Esterly et al⁽¹⁷⁾

M/M/F

Birth

2x7 mm solitary brown nodular plaque/ Vesicopustular, crusted, erythematous umbilicated, progressing to papular/ Erythematous, umbilicated papules and ulcerated lesion in axilla

T/ H, T/ T, G

Excised /2 mo/ 1.5 years

F: Diabetes insipidus at 4 years old

Chun et al⁽¹⁸⁾

F

Birth

Solitary hemorrhagic, crusted papule

H

3 mo

Scaring

Hashimoto et al⁽¹⁹⁾

3M

3.5 mo/ birth/ birth

0.2 X0.5 cm yellow papular eruption, the diaper area some with central umbilication/ yellow papules, nodules/solitary, ulcerated, dark red-brown nodule

G/ E, G / G

14 mo/ ?/ excised

Kirkland et al⁽²⁰⁾

M

Birth

1 cm solitary reddish-brown ulcerated nodule

E

Excision

Shy et al⁽²¹⁾

M

Birth

1 cm solitary isolated skin lesion

T

1 mo

Hypopigmented scar

Ofuji et al⁽²²⁾

M

Birth

Solitary pea sized elevated, darkish-red nodule that ulcerated and developed a thick black crust

E

36 days

Larralde et al^{(23, 45)}

8F, 4M

Birth

Numerous brownish-red papules, papulovesicles, crusts, and nodules. One patient had ulcerated lesions

H, E, P, U

1-3 mo

Ulcerated lesions left hypochromic macules. One patient had relapse at 8 mo with systemic involvement and died at 11 mo

Morgan et al⁽²⁴⁾

M

Birth

Diffuse, erythematous, crusted erosions, some intact vesicles

H, T, E, P

3 mo

Belhadjali et al⁽²⁵⁾

M/F

1 mo/ 20 days

5-15 mm yellowish papules, some with ulcerations and scabs/ 5-20 mm brownish-red papulonodules with a smooth surface

T, E, P/ H, T

Few w/ 4 mo

Skin recurrence at 8 mo old, but resolved in a few weeks with hyperpigmented scars

Timpatanapong et al⁽²⁶⁾

M

Birth

Brownish red to bluish red, firm, papulonodular. Large, 8 cm red, apple-like tumor mass on P that showed surface necrosis after 1 w

H, E, P

3 mo

Hyperpigmented scar

Kapila et al⁽²⁷⁾

F

Birth

Small, purpuric. indurated, red to purple papules and nodules. Groin lesion, 1.0X 0.5 cm, deeply ulcerated raised, beefy border

H, T, E, P, G

3 mo

Bonifazi et al⁽²⁸⁾

M

Birth

Papulonodular lesions, some formed a hemorrhagic crust

H, P,G

40 days

Kodet et al⁽²⁹⁾

M

Birth

5-10 mm pink to violaceous nodules

H, T, E, P

2 mo

Kim et al⁽³⁰⁾

F

Birth

2-4 mm round to ovid vessicles with erythematous bases most superficially eroded or hemorrhagic crusted

H, T, E, P

2 w

Pujol et al⁽³¹⁾

M

Birth

Pinhead to 2-5 mm reddish pink papules rangig, some were thinly crusted. Scalp was covered with thick, yellow crusts. 8  mm yellowish papule on left leg

H, T, E, G

6 mo

Solitary, 2 cm  ulcer presented on gums at 6 mo and resolved within 2 mo

Hatakeyama et al⁽³²⁾

M

2 mo

Macules, papules, petechiae, crust

H, T, G

9 mo

Relapse with isolated thymic LCH 2 mo after skin lesion regression

Lau et al⁽³³⁾

12 cases

3.45 mo average

Macules, papules, plaques, scales, vesicles, pustules, crusts, and petechiae

H, T, G

Systemic relapse of 4 patients between 5 w and 5 mo. 2 died form relapse complications

Herman et al⁽³⁴⁾

F

Birth

2-4 mm vessicles, superficial erosions, and hemorrhagic crusts, with occasional firm reddish-brown papules

H, T, E, U

2 mo

Longaker et al⁽³⁵⁾

F/M/M/M

Birth

2-3 mm erythematous papulovesicles followed by crusting and flat-topped, yellow-brown papules/ crusted papulovesicular eruption, 3mm erosion on palate, three firm nodules with central crusting on the scalp/ vesicopustules, crusted lesions/ 2-6 mm red-blue papulovesicles and  6 mm exophytic indurated papule with overlying scaly crust on leg

D, U

4 mo/ 4 mo/ 3w/ 6 w

M1: 5 cm LCH mass in the temporal bone and musculature at 6 mo resolved with chemotherapy. M2: recurrent LCH with hypopigmented, atrophic macules at 3 mo, spontaneously resolved by 11 mo M3: Milia

Jang et al⁽³⁸⁾

1F, 3M

7 mo/ Birth/ 1 mo/ 2 mo

Multiple, discrete, erythematous papules

H, T, E/ H, T, E/  D /H, F

4 mo /1 mo /2 mo /4 mo

Ricart et al⁽⁴⁰⁾

F

Birth

5 to 15 mm Red-brownish papules, some had a greyish base, and others had a scaly crust

H, T, E

2 mo

Battistella et al⁽⁴¹⁾

10M, 11F

Mean onset of 12 days

Hypopigmented macules, isolated keratotic palmoplantar lesions, necrotic lesions, intertrigo, papules, ulcers and crusts, 4 with solitary lesions

H, T, E, P, S, G, D

Mean duration of 3.6 mo

No relapse after a mean follow-up of 30 mo, hypopigmented atrophic lesions in 6 patients, hyperpigmented macules in 1 patient, and milia in 3 patients

Hoeger et al⁽⁴⁶⁾

M

Birth

Varicelliform rash at birth. Recurrence with  papulonodular and papulovesicular pruritic nodular papules, accompanied by painful erosions and fissures at 8 mo

D

Skin lesion recurrence at 8 mo treated successfully with combined chemotherapy. Diabetes insipidus at 2 years. Death at 28 mo

Whitehead et al⁽⁴⁷⁾

F

Birth

0.5-1 cm papular, hemorrhagic, encrusted lesions.  Recurrent lesions smaller and not hemorrhagic

D, E Recurrence on H

6 w

Skin recurrence at 6 mo with complete resolution at 9 mo

Higgins et al⁽⁴⁸⁾

F

Birth

Widespread blistering followed by crusted papules and erosions 2 days later

H, T, E

6 w

Milia and anetoderma

Kanitakis et al⁽⁴⁹⁾

F

Birth

1-8 mm firm, red-violaceous, indolent nodular lesions, some with central whitish depression

H, T, E

3 mo

Hyperpigmentation

Huang et al⁽⁵⁰⁾

F

Birth

Mimicking diffuse neonatal hemangiomatosis: multiple hemangioma-like lesions, 0.5 to 4 cm, light brown, red or dull red papules, nodules tumors and hemorrhagic crusted plaques

H, T, E

3 mo

Hyperpigmentation and scars

Schaumburg-Lever et al⁽⁵¹⁾

F

Birth

Up to 1 cm, brownish nodules, several lesions had a greyish base

H, T

8 mo

Hyperpigmentation that resolved

Walia et al⁽⁵²⁾

M

Birth

4-10 mm brownish-red shiny papulonodules, with some vesicles. Nodule on leg was 2 cm.

H, T, E

6 mo

Hypopigmented scars

Pavlović et al⁽⁵³⁾

F

Birth

Erythematous and crusted papules, nodules, pseudovesicles

D

6 w

Atrophic scars

Hashimoto et al⁽⁵⁴⁾

M

Birth

1-2 mm pale, discrete, confluent, or lichenified non-puritic papules, some were umbilicated

H, T, E

7 mo

Hypopigmented, atrophic scars

Ornvold et al⁽⁵⁵⁾

M

Birth

2-10 mm firm bluish nodules, petichea, some forming crusts

H, T, E

a few w

Scarring

Thong et al⁽⁵⁶⁾

F

Birth

Thumb-sized, irregularly shaped, erythematous scaling atrophic and erosive patches, No vesicle or nodule

H, T, E

several mo

Rabkin et al⁽⁵⁷⁾

F

Birth

Red nodules

D

1 mo

Marsh et al⁽⁵⁸⁾

F

Birth

1-7 mm papular, circular to oval crusting lesions on an erythematous base, some were ulcerated and some were vesicular

H, T, E

Alexis et al⁽⁵⁹⁾

M

Birth

Reddish-brown to violacious small papules to a 1 cm nodule

H, T, E

1 mo

Zunino-Goutorbe et al⁽⁶⁰⁾

2M

Birth

5 mm and 10mm solitary nodular, erythematous, or ulcerated, erythematous, crusted nodule

H/H

1 mo /3 mo

Avram et al⁽⁶¹⁾

F

Birth

3 to 7 mm erosions and papules with a hard serosanguineous crust, some with surrounding pink erythema

H, T, E

1 mo

Hashimoto et al⁽⁶²⁾

M

17 days

Pea-sized, dark brownish-blue nodules, progressed to penny-sized, reddish-blue, movable nodules in 6 days

H, T, E

3 w

Hashimoto et al⁽⁶³⁾

F

Birth

2-4 mm elevated, firm, reddish-brown nodules, that formed crusts

H, T

3.5 mo

Slott Jensen et al⁽⁶⁴⁾

2M

Birth

Reddish-bluish nodules, 6x7 mm yellowish-brown papule, 8 mm red-brown slightly elevated tumor 6 mm reddish brown vesicular and superficially ulcerated lesion

H, T, E

1 yr, 2 mo

Hyperpigmentation that resolved

Murata et al⁽⁶⁵⁾

F

4 Months

Solitary reddish-yellow nodule, non- ulcerated, rubber-like consistency with central pallor and peripheral telangiectasia

H

2 mo

Ballin et al⁽⁶⁶⁾

M

Birth

Multiple erythematous scaly papules

H, T, E

Unknown

Orle et al⁽⁶⁷⁾

F

Birth

papulonodular reddish-brown erythematous lesions and crusts of different sizes

H, T, E

5 weeks

Dorjsuren et al⁽⁶⁸⁾

F

Birth

7 mm Solitary  domeshaped, skin colored papule with crust

P

2 mo