Dermatology Flashcards
X-linked dominant gene IKBKG (inhibitor of kappa B kinase gamma, previously NEMO) - what is the disorder?
Incontinentia Pigmenti
What are the four stages of incontinenta pigmenti?
1) Bullous stage - X-linked dominant gene IKBKG (inhibitor of kappa B kinase gamma, previously NEMO),
2) Verrucous stage - dry and hyperkeratotic, forming verrucous plaques after 4mo
3) Pigmentary stage - Hyperpigmentation is more often apparent on the trunk than the limbs and is distributed in macular whorls, reticulated patches, flecks, and linear streaks that follow Blaschko lines. These lesions disappear after age 14
4) Atretic stage - hairless, anhidrotic, hypopigmented patches or streaks occur as a late manifestation
What other deficits are seen with Incontinenta pigmenti?
Dental anomalies, CNS manifestations (ID, seizures, microcephaly), hemiplegia, hemiparesis, spasticity, microcephaly, and cerebellar ataxia.
Ocular anomalies, such as retinal neovascularization, microphthalmos, strabismus, optic nerve atrophy, cataracts, and retrolenticular masses, occur in > 30% of children
A herald patch and then christmas tree pattern is seen in this condition - what is it called?
What virus often contributes to it?
Pityriasis rosea
HHV 6 and HHV 7
What syndrome?
- Acute febrile neutrophilic dermatosis.
• Presence of painful, oedematous, and erythematous
papules, plaques, or nodules on the skin.
Sweet syndrome
(Lesions are usually several millimetres to several centimetres in diameter and exhibit a brightly erythematous or violaceous colour)
With hair loss, if the hair is falling out in clumps - what should you think about?
TELOGEN EFFLUVIUM
With hair loss, if the hair is falling out and there is an associated itch?
Tinea Capitis
Exclamation-point hairs are associated with?
Alopecia Areata
Positive hair-pull test result is associated with?
Telogen Effluvium
Alopecia areata is a _________ cell drived autoimmune disorder producing nonscarring alopecia.
T-cell driven disorder
Most common eye anomaly with sturge weber syndrome?
Glaucoma
What is the gene mutation and inheritance pattern of Hyper IgE syndrome?
STAT3 and AD
What is this?
Cold abscesses Recurrent sinopulmonary infections Mucocutaneous candidiasis Torturousity of vessels Retention of primary teeth
Hyper IgE syndrome
A skin biopsy reveals satellite dyskeratosis =
Transfusion related GvHD
What does intra- epidermal cleavage at the granular layer indicative of?
SSSS
