Periorbital edema Teresa Jaeger1,2*, MD, Vagkan Mourantchanian1*, MD, Jan Gutermuth3, MD, R€ udiger Hein1, MD, Christian Andres1, MD, Johannes Ring1, MD, PhD, and Knut Brockow1, MD
1 Department of Dermatology and Allergy €t Biederstein, Technische Universita €nchen, 2Center of Allergy and Mu Environment (ZAUM), Technische € t Mu €nchen and Helmholtz Center, Universita Munich, Germany, and 3Department of Dermatology, Vrije Universiteit Brussel (VUB), Brussels, Belgium
Correspondence Teresa Jaeger, MD Department of Dermatology and Allergy Biederstein €t Mu €nchen Technische Universita Biedersteiner Str. 29 80802 Munich, Germany E-mail: [email protected]
What is your diagnosis? (b)
Figure 2 Urticarial macules on the d ecollete (a) and back (b)
Conflicts of interest: None. *These authors contributed equally to the clinicopathological case.
Patient’s History A 48-year-old Caucasian man presented with a 4-month history of swelling of the eyelids (Fig. 1). Furthermore, he had a non-fibrosing alopecia, Hashimoto thyroiditis, and hepatitis C. There was no evidence of a cava superior syndrome or malignant disease from computed tomography.
Figure 3 Histological examination (hematoxylin and eosin 9100, inset 9600) showed orthokeratosis, mucinous dermis, and vacuolar degeneration of the basal cell layer. Perivascular and periadnexial lymphohistiocytic infiltrate in the upper and deeper dermis was found
A few months later the patient developed urticarial macules on the back and neck (Fig. 2) as well as oral ulcerations. Histopathology
Figure 1 Four-month swelling of the eyelids ª 2014 The International Society of Dermatology
Histological examination was performed (upper back), which showed orthokeratosis, mucinous dermis, and a vacuolar degeneration of the basal cell layer. A perivascular and periadnexial lymphohistiocytic infiltrate in the upper and deeper dermis was found (Fig. 3). Direct immunofluorescence was negative. International Journal of Dermatology 2014, 53, 937–939
Jaeger et al.
Unclear periorbital edema and swelling of the eyelids
In addition to acute urticaria with angioedema and vena cava superior syndrome, clinical differential diagnoses now included lupus erythematosus or dermatomyositis. Laboratory findings
Following the diagnostic investigations, our patient showed seven of the 17 newly defined criteria of systemic lupus erythematosus (Table 1)1: clinical parameters (acute cutaneous lupus erythematosus, non-fibrosing alopecia, oral ulceration, anemia, leukocytopenia, thrombocytopenia; Table 2), and immunological parameters (elevated antinuclear antibodies 1:160 (normal: