We present the features of six children who have been seen amongst 154 consecutive new patients with lichen planus in our outpatients clinic. All but one of the children had unusual forms of the disease and there was a family history of lichen planus in three. Lichen planus is rare in childhood and often assumes atypical clinical patterns.

We describe a patient in whom chronic leg ulceration was due to prolidase deficiency. The clinical features of this condition are described and we discuss the metabolic abnormality and the treatment regimes which have been employed. We also report the further finding of erosive cystitis, which we consider should be added to the list of clinical features of prolidase deficiency.

Four patients with erythropoietic protoporphyria (EPP) are reported, in whom oral iron produced clear-cut clinical and biochemical deterioration. This suggests that there are two biochemically and genetically distinct sub-groups of EPP patients distinguished by their dramatically different response to oral iron.

A patient with extensive histologically proven Hailey-Hailey disease is described whose initial clinical presentation was suggestive of erythema multiforme or toxic epidermal necrolysis. This potentially misleading morphology of acute proven Hailey-Hailey disease has not been described previously and may be a consequence of bacterial infection exacerbating acantholysis.

No abstract