Dermatology

Question 1

Incontentia pigmenti

Answer 1

-This is typically lethal in males antenatally. - X-linked

In most patients, cutaneous manifestations are present at birth or occur within the first 2 weeks of life. The cutaneous manifestations usually appear in a characteristic, chronologic sequence.
-Other systemic manifestations, including ocular defects, CNS abnormalities, and dental abnormalities, may not be recognised until infancy or early childhood.

-Diagnostic criteria for incontinentia pigmenti have been proposed. In the absence of a family history, the presence of at least 1 major criterion is necessary. The presence of minor criteria supports the diagnosis of incontinentia pigmenti.

Major criteria are:

typical neonatal vesicular rash with eosinophilia

• typical blaschkoid hyperpigmentation on the trunk, fading in adolescence
• linear, atrophic hairless lesions.

Minor criteria are:

• dental anomalies,
• alopecia,
• wooly hair,
• abnormal nails

With a definitive family history, the presence of any major criterion strongly supports the diagnosis of incontinentia pigmenti.

Question 2

epidermolysis bullosa

Answer 2

• group of inherited diseases characterised by skin fragility and blister formation caused by minor skin trauma

Question 3

HSV

Answer 3

• Herpes simplex virus (HSV) infection – skin, eye, and mouth (SEM) disease accounts for approximately 45% of neonatal HSV.
• SEM disease usually presents in the first two weeks of life but may occur at any time during the first six weeks of life (usually in the first month).
• Localised skin disease is associated with coalescing or clustering vesicular lesions with an erythematous base

Question 4

Ichthyosis

Answer 4

– the ichthyosiform dermatoses are a diverse group of hereditary skin disorders characterised by the accumulation of “fish-like” scales resulting from abnormal epidermal cell kinetics or differentiation.
-The severity of the individual disorders ranges from asymptomatic to life-threatening.

• Icthyosis vulgaris – mildest form. Presents during childhood, not apparent in the newborn.
• X-linked icthyosis – affects males, onset at 2-6 weeks of age, worsens with age. Large brown adherent scales.
• Lamellar icthyosis – affected patients present as a “collodian baby” at birth.
• CIE (congenital icthyosiform erythroderma) – also presents as a “collodian baby.”
• Epidermolytic ichthyosis – also known as bullous ichthyosis or bullous CIE. Presents with widespread blistering and erythema.
• Harlequin ichthyosis – the most severe form, often lethal perinatally.

Question 5

SJS

Answer 5

ADD

Question 6

SSSS

Answer 6

ADD

Question 7

TEN

Answer 7

ADD

Question 8

Zinc deficiency

Answer 8

can produce a bullous pustular dermatitis, often on the face & anogenital area