Common Transient Neonatal Dermatoses

1. Common Transient Neonatal Dermatoses Harper’s Textbook of Pediatric Dermatology Presented by :M.Shahsavari

2. Neonatal desquamation • postmaturityin the neonate, and sometimes dysmaturity • In healthy full - term neonates, increased epidermal desquamation is also present during the first 3 months, with an overallprevalenceof 39.5% • In extreme cases, because of similarities with the collodionbaby syndrome, a congenital ichthyosis may be considered

3. in cases of postmaturitydesquamation,thereis no ectropion,noeclabium and the gloved appearance is lacking • in massive desquamation should alert the doctor to X - Linked recessivehypohidroticdysplasia

5. Harlequin colour change • transient erythema involving one - half of the infant ’ s body with simultaneous blanching of the other side and a sharp demarcation on the midline • the pathophysiology remainsunknown • in the subgroup of infants with HCC and non – epileptic tonic seizures triggered by abnormal painful stimulations (PEPD), it has been show that the disorder is produced by mutations in the SCN9A gene

8. Premature infants are more commonly affected than full terminfants • Flushing and isolated HCC • Harlequin colour change usually occurs on thethirdor fourth day of life, though it has been noted upuntil the 21st day of life • The midline demarcation of the erythema and the transitory colour changes (30 s to 20 min) are diagnostic

9. the face and genitalia are usually spared • The tongue and lips are unaffected • Turning the infant on the other side may induce blanching of the red side and reddening of the pale side

10. Paroxysmal extreme pain disorder ( PEPD ) • Typical flares are precipitated by perineal pain or stimulation, such as insertion of a thermometer into the anus, and consistof stiffening in flexion position, turning pale, and screaminginconsolably, followed by hemicorporealflushing • after the attack, infants may experience intense sweating, and neurological examination is normal between attacks

11. Changes in skin colour should subside after the third week in common HCC, and during the first years in PEPD • Differential diagnosis and treatmen • capillary malformation • cutis marmoratatelangiectaticacongenita • Transient dermatographism • No treatment is required • carbamazepinetherapy may be effective to reduce the frequency of the flares

12. Transient localized hyperpigmentation • The maturation of the pigmentary system is not completed at birth and some areas may appear markedly hyperpigmented • It is usually a prominent feature of dark - skinned newborns, but it has also been observed in fair - skinned newborns • Most frequently involved: • The genital area(scrotum) • the dorsal surfaces of the distal phalanges of both hands and feet are the most frequently involved

13. Less frequently, other body areas may be involved, including nipple and areola, helix, umbilicus, and sometimes large skin folds • Perineal or periungual hyperpigmentation is considered as a physiological melanic pigmentation mainly observedduring the first year of life and declining around the age of 2 years

15. Sterile transient neonatalpapulopustular eruptions

16. Erythema toxicumneonatorum ( ETN) • the most common transient rash in healthy term neonates, affecting 43% of the newborns • It is characterized by small erythematous macules with or without a central papule or pustule • ETN arises between the first and fourth days of life and lasts 2 or 3 days and delayed onset, as late as 10 days of age in term infants, has also been observed

17. Duration is brief, usually 2 – 3 days. Pustular eruptions persist longer • Relapses and ETN - like eruptions after the end of the first month may occur rarely • Two types of eruptions are encountered • The most frequent clinical type consists of collections of erythematous and papular lesions (70% of cases). (flea bite ’picture)

18. In 30% of cases the eruption is predominantly pustular, occasionally surrounded by a small erythematous rim • Lesions are located mostly on the trunk, with a predilection for the back, but are readilyfound on the upper arms, thighs and face, where they may predominate • The palms and soles are relativelyspared • Atypical cases of ETN with pustules localized to the genital areas have been reported

20. Aetiology • The cause of ETN is not yet established • too long exposure with the meconium • According to Leiner , many newborn infants with the condition have dyspeptic disorders; thus, he proposed the term erythema toxicum ’ because he postulated that systemic absorption of enterotoxins accounted for the rash

21. the allergic theory(Eosinophilia in skin biopsies and blood) • a reaction to mechanical stimulation or simple pressure • the incidence of ETN increases with maturity, as determined by birthweight, gestational age or breast hypertrophy grade in the mother

22. the incidence is controversial, and ranges from 30% to 70% • no predilection according to sex • A lower incidence was found in black children • the incidence is positively correlated with several parameters of maturity, including birthweight, and also with the number of pregnancies

25. Differential diagnosis. • generalized sepsis(poor feeding and other signs of serious illness) • viral diseases and Candida infection (Tzanck smear) • incontinentiapigmenti • Eosinophilicpustular folliculitis • Atypical forms of self - healing histiocytosis

26. Job – Buckley syndrome may present at birth with eosinophilic eruption, as may Omennsevere combined immunodeficiency • Acropustulosis of infancy may also, rarely, begin at birth or in the first 3 weeks, and pustules may contain eosinophils. Most cases, however, are characterized by a neutrophilicinfiltrate and onset after the neonatal period. The location of lesions and the relapsing course permits a diagnosis.

27. Pathology • In macular erythematous lesions, edema of the upper dermis was associated with a mild cellular infiltration of eosinophils and neutrophils in a perivascular or diffuse distribution • in papules, more edema and cellular infiltration is found, with a predominance of eosinophilicleucocytes • Of cells filling the pustule, 70 – 95% are eosinophils • Treatment. No treatment is necessary or indicated • administration of an antihistamine, Pyribenzamine, to a series of 75 babies with ETN was found to reduce by half the duration of the rash

28. Transient pustularmelanosis • characterized by pustules that are present at birth and evolve into areas of macular pigmentation • more common in black neonates • The content of the pustules is mostly neutrophilic, and the duration of the rash, especially the pigmentation, is longer

29. Lesions are located on the chin, neck, nape, upper chest, lower back and buttocks but also on the lower abdomen and medial side of the thighs • Rarely, scalp, palms and soles are involved • In typical cases, at the onset, pigmented macules coexist with flaccid vesiculopustules • they rupture easily,leavinga collarette of fine white scale ,Individualpustules dry out and may leave a flat brownish crust

30. Unscratched crusts may persist for a few weeks, and in darker - skinned patients pigmented freckles may persist for a few months (lentiginosisneonatorum) • Aetiology • The higher incidence in black neonates may be related to an accelerated stimulation of native melanocytes due to cytokines and to release of growth factor by cellsin the epidermal infiltrate

31. Pathology • fontana– Masson staining reveals an increased amount of melanin in the basal and suprabasallayers • Most characteristically, the subcornealpustule contains polymorphonuclear leucocytes and scattered eosinophils • In the dermis, eosinophilsand/or neutrophils may be seen around capillariesandaroundthe upper portion of the hair follicle • No treatment is required

34. Miliaria • occlusion of sweat ducts at various levels, resulting in leakage of sweat in the epidermis or papillary dermis • strong connection between the use of occlusiveagents such as ethyl chloride spray, iontophoresis of various chemicals, ultraviolet irradiation and occlusive plastic wraps and the induction of miliariacrystallina or rubrain adult volunteers subjected to warm conditions

35. A causative role for cutaneous bacteriahas long been suspected (Staphylococcus epidermidis ) • an initial functional or ultrastructural obstructiondamages the acrosyringiumand leads tothe reparative formation of a parakeratoticplug whichperpetuatesthe disease process

36. Miliariacrystallina ( sudamina) • 1 – 2 mm superficial clear non - inflammatory vesicles, resembling drops of water, which are asymptomatic • common in the summer months and is also noted in infants housed in incubators • Lesions occur mostly on the forehead, and on the sixth to seventh day of life • They spread over the scalp in a few hours • Onset before the fourth day of life is exceptional

37. congenital cases have been reported, attributed to a maternal febrile illness prior to delivery, as well as to the moist occlusive environment of amniotic fluid and vernixcaseosa • in congenital cases, the lesions are generalized but spare the palms, solesand mucosae

38. Miliariarubra ( prickly heat) and miliariaprofunda • Miliaria rubra occurs later than miliaria crystallina • Typical onset is between the 11th and 15th days of life • outbreaks may be preceded by episodes of miliariacrystalline • occurs mostly in hot, humid conditions • It usually affects sites of friction or occlusion, such as the neck and face, but also occurs on the trunk

39. Lesions are erythematous non follicular1-3 mmpapules or papulovesicles, which may evolveinto pustules (miliariapustulosa)

40. Miliariaprofunda • firm, pale papules on the trunk and extremities • may follow miliariarubra • unusual in newborns • In miliariarubra, the level of ductal obstruction is intraepidermal • In miliaria profunda, eccrine ducts are obstructed at the dermoepidermal junction

41. in type I pseudohypoaldosteronism, pustular miliariarubrais considered to be a specific cutaneous finding(salt - losing crises ) • Treatment • the patient should be removed from the warm and humid environment • Cool bathing and air conditioning arethe best therapeutic measures

42. In the case of suspicion of superinfection, antibiotics may be started • chlorhexidine cleansing solution or a topical antibiotic (such as erythromycin or clindamycin) may beadvised • One case of extensive miliariaprofundaresponded to isotretinointherapy

43. Milia: Bohn and Epstein pearls • the most common transient skin disorders in neonatal (30-40 %) • 1– 2 mm white or yellow papules on the nose, chin, cheeks and forehead • The nose is predominantly affected • Less commonly,lesionsmay occur on the trunk and extremities • Miliaareepidermal cysts derived from the pilosebaceousfollicle

44. they are normally spontaneously extruded in a few weeks • Persistent or extensive miliaare seen in • Marie Unna hypotrichosis, orofacial - digital syndrome type I, Basansyndrome, and the X –linked Bazex –Dupré –Christoldisease, which is also characterized by hypotrichosis

45. Epstein pearls – epidermal cysts on the palate – are present in the majority of newborn infants • When they occur on the alveolar margins, they are termed Bohn cysts

47. Perianal dermatitis • an area of erythema centred on the anus and occasionally accompanied by erosion and bleeding • Prevalence : 4% to 18.9% • usually attributed to formula milk feeding in the newborn and abnormal faecal pH • It is observed between the fourth and seventh days of life • 6.5 times more frequent in premature babies

48. Adnexal polyp of neonatal skin • 4.1% of Japanese neonates • small, usually solitary, skin tag situated around the nipple • it detaches spontaneously • Two cases of persistent lesions, in a 53 - and a 370 - day - old - infant, have been reported