Atypical cutaneous sarcoidosis with diffuse, indurated erythema

ARTICLE

Auteur(s) : Yayoi Tada¹, Naoko Kanda², Takamitsu Ohnishi², Shinichi Watanabe²

¹Department of Dermatology, Faculty of Medicine, University of Tokyo, Hongo 7-3-1, Bunkyo-ku, 113-8655 Tokyo, Japan
²Teikyo University School of Medicine, Tokyo, Japan

A 74-year-old Japanese man presented with inflamed, fresh red, indurated erythema with local warmth and slight pruritus, extending diffusely from the clavicle to the upper back and the scapular region (figures 1A, B). One month after the first visit, a new skin lesion appeared in the lumbar region. One year previously, the patient had visited a hospital with the complaints of exertional dyspnea, chest pain and weight loss (10 kg in 2 months) and was diagnosed as having sarcoidosis following detailed examination at the Department of Pulmonary Medicine; he was thereafter under follow-up, without any medications. A plain X-ray and computed tomography of the chest at that time revealed diffuse granular opacities without apparent bilateral hilar and mediastinal lymphadenopathy. Flow-cytometric analysis of the bronchoalveolar lavage (BAL) cells revealed CD3+ cells 88.1%, CD4+ cells 49.8% and CD8+ cells 36.1%, and a transbronchial lung biopsy revealed the presence of granulomas. Neither ocular examination nor an electrocardiogram, Holter monitoring, or respiratory function testing revealed any abnormalities. At the time of the patient’s first visit to our hospital, laboratory examinations revealed a normal blood count and urinalysis, and increased serum levels of angiotensin-converting enzyme (24.3 U/mL >21 U/mL), lysozyme (21.8 μg/mL > 10 μg/mL), immunoglobulin G (2,120 mg/mL > 1,600 μg/mL) and C-reactive protein (3.13 mg/mL > 0.3 mg/mL). The serum levels of calcium, creatine phosphokinase and β-D-glucan, and also the blood sugar levels were within their respective normal ranges. Serum testing for antinuclear antibodies was negative. A skin biopsy was obtained from a lesion on the upper back. Histology revealed the formation of multiple epithelioid-type granulomas around the skin appendages and vessels, extending from the superficial to the deep dermis, surrounded by minimal lymphocytic infiltration, but no caseous necrosis, compatible with sarcoidosis (figure 1C). Furthermore, marked edema with dilated blood vessels was observed in the upper dermis, corresponding to the atypical clinical appearance of severe erythema and swelling in our patient. We diagnosed the eruption as skin lesions associated with systemic sarcoidosis. Topical application of difluprednate ointment for 9 months, followed by that of clobetasol propionate ointment for 1 month resulted in the disappearance of the eruption, with residual pigmentation.

According to the classification of cutaneous sarcoidosis, the eruption in our case corresponded best to the plaque form of the disease [1]. However, the diffuse distribution of the eruption from the clavicle to the upper back and the scapular region and the local warmth and induration in our case was unusual, and different from the typical clinical features of the plaque form. This unusual clinical manifestation is likely to be due to the involvement of the skin throughout the dermis over a wide area. With skin sarcoidosis, description of local warmth and edema similar to our case is found in subcutaneous sarcoidosis mimicking cellulites, which discloses extensive granulomatous panniculitis [2, 3] but is not reported in plaque form sarcoidosis, which suggests that the widespread, extensive inflammation seen in our case is unique. To the best of our knowledge, cases similar to ours have not yet been reported in the literature. We thus report our present patient as a case of atypical cutaneous sarcoidosis.

Acknowledgements

References

2 Falagas ME, Vergidis PI. Narrative review: diseases that masquerade as infectious cellulitis. Ann Intern Med 2005; 142: 47-55.

3 Papadavid E, Dalamaga M, Stavrianeas N, Papiris SA. Subcutaneous sarcoidosis masquerading as cellulitis. Dermatology 2008; 217: 212-4.