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Case of acquired cutis laxa with preceding urticarial eruption treated by diphenyl sulfone
Author(s) -
Takenaka Yuko,
Ishiguro Naoko,
Kawashima Makoto
Publication year - 2018
Publication title -
the journal of dermatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.9
H-Index - 65
eISSN - 1346-8138
pISSN - 0385-2407
DOI - 10.1111/1346-8138.14465
Subject(s) - medicine , pathology , histopathology , cutis laxa , abdomen , elastic fiber , anatomy
Dear Editor, A 35-year-old Chinese woman presented with a 9-month history of progressive loosening and wrinkling. For the preceding 3 years, she had suffered from urticarial eruptions which lasted several days, and were intractable to oral antihistamines. Physical examination revealed looseness and wrinkling of the eyelids, and around the mouth and chin (Fig. 1a). The earlobes were pendulous and loosened (Fig. 1b). Wrinkling with decreased elasticity was observed on the neck (Fig. 1c) and axillae (Fig. 1d). Edematous erythema without pruritus was observed on her arm (Fig. 1e) and abdomen. There was no hyperextensibility of the joints and fragility of the skin. Blood test and urine analysis were within the normal range. Chest X ray, chest and abdominal computed tomography, echocardiography and gastroscopy were normal. Histopathological examination from a urticarial eruption showed infiltrations of neutrophils around the blood vessels and between the collagen bundles (Fig. 1f). The histopathology of inelastic skin showed several clefts between the collagen bundles, and infiltrations of neutrophils and lymphocytes around the blood vessels. There was no infiltration of mast cells both in an urticarial eruption and in elastic skin. Elastica van Gieson staining revealed that the elastic fibers were sparse and shortened (Fig. 1g). In electron microscopy, several elastic fibers were present in each field under a 94000 magnification. We observed the adherence of one neutrophil to one fiber in almost all elastic fibers in each field (Fig. 1h). The length of the elastic fibers was less than normal. We diagnosed the patient with acquired cutis laxa (CL) with urticarial eruption. Treatment was initiated with diphenyl sulfone (DDS) to alleviate the urticarial eruption. There has been neither urticarial eruptions nor progress of loosening for 5 years under the treatment. Acquired CL is characterized by a reduced number and abnormal properties of elastic fiber. The disease affects the