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Mastocytosis is diagnosed without difficulty if it presents with easily recognizable lesions of urticaria pigmentosa. Recently, we have identified hardly visible skin lesions of mastocytosis in Hymenoptera venom allergic patients ("occult mastocytosis"). In addition, in approximately 15% of the patients with typical cutaneous lesions, urticaria pigmentosa was at first mistaken for other conditions and thus not linked to simultaneous symptoms of systemic mastocytosis. In most patients with unrecognized mastocytosis, the diagnosis was supported by raised basal serum tryptase levels. Cutaneous mastocytosis is often overlooked and more frequent than assumed. Measurement of basal serum tryptase concentrations can make an important contribution to the diagnosis of mastocytosis, but it does not replace a meticulous skin examination.

A 44 years old male is reported who had aquired dermatochalasis and urticaria pigmentosa at the same time. This is interpreted as a subsequent phenomenon of a dermatosparaxis endoplasmatica. Clinic, histology and electron microscopy are elaborated. Systemic involvement is verified by x-rays. Histologically, predominant is the severe atrophy of the elastica, the collagenous degeneration, the presence of mastocytes, the subepidermal blister formation and the secondary xanthomatosis. The picture deviates from the common dermatosparaxis occuring in calves by the paracrystalline content of the procollagene in the endoplasmatic reticulum as verified by electron microscopy. A severe disturbance of the synthesis of collagene exists in the state of procollagene for which here the term "dermatosparaxis endoplasmatica" is suggested.