Transcript PowerPoint Presentation - Abdel Hamid Derm Atlas

Childhood rashes
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The following items will be discussed:
@ Acrodermatitis enteropathica
@ Dermatosis affecting the napkin area
@ Gianotti- Crosti syndrome
Papular acrodermatitis of childhood
@ Erythema toxicum neonatorum
@ Neonate rashes
@ Blueberry muffin babies
@ Subcutaneous fat necrosis of the newborn
@ Neonatal fat necrosis of the newborn
@ Langer
Acrodermatitis enteropathica
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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
Acrodermatitis enteropathica
Acrodermatitis enteropathica
Dermatosis affecting the napkin area
Disorder
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
Gianotti-Crosti syndrome
papular acrodermatitis of childhood
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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.
Gianotti- Crosti syndrome
Erythema toxicum neonatorum
Erythema toxicum neonatorum
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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
Neonate rashes
Neonate rash
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Milia in neonate
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Cutis marmorata in neonate
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Napkin dedrmastitis in neonate
Childhood rashes
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Mongolian spot in neonate
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Miliaria crystallina
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Acne neonatorum
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Craddle cap (seborrheic
dermatitis)
Blue berry muffin babies
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Blueberry muffin babies present
with widespread rash of blue red
papules & nodules at birth, The
rash is due to congenital infection
in utero
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
Subcutaneous fat necrosis of the newborn
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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
Subcutaneous fat necrosis of the newborn
• Neonatal herpes simplex
infection
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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%
Langerhans cell histiocytosis
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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
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
Multifocal
Multisystem
LCH
(Malignant
LettererSiwe
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
Langerhans cell histiocytosis