1. Childhood rashes The following items will be discussed:
1. Acrodermatitis enteropathica
2. Dermatosis affecting the napkin area
3. Maculopapular childhood rashes
4. Gianotti- Crosti syndrome
5. Papular acrodermatitis of childhood
6. Erythema toxicum neonatorum
7. Blueberry muffin babies
8. Subcutaneous fat necrosis of the newborn
9. Neonatal herpes simplex infection.
1O. Langerhans cell histiocytosis
11. childhood hypopigmentation disorders

2. Acrodermatitis enteropathica Zinc defeciency dermatosis
It is an eczematous skin rash related to zinc deficiency. It may be congenital or acquired secondary to prematurity or dietary deficiency. The congenital form is due to a mutation in a trasnsmembrane zinc uptakeprotein & is inherited in an autosomal recessive fashion. Features include:
@ Red inflamed patches of dry skin, particularly around body orifices such ass the mouth, anus, eyes & skin on elbows, knees, hands & feet. It may look like ATOPIC DERMATITIS
@ Patches evolve into crusted, blistered, pus filled eroded lesions
@ There is usually sharp demarcation between affected & normal skin
@ Skin around nails becomes inflamed (paronychia) & their may be nail ridging @ Mental retardation
@ Diffuse hair loss on the scalp, eyebrows & eye lashes.
@ Secondary infection with candida albicans & Staph aureus
@ Red glossy tongue & nail ulcers @ diarrhea
@ Impaired wound healing @ Deat
Treatment is with zinc supplement

3. 1. Acrodermatitis enteropathica

4. Acrodermatitis enteropathica

5. acrodermatitis enteropathica
Confluent parakeratosis
Extensive ballooning degeneration of the upper epidermis where the keratinocytes show pale cytoplasm and pyknotic nuclei
Confluent and focal keratinocytic necrosis
Intraepidermal vesiculation
Psoriasiform hyperplasia of the epidermis
Edema of the papillary dermis
A patchy lichenoid infiltrate consisting of lymphocytes, histiocytes, eosinophils and some neutrophils
Marked extravasation of red blood cells

6. 2. Napkin dermatitis
Candida albicans: erythematous papules and plaques with small satellite spots or superficial pustules
Infantile seborrheic dermatitis: cradle cap and bilateral salmon pink patches, often desquamating, in skin folds
Atopic dermatitis: bilateral scratched, dry plaques anywhere, but uncommon in nappy area; family history common
Psoriasis: persistent, well-circumscribed, symmetrical, shiny, red, scaly or macerated plaques; other sites may be involved; family history common
Disseminated eczema :
or auto eczematization: rash in distal sites associated with severe napkin rash
Primary irritant dermatitis: erythematous desquamative edamatous lesions on the diaper region, sparing the folds

7. Dermatosis affecting the napkin area
Disorder
Dermatosis affecting the napkin area
Features
Psoriasis
Scattered beefy red papules which may coalesce into well defined plaques, often lacks scale. Look for other skin/ nail signs of psoriasis or a family history
Primary
candidiasis
Erythema with satellite pustules, may be sharply demarcated with a raised edge, involves groin folds, often follows systemic antibiotics.
Acrodermatitis enteropathica
Treatment resistant eczematous looking rash, may blister or become crusty, look for perioral involvement
Granuloma
gluteale infantum
Red papule granulomastous nodules, a foreign body reaction to talc or barrier preparations, benign & self limiting
Langerhans cell
histiocytosis
Erythema & scale, progresses to purpuric nodules, extremely rare & may be serious

8. Neonate rashes

9. Neonate rash Milia in neonate Cutis marmorata in neonate
Napkin dedrmastitis in neonate

10. Childhood rashes Mongolian spot in neonate Miliaria crystallina
Acne neonatorum
Craddle cap (seborrheic dermatitis)

11. 3.

12. Maculopapular Childhood Rashes
Chicken Pox
Incubation: 10-21d, infective until crusted over
Rash: vesicles on macules (dewdrops on rosepetals),
Very pruritic!
Other symptoms: 1-3d prodrome of fever and respiratory symptoms
Treatment: supportive, acyclovir for severe disease, VZIG for post-exposure prophylaxis
Complications: 1st or 2nd trimester = congenital varicella syndrome

13. Maculopapular Childhood Rashes
Roseola
Incubation: 10-14d, dx with measles IgM
Rash: maculopapular, starts on face.
Non-pruritic!
Other symptoms: the 3 Cs
1) Cough 2) Coryza (runny nose) 3) Conjunctivitis
Koplik spots in mouth 1-2d before rash
Treatment: supportive, prophylactic Ig
Complications: secondary bacterial infection, encephalitis (1:1000), subacute sclerosing panencephalitis (1:100000)
Rubella or German Measles : third disease
Incubation: 14-21d, infective 5d before rash and 7d after
Rash: pink maculopapular, starts on face.
Pruritic!
Other symptoms: non-specific
Treatment: supportive
Complications: congenital rubella syndrome (very bad*), first four months of pregnancy highest risk (this is why we check rubella immunity status in prenatal screening)
* CONGENITAL RUBELLA SYNDROME “BLUEBERRY MUFFIN BABY” (PURPURA). CATARACTS/CONGENITAL GLAUCOMA, CONGENITAL HEART DISEASE, HEPATOSPLENOMEGALY, JAUNDICE, MICROCEPHALY, DEVELOPMENTAL DELAY
Fifth Disease or Erythema Infectiosum
Incubation: 4-14d, infective prior to onset of rash
Rash: slapped cheeks (raised uniform maculopapular lesions on cheeks), may appear on extensor surfaces
Usually not pruritic
Other symptoms: flu-like illness ~3d prior to rash
Treatment: supportive, blood transfusions if aplastic crisis
Complications: arthritis (10%), vasculitis
Aplastic crisis: reticulocytopenia, not bad in normal people, very bad anemia if you already have chronic hemolytic anemia
During pregnancy: fetal hydrops/fetal loss

14. Rash of rubella on skin of child's back
Rash of rubella on skin of child's back. Distribution is similar to that of measles but the lesions are less intensely red.
Rubella, also known as German measles or three-day measles, is a disease
caused by the rubella virus.

15. Kawasaki disease

16. Kawasaki disease
A) Bilateral, nonexudative conjuinctivitis with perilimbal sparing “conjunctival injection" (B) Strawberry tongue and bright red, swollen lips with vertical cracking and bleeding (C) Erythematous rash involving perineum (D) Redness of the palms, which is often accompanied by painful, brawny swelling of the back of the hands (E) Redness of the soles, and swelling of the back of the feet (F) Peeling of the skin of the fingers (G) Redness and induration at the site of a previous vaccination with Bacillus Calmettus - Guerin (H) Perianal redness and peeling

17. Kawasaki disease

18. IJDVL 78: 251, 2O12
Common causes of fever with rash in a child

19. Differential diagnoses based on morphology of rash

20. Summary of Paediatric Skin Rashes

21. Paediatric Skin Rashes
Is the rash papular (raised)?
Consider:
Urticaria
Mollascum contagiosum (pearly or fleshy, umbilicated, ie central depression in papule)
Scabies (itchy, excoriated, S-shaped burrows, which should be visible with a magnifying glass)
Insect bitesI
Kerastosis pilaris (keratin accumulation at the base of hair follicles)
Is it red and scaly?
With epidermal breakage (eczematous)?
Atopic eczema, typically involves itching erythematous patches, papules and plaques with moist crusted erosions on the face, neck and upper trunk, and also the elbows and knees.
Without epidermal breakage
Seborrhoeic dermatitis
Psoriasis
Tinea corporis/capitis
Pityriasis rosea

22. Paediatric Skin Rashes
Is it red but not scaly (and NOT purpuric)?
Consider:
Cellulitis
Kawasaka’s disease
Scarlet fever and the viral exanthemas, for example:
Roseola infantum – (sixth disease).
Primary human herpes virus (HHV-6 and HHV-7). The most common age is under two years. It is a frequent cause of infantile febrile seizures. Small blanchable pink macules and papules found on the trunk and neck. It is associated with high fever prior to defervescence and appearance of a rash on the fourth day. It is often asymptomatic.
Erythema infectosum - (slapped cheek syndrome or fifth disease) caused by parvovirus B19.
Measeles - presents as erythematous macules and papules - initially discrete, may become confluent on the face, neck and shoulders.[ On mucous membranes, Koplik's spots (tiny bluish-white papules with erythematous areolae) may develop. Also, upper respiratory tract infection with cough, malaise and fever subsiding as the rash increases (measles prodrome = the 4 Cs - cough, coryza, conjunctivitis and very cranky!).
Rubella (German measle) pink macules and papules starting on the forehead and spreading to the face, trunk and extremities on the first day. Fades from the face on the second day and the rest of the body by the third day. Petechiae on the soft palate before the rash. Low fever.
Scarlet fever (= scarlatina) exotoxin-mediated rash (Group A streptococcus) - sore throat, then general erythema (classically with perioral sparing), followed by confluent petechiae in skin folds (Pastia's sign) due to increased capillary fragility. Strawberry tongue (initially white, then red). Skin desquamation (peeling) frequently follows the rash.
Atopic dermatitis & eczema

23. Paediatric Skin Rashes
Is it red and purpuric?
Consider:
Meningococcal meningitis (not common but it should be excluded). Early on there may be a 2-10 mm macular or maculopapular rash (becoming apparent within the first 24 hours of disease) which is is sparsely distributed on the face, trunk and lower extremities and planches on pressure. Later as the disease develops, petechiae in the centre of macules become haemorrhagic (and do not blanche). Use the 'glass test' to assess 'blanchability' of the rash by placing a glass tumbler against lesions and applying pressure.
Henoch-Schönlein purpura
Idiopathic thrombocytopenic purpura (ITP), leukaemia and other haematological disorders
Trauma, nonaccidental injury
Enteroviral infections
Other miscellaneous conditions
Warts
Head lice

24. Gianotti-Crosti syndrome papular acrodermatitis of childhood
It is a rare eruption that often follows an upper respiratory tract infection& can be associated with mild systemic upset.. It affects children at any age with an equal sex distribution. It is a reaction of the skin to a viral infection: Hepatitis B, Epstein Barr virus are the most frequently reputed etiology. Other incriminated virus: Hepatitis A, cytomegalovirus, coxsacki virus, adenovirus, enterovirus, rubella…
The rash consists of multiple small monomorphus lichenoid papules that may be skin colored or red. It is non-itchy , symmetrical & affects the face, extremities, buttocks, palms & soles. It may be associated with a low grade fever, lymphadenopathy & hepatosplenomegaly. The rash is self limiting & normally settles in 2 – 8 weeks .
Differential diagnosis: Acrodermatitis enteropathica, erythema multiformis, hand,foot & mouth disease, Henoch - Shonlein purpura, Kawasaki disease, lichen planus, papular urticaria, scabies.
Treatment is with emollient. Topical steroid may exacerbate the condition.

25. Papular acrodermatitis of childhood
The lesions predominantly affect the limbs and spare the trunk, are monomorphic (not polymorphic) red papules and occur in childhood. Although in the cases described by Gianotti and Crosti hepatitis B was identified as the cause, this has not been the British or North American experience where the children have been otherwise well. The eruption disappears after a few weeks

26. 4.Gianotti- Crosti syndrome

27. 4. Gianotti-Crosti syndrome 5. papular acrodermatitis of childhood
The Gianotti-Crosti syndrome is a characteristic response of the skin to viral infection in which there is a papular rash which lasts for several weeks. Other names sometimes used for this skin condition include papulovesicular acrodermatitis of childhood, papular acrodermatitis of childhood and acrodermatitis papulosa infantum.
The specific viruses causing Gianotti-Crosti syndrome include: hepatitis B inection, Epestein Barr virus, Enterovirus infection, Echo viruses, respiratory syncetial virus
Who is affected?
Gianotti-Crosti syndrome mainly affects children between the ages of 6 months and 12 years. A clustering of cases is often observed. A preceding upper respiratory infection is common.

28. Gianotti-Crosti syndrome An Bras. Derm. 79: nO. 6, 2OO4
Gianotti-Crosti disease is also called acrodermatitis of childhood. These red, elevated lesions do not contain pus and can occur on the limbs, buttocks, face, and neck.

29. Gianotti-Crosti syndrome
Focal parakeratosis overlying a small wedge-shaped lesion
Focal spongiosis in upper epidermis with atypical lymphoid cells
Slight psoriasiform hyperplasia
Marked edema of papillary dermis and extravasation of erythrocytes
A moderately dense superficial and deep perivascular infiltrate consisting of lymphocytes and histiocytes

30. 6. Erythema toxicum neonatorum

31. Erythema toxicum neonatorum
It appears in up to half of neonates carried to term, usually between day 2 -5 after birth. it clears up by 2 weeks of age. It appears as blotching erythematous macules with central vesicles, papules or pustules. It does not require treatment.
It is due to activation of immune system. Some neonates are more sensitive than others& develop erythematous spots all over the body or may be due to hypersensitivity to detergents in bed sheets & clothing

32. Erythema toxicum neonatorum
An intrafollicular subcorneal pustule containing eosinophils and neutrophils
Eosinophillic exocytosis and spongiosis of the epidermis
Edema of the papillary dermis
A moderately dense infiltrate of eosinophils around the superficial blood vessels
Eosinophils between collagen bundles and around follicular epithelium

33. 7. Blue berry muffin babies
Blueberry muffin babies present with widespread rash of blue red papules & nodules at birth, The rash is due to congenital infection in utero

34. Blueberry muffin syndrome

35. Causes of blue berry muffin baby
Disease
Causes of blue berry muffin baby
Additional features
Toxoplasmosis
Hepatosplenomegaly, lymphadenopathi\y,
Hydrocephalus, retinitis& seizures. Prognosis is poor
Herpes zoster
Scarring limb contractures, encephalitis, malformed limbs, growth retardation
Human immunodeficiency
virus
Babies may have opportunistic infections & hepatosplenomegaly
Cytomegalovirus
May be severe with deafness, mental retardation &
seizures
Rubella
Mental retardation, deafness , microcephaly, cataracts, hepatosplenomegaly, pneumonitis, myocarditis, encephalitis, retinopathy

36. Blueberry Muffin Baby
Generalised non-blanching, blue-red macules or firm dome-shaped papules on a neonate’s skin, mostly over the head, neck, trunk: a sign of extramedullary dermal haematopoiesis persisting beyond what normally occurs up to the fifth month of gestation. In the strictest sense the term describes the appearance of congenital TORCH infections; but nowadays may also refer to similar lesions in some haematologic dyscrasias and vascular malformations..
Congenital Infections
rubella — for which this term was first coined
CMV (cytomegalovirus) — most common cause
rarely, other TORCH infections
Haemolytic diseases (Coombs positive haemolytic anaemia, unconjugated hyper-bilirubin-aemia, hydrops)
haemolytic disease of the newborn
hereditary spherocytosis
Malignancies
neuroblastoma (blanchable lesions, raccoon eyes, heterochromia irides)
AML (acute myeloid leukaemia) with leukaemia cutis (chloromas, pallor, lethargy, hepatosplenomegaly, fever, leukocytosis)
Langerhans cell histiocytosis (yellow-brown papules with central ulceration, multiple petechiae)
Vascular malformations
multiple haemangiomas of infancy
multifocal lymphangioendotheliomatosis
blue rubber bleb naevus syndrome
multiple glomangiomas
others
neonatal lupus erythematosus (occasionally)

37. 8. Subcutaneous fat necrosis of the newborn
It is a rare condition seen in babies of 2-3 weeks of age. It presents with one or more erythematous subcutaneous nodules that may coalesce into plaques. It is often precipitated by cold 7 seen on the trunk, arms buttocks, thighs & cheeks. Rarely, the skin may ulcerate & necrotic fat exude. It can be associated with significant hypercalcemia that may require treatment. The condition is self limiting & resolves over a few months

38. Subcutaneous fat necrosis of the newborn

39. Subcutaneous fat necrosis of the newborn
The exact cause of subcutaneous fat necrosis is not known. A number of factors seem to be more common in affected infants including:
@ Fetal stress during delivery @ Low oxygen level @ Cold Caesarian section @ Large birth weight @ Infection
However most babies who are exposed to these factors do not get subcutaneous fat necrosis.
What are the complications?
The nodules of subcutaneous fat necrosis may be painful. They may last from weeks to months before they disappear. Occasionally the infant may be left with a dimpled area of reduced fat.
The most common complication of subcutaneous fat necrosis is hypercalcaemia (high blood calcium). This is detected on a blood test, so babies with subcutaneous fat necrosis should have their blood calcium checked periodically for the first few months of life. Hypercalcaemia can cause irritability, constipation, poor weight gain and, very rarely, heart rhythm disturbance.
Thrombocytopaenia (low platelets) and hyperlipidaemia (raised levels of fat in the blood) have also been observed

40. Subcutaneous fat necrosis of the
Subcutaneous fat necrosis of the. Histologic Features: lCharacteristic radial arrays of needle-shaped clefts (dissolved triglyceride crystals) within macrophages and adipocytes Patchy fat necrosis Mixed inflammatory infiltrate, both septal and lobular

41. 9. Neonatal herpes simplex infection
It occurs during delivery with
Direct spreads from vaginal
Lesions, when lesions do occur they are typically papules that progress to grouped vesicles. The infection may be limited to the skin, involve the central nervous system or can be disseminated. Untreated disseminated infection has a mortality of over 8O%

42. Neonatal Herpes Simplex Infection
Clusters of vesicles on an erythematous base are characteristic and may be present on almost any part of the body.

43. Neonatal Herpes Simplex Virus Infection
Algorithm for testing and counseling women with involvement of the male partner. AROM = artificial rupture of membranes
Algorithm for testing and counseling women without involvement of the male partner. AROM= artificial rupture of membranes.

44. 1O. Langerhans cell histiocytosis
It is a part of clinical histiocytosis which are characterized by abnormal proliferation of histiocytes. It is clinically derived into three groups:
1. Unifocal LCH( older name is eosinophilic granuloma which is now a misnomer)
2. Multifocal unisystem LCH. The triad of diabetes insipidus ,exophthalmos& lytic bone lesion is known as Hand- Schuller- Christian triad.
3. Multifocal multisystem LCH . Also called Letter –Siwe disease
@ Some consider LCH a reactive process.
Argument with: Occurrence of spontaneous remission, extreme secretion of multiple cytokines by dendretic cells& by standers cells in lesional tissue, favorable prognosis& good survival rate without organs involvement
@ Neoplastic process:
Argument supporting: Clonality is an important attribute of cancer; activating mutation of protooncogen in the Raf family. BRAF gene

45. (Eosinophilic granuloma) Age affected Over 6 years Prognosis Good
Unifocal LCH
(Eosinophilic granuloma)
Age affected
Over 6 years
Prognosis
Good
Slowly progressive single lesion in bone, skin or soft tissues. Often an osteolytic lesion in a long or flat bone, it may also present as an ulcer, otitis media (mastoid lesion) or tooth disruption (jaw lesion). Treated with curettage or low dose radiotherapy. 1O% progress to multifocal disease
Multifocal
System LCH
3 – 6 years
Moderate
Classical triad of diabetes insipidus (pituitary involvement), exopthalmos & lytic bone lesions. It may also present with a seborrheic dermatitis-like rash, ulceration, bone pain & chronic cough. Treated with radiotherapy & chemotherapy. Spontaneous remission is possible. Lung involvement is a poor sign
Multisystem
LCH
(Malignant
Letterer-Siwe disease)
Less 3
years
Poor
Rapidly progressive multisystem disease. Patient may be systemically unwell with as generalised skin eruption, multiple LCH lesions, anemia, hepatosplenomegaly, lymphadenopathy& lung involvement. Treatment is with immunosuppressives & chemotherapy. It is often fulminant & fatal

46. Langerhans cell histiocytosis histiocytosis X

47. Langerhans Cell Histiocytosis

48. Langerhans cell histiocytosis
C D 1 a marker specific

49. Langerhans cell histiocytosis

50. 11. Childhood Hypopigmentation Disorders Acta Dedrmato Venereologica 9O : 6, 2O1O

51. Localized hypopigmentation presenting in early childhood Acta Dermato Venereologica 9O : 6, 2O1O

52. Generalized hypopigmentation presenting in an older child Acta Dermato Venereologica 9O :6, 2O1O

53. Hypopigmentary disorders presenting in later childhood Acta DetrmastoVenereologica 9O :6, 2O1O

54. Diffuse hypomelanoses in children
Diffuse hypomelanoses in children. OCA= oculocutaneous albinism, SD= syndrome, *Eye abnormalities: discussion, **Eye abnormalities also possible

55. Cutaneous hypomelanoses in children with localized or diffuse light hair

56. Depigmentation; (a) Vitiligo; (b) Halo nevi; (c) Piebaldism

57. Piebaldism; (a) Before and (b) After treatment with non-cultured epidermal cell transplantation

58. Hypopigmentation; (a) Naevus depigmentosus; (b) 'Ash leaf' macula in tuberous sclerosis