Case of acquired cutis laxa with preceding urticarial eruption treated by diphenyl sulfone.

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 ×4000 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.

Figure 1

(a) Loose skin and wrinkles around the mouth and chin. (b) Pendulous skin and longitudinal wrinkles on the ear lobes. (c) Inelastic skin with some wrinkles and folds on the anterior neck. (d) Inelastic skin with some wrinkles and folds in the left axillae. (e) Edematous erythemas on the upper arm. (f) Neutrophilic inflammatory infiltrations around vessels and between collagen bundles in the whole dermis in urticarial eruption on the left arm (hematoxylin–eosin). (g) Elastic fibers were sparse, shortened and fragmented in the dermis (elastica van Gieson stain). (h) Neutrophils adhering to the elastic fibers (electron microscopy, ×4000). C, collagen fiber; E, elastic fiber; N, neutrophil.

Acquired CL is characterized by a reduced number and abnormal properties of elastic fiber. The disease affects the elastic fibers, resulting in loss of elasticity. The pathogenesis of acquired CL is unknown, but various hypotheses have been advanced including excessive elastase activity of neutrophils, an immune‐mediated mechanism or dysfunction in elastase inhibitors.1

Over 20 years, six cases of acquired CL with urticarial eruption were reported. In each case, after several years of urticarial eruption, loosening and wrinkling began. Thus, acquired CL might have been triggered by urticarial eruption. In three cases,2, 3, 4 a biopsy was performed on an urticarial lesion, and revealed the presence of neutrophils, while the elastic fibers were thin and fragmented. In our case, neutrophils adhered to elastic fibers. We suggest that abnormality of elastic fibers may be associated with infiltration of neutrophils. Bouloc et al.4 reported 2–3‐times more fibroblast elastase activity in an urticarial eruption in culture, than in the control and lax skin. However, the exact mechanism of neutrophilic infiltration and the process of elastolysis have not been determined.

No definitively effective therapy for acquired CL has been established. In our case, we suspect that neutrophils played a significant role in the mechanism of elastic fiber destruction. We chose DDS to target the neutrophils and, based on the results, we infer that DDS may offer an effective treatment for acquired CL with urticarial eruption.