[Erythema elevatum et diutinum]. / Erythema elevatum et diutinum.

Erythema elevatum et diutinum (EED) is a rare, chronic, cutaneous, leukocytoclastic vasculitis. It is characterized by symmetric, plaques, papules and nodules occurring preferentially on the extensor aspects of the arms. An association with chronic infections, neoplasms, especially myeloproliferative diseases and paraproteinemia, as well as autoimmune disorders (rheumatoid arthritis, Crohn disease) has been described. An 83-year-old woman with EED presented with symmetric, skin colored, firm nodules on the extensor surfaces of her fingers for two years. This case report summarizes the central clinical aspects and differential diagnosis of EED as well as its therapeutic options.

[Incontinentia pigmenti (Bloch-Sulzberger syndrome)]. / Incontinentia pigmenti (Bloch-Sulzberger-Syndrom).

A female infant, aged two weeks, presented with linear erythematous crusted papules, plaques and blisters on the right leg which had occurred two days after birth. Histological examination revealed typical features of incontinentia pigmenti in the inflammatory stage. Incontinentia pigmenti is a rare X-linked dominant genodermatosis caused by mutations in the NEMO gene located at Xq28 affecting the skin, different organ systems, the central nervous system, eyes, teeth and skeleton with variable expression. We summarize important clinical and diagnostic aspects of incontinentia pigmenti as well as its genetic and molecular basis.

[Classical and atypical presentations of Galli-Galli disease]. / Klassische und atypische Präsentation des Morbus Galli-Galli.

Galli-Galli disease is a rare genodermatosis which classically presents with reticulate hyperpigmentation of the flexures and intertriginous areas. Recently, an atypical clinical presentation of Galli-Galli disease with monomorphic lentigo-like maculae and papules has been reported. We describe two cases of Galli-Galli disease, one patient presenting with a classical form and another with atypical skin lesions. In spite of the clinical differences, the histopathological examination - with filiform elongated rete ridges and acantholysis - confirmed the diagnosis of Galli-Galli disease in both cases.

[Solarium-induced pseudoporphyria and variegate porphyria as rare differential diagnoses of porphyria cutanea tarda]. / Solariuminduzierte Pseudoporphyrie und Porphyria variegata als seltene Differenzialdiagnosen der Porphyria cutanea tarda.

Three patients presented with typical porphyria cutanea tarda-like vesicles, erosions and scars as well as increased fragility, primarily on the back of the hands. In two of the three, porphyrin workup was normal. Skin biopsy was compatible with porphyria cutanea tarda (PCT) or pseudoporphyria. The common aspect in the patients' history was the frequent use of solaria for many years, so that UV-induced pseudoporphyria was diagnosed. Treatment was strict abstention from UV radiation and regular dermatologic controls for signs of skin damage. Porphyrin analysis in the third patient showed normal excretion of total urine porphyrins and precursors; however, fecal porphyrins were elevated with dominating coproporphyrins in HPLC and the plasma fluorescence scan yielded a peak at 625 nm. Subsequent mutation analysis showed a mutation in the protoporphyrinogen oxidase gene, thereby confirming the diagnosis of variegate porphyria. Five months after the initial diagnosis the patient presented with the first acute attack. Further investigations revealed a metastasized carcinoma of the colon, which probably triggered the acute attack. Our cases show rare differential diagnoses in patients presenting with typical PCT-like skin lesions. The discrimination between porphyria cutanea tarda and its differential diagnoses is very important since it has an important impact not only on the treatment modality but also on the course and the prognosis of the disease.