SHORT COMMUNICATION
979
ActaDV ActaDV
Advances in dermatology and venereology Acta Dermato-Venereologica
Uncommon Presentation of Scleromyxoedema with a Distribution Pattern Mimicking a Photodermatosis
Michael Constantin KIRCHBERGER, Michael STICHERLING and Michael ERDMANN * Department of Dermatology, University Erlangen, Ulmenweg 18, DE-91052 Erlangen, Germany. * E-mail: Michael. Erdmann @ uk-erlangen. de Accepted Apr 18, 2017; Epub ahead of print Apr 19, 2017
Scleromyxoedema was first described by Dubreuilh( 1) & Reitmann( 2) as lichen myxoedematosus. Also known as Arndt-Gottron disease, this disorder belongs to the group of primary cutaneous mucinoses and presents as a generalized waxy, papular eruption, usually accompanied by monoclonal gammopathy. Without therapy, the dermal mucinosis spreads and systemic manifestations can occur, resulting in significant morbidity and mortality. We report here a case of scleromyxoedema in a young woman, which initially mimicked a photodermatosis that responded to intravenous immunoglobulin( IVIG) therapy.
CASE REPORT
A 32-year-old woman initially presented to her local dermatologist with facial erythema after a holiday in Egypt. Within several days the erythema had spread to her upper extremities and neckline. Consistent with photodermatosis, the erythema was strictly limited to areas of sun exposure, omitting the periorbital region, the skin beneath the chin, and 2 linear areas over the temples( Fig. 1). A biopsy showed chronic eczema and the physician diagnosed a dermatitis solaris, which was treated with topical glucocorticoids without improvement. Because of spreading and continuous induration of the eruption, the patient presented at our department. She described limited mobility and rigid facial expression with difficulties in opening her mouth. Light provocation and patch testing for contact eczema did not reveal any
Fig. 1.( a) Frontal and( b) profile view of the face showing erythema omitting the periorbital region and over the temples. A written permission is given by the patient to publish these photos.
pathological findings. Blood testing showed monoclonal gammopathy of immunoglobulin G with predominant lambda light chains. Tests for antinuclear antibodies and myositis-associated autoantibodies were negative. Capillaroscopy revealed a normal capillaroscopic pattern. Haematoxylin and eosin( H & E) and Alcian blue staining of a skin biopsy revealed prominent and diffuse mucinosis in the upper dermis with increased collagen deposition and irregularly arranged fibroblasts( Fig. 2) with no histological evidence of cutaneous lupus erythematodes or dermatomyositis. Based on the clinical and histological findings and histology, scleromyxoedema was diagnosed. We initiated therapy with 200 mg hydroxychloroquine administered 3 times daily, which led to waning of the erythema. However, the induration and coarseness of the skin increased. Therefore, we began therapy with IVIG( 2 g / kg body weight, every 4 weeks). This treatment rapidly improved the appearance of the skin, and an almost complete resolution was observed after 3 cycles of IVIG( Fig. 3). Remission is still ongoing under continuation of IVIG( 1.5 g / kg body weight, every 4 weeks). Further reductions in the dose led to recurrent disease.
DISCUSSION
Diagnosis of scleromyxoedema is based on a generalized papular and sclerodermoid eruption, monoclonal gammopathy, absence of a thyroid disorder, and the histological triad of mucinosis, fibrosis, and fibroblast proliferation( 3). Scleromyxoedema can mimic systemic scleroderma, with symptoms such as sclerodactyly, oesophageal dysmotility, and Raynaud’ s phenomenon.
Fig. 2. Alcian blue staining revealing prominent and diffuse mucinosis in the upper dermis with increased collagen deposition and irregularly arranged fibroblasts.
This is an open access article under the CC BY-NC license. www. medicaljournals. se / acta Journal Compilation © 2017 Acta Dermato-Venereologica. doi: 10.2340 / 00015555-2684 Acta Derm Venereol 2017; 97: 979 – 980