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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