| Neonatal Dermatology | ||
| #1 | Healthy infant with subcutaneous nodules. Histology shows lymphocytes, histiocytes, giant cells, needle-shaped clefts, fat necrosis, calcification. Hypercalcemia upon resolution.  |
Subcutaneous Fat Necrosis of the Newborn |
| #2 |  |
Aplasia Cutis Congenita (ACC) |
| #3 |  |
Acute Hemorrhagic Edema of Infancy (Finkelstein Disease) |
| #4 |  |
Bohn Nodule |
| #5 | Infant with Hirschprung's  |
Jacquet's Erosive Dermatitis |
| Childhood Infectious Disease | ||
| #1 | Etiology of hand, foot and mouth disease | Coxsackie A16 |
| #2 |  |
Erythema Infectiosum / Fifth Disease (Parvovirus B19) |
| #3 | Leading cause of acquired heart disease in children | Kawasaki's disease |
| #4 |  |
Gianotti-Crosti Syndrome (avoid steroid creams) |
| #5 | Child with Rubella  |
Forschheimer Spots |
| Pigmented Lesions | ||
| #1 | Genetic defect?  |
Peutz-Jeghers Syndrome (STK11 / LKB1) |
| #2 |  |
Nevus Spilus / Speckled Lentiginous Nevus |
| #3 | This finding along with macrocephaly, lipomas and hemangiomas  |
Bannayan-Riley Ruvalcaba |
| #4 | VBR1 gene, nasal alar hypoplasia, hypothyroidism, congenital deafness, pancreatic achylia  |
Johanson-Blizzard Syndrome |
| #5 | Keratin 14 mutation, palmoplantar hypohidrosis, hyperkeratoses, poor dentition, no dermatoglyphics.  |
Naegeli-Franceschetti-Jadassohn |
| Dermal Tumors and Disorders | ||
| #1 | What malignancy is associated with NF-1 and multiple Juvenile Xanthogranulomas? | CML |
| #2 | Positive Darier's Sign. What is the mutated receptor?  |
C-Kit (CD 117) |
| #3 |  |
Dermoid Cyst |
| #4 |  |
Infantile Digital Fibroma |
| #5 | Oral mucosal and gingival lesions, alveolar bone loss, and this finding:  |
Floating teeth, Langerhans Cell Histiocytoses |
| Vascular Lesions | ||
| #1 |  |
Lymphangioma Circumscriptum |
| #2 | s/p parotid surgery:  |
Auriculotemporal Syndrome (Frey's Syndrome) |
| #3 | Livedo reticularis, CVAs, antiphospholipid antibodies | Sneddon Syndrome |
| #4 | FLTA gene, autosomal dominant lymphedema  |
Milroy's Disease |
| #5 | RASA1 mutation, multiple fast-flow AVMs and limb hypertrophy  |
Parkes-Weber Syndrome |
| Final Question | |
| What is the gene defective in Acrodermatitis Enteropathica and the zinc-dependent enzyme that is low in that disorder? | SLC39A4, alkaline phosphatase |