Erythropoietic protoporphyria without skin symptoms-you do not always see what they feel

Erythropoietic protoporphyria (EPP) is an inherited disorder of the porphyrin metabolism that often remains undiagnosed in children. We report on a 4-year-old girl who had been suffering for 1 year from recurrent painful crises affecting her hands, feet, and nose following sun exposure. Objective skin lesions were absent until the age of 6. Porphyrin analysis revealed elevated free erythrocyte protoporphyrin (FEP) levels confirming the diagnosis of EPP. This illustrates that skin lesions might be completely absent in children affected with EPP, a fact that has only been reported once previously. Because EPP can manifest with few and unspecific cutaneous symptoms or no skin lesions at all, like in this patient, the diagnosis of EPP might be delayed or missed. EPP should be excluded in all photosensitive children, especially when discomfort is disproportionate to the extent of the cutaneous lesions. The clinic, pathophysiology, diagnosis, complications, and therapy of EPP are discussed

Two siblings with lichen planus and squamous cell carcinoma of the oesophagus

Lichen planus is a mucocutaneous disease which can also affect the oesophagus. Unlike in oral lichen planus an increased risk for the development of squamous cell carcinoma in the oesophagus has not been established. We describe two sisters with a history of long-standing cutaneous lichen planus who developed oesophageal squamous cell carcinoma, diagnosed at the ages of 68 and 70 years, respectively. In one of the cases, dysplastic areas were identified by high-magnification chromoendoscopy. In both cases, oesophageal resection was carried out with a curative intent. For the first time these sibling case reports suggest an increased precancerous potential of oesophageal lichen planu