Fitz9. Ch49: Keratosis Pilaris

Question 1

Case: small keratotic follicular papules with variable degrees of perifollicular erythema on the lateral cheeks, extensor arms, thighs and buttocks

1. disease
2. associated diseases
3. Histopath?
4. mutation?

Answer 1

1. keratosis pilaris
2. KIA: ichthyosis vulgaris, atopic dermatitis
3. follicular keratosis
4. filaggrin

Question 2

markedly noticeable ERYTHEMA and follicular papules on cheeks, forehead, neck

Answer 2

keratosis pilaris/ follicularis rubra

Question 3

young male with erytheam and follicular papules with HYPERPIGMENTATION on symmetric preauricular and maxillary areas

Answer 3

Erythromelanosis follicularis facieie et coli

Question 4

erythema and small keratotic follicular papules on the lateral third of the eyebrows causing scarring alopecia

1. disease?
2. seen in what syndromes? (2)
3. disease if in the cheeks?

Answer 4

1. ulerythema ophryogenes: eyebrows
2. Noonan and cardiofaciocutaneous syndromes
3. keratosis pilaris atrophicans: cheeks

Question 5

honey-comb/ reticular/ worm-eaten like atrophy on the cheeks; no alopecia; with sparse comedones and milia

1. disease?
2. associated with what syndrome? mutation?

Answer 5

1. atrophoderma vermiculatum/ folliculitis ulerythematosa reticulata
2. Loeys-Dietz syndrome (TGFBR2)

Question 6

girl with spiny lesions on the scalp progressing to SCARRING patchy alopecia and following the lines of Blaschko with nail dystrophy and hyperkeratosis of elbows

1. disease?
2. triad?

Answer 6

1. keratosis follicularis spinulosa decavans (KFSD)

2. follicular hyperkeratosis (ichthyosis, alopecia, atrichia, PHOTOPHOBIA

Question 7

NONSCARRING congenital atrichia with spiny follicular projections (NUTMEG GRATER), photophobia

Answer 7

ichthyosis follicularis atrichia and photophobia syndrome

IFAP

Question 8

skin colored to erythematous folliculocentric papules with brittle keratotic spiny spicules; leonine facies

1. disease?
2. drug-induced folliculodystrophy
3. viral-induced?
4. histopath?

Answer 8

1. trichodysplasia spinulosa
2. cyclosporine
3. polyomavirus
4. expanding inner root sheath with eosinophilic trichohyaline granules