Sarcoidosis presenting transepidermal elimination

ARTICLE

ejd.2011.1373

Auteur(s) : Kozo Yoneda¹ demitsu@omiya.jichi.ac.jp, Maki Kakurai², Naoka Umemoto², Kozo Nakai¹, Yasuo Kubota¹, Toshio Demitsu²

¹ Department of Dermatology, Kagawa University, 1750-1 Ikenobe Kitagun Mikicho, 761-0793 Kagawa Japan,

² Department of Dermatology, Jichi Medical University, Saitama Japan

The histological features of necrobiosis and also asteroid bodies are not rare in cutaneous sarcoidosis [1-3]. We present a case that histologically exhibited both necrobiotic granuloma with transepidermal elimination and non-caseating, epithelioid cell tubercles in the same specimen, which is considered usual in sarcoidosis.

A 33-year-old Japanese woman presented with several infiltrated erythematous plaques on her face. She had noted these skin lesions during the previous nine months. She had no medical history of diabetes mellitus. She denied any symptoms suggesting lung disease, such as dyspnea, dry cough, or chest pain. Physical examination demonstrated several infiltrated erythematous plaques with elevated borders and depressed centers on the bilateral cheeks. The lesions measured 1-2 cm in diameter (figure 1A). There were no ulcerative changes in the lesions. Serum angiotensin converting enzyme and lysozyme levels were within normal limits. Other hematological or biochemical analyses were almost normal, except for mild anemia. Radiological studies including chest X-ray, computed tomography, and systemic scintigraphy using 67Ga-citrate demonstrated bilateral hilar and mediastinal lymphadenopathy. Ophthalmological examination showed no evidence of sarcoidosis. A biopsy specimen taken from an infiltrated erythematous plaque included tissue from the center to the edge of the lesion. Histopathological examination exhibited remarkable necrobiosis surrounded by histiocytic granulomas in the upper dermis (figures 1B, C, D). Granulomas in the papillary dermis demonstrated transepidermal elimination; open transepidermal canals filled with necrobiotic connective tissue connecting to an underlying granuloma (figures 1C, D). Langhans type multinucleated gaint cells, some of which included asteroid bodies, were also seen in the upper and lower dermis (figure 1E). In addition, round-shaped, non-caseating, epithelioid cell granulomas with a moderate admixture of lymphoid cells were also detected in the deep dermis (figure 1F). There were no fungi, mycobacteria, or foreign bodies demonstrated by periodic acid-Schiff stain and elastica van Gieson stain. Mucin or lipids could not be seen. Topical tacrolimus therapy and corticosteroid tape resulted in an improvement of the skin lesions. Recently, Apalla et al. reported the existence of tumor-related sarcoidosis [4]. We have followed the patient very carefully for five years without observing any further abnormal enlargement of hilar or mediastinal lymph nodes. We think the possibility of coincidence of sarcoidosis and non-Hodgkin lymphoma is very low in our patient.

Cutaneous sarcoidosis sometimes presents unusual forms and transepidermal elimination in cutaneous sarcoidosis is rare although some cases have already been reported [1-3], but the clinical appearance of present case is interesting. Necrobiotic granulomas with transepithelial elimination were found in the upper dermis, whereas non-caseating, epithleioid cell tubercles were seen in the lower dermis. The former corresponds to the features of perforating granuloma annulare, and the latter is mostly suggestive of other granulomatous diseases including cutaneous sarcoidosis. We think there are two possible pathomechanisms that could account for unique pathophysiology.

One possibility is that our patient had cutaneous sarcoidosis accompanied by necrobiotic granuloma with transepidermal elimination. The histology of sarcoidosis is more diverse than previously recognized [1, 5]. Sarcoidosis may histologically present necrobiotic granuloma as well as epitheliod cell granuloma. Ball et al. reported that 43% cutaneous sarcoidosis demonstrated histological focal necrosis [1]. In their two cases, histologic features of cutaneous sarcoidosis resembled granuloma annulare or necrobiosis lipoidica. Kuramoto et al reported a case of subcutaneous sarcoidosis with extensive caseation necrosis, which reflects a regressing stage [6]. Cutaneous sarcoidal granuloma might evolve into granuloma annulare on histology. Another possibility is that our case could have had coexisting cutaneous sarcoidosis and granuloma annulare. Sarcoidosis may associate with granuloma annulare and necrobiosis lipoidica [2, 3]. However, the coexistence of granuloma annulare and cutaneous sarcoidosis in the same skin specimen has not been reported previously.

Disclosure

Acknowledgements: We are grateful to Hideto Yokokura, Ryuichi Azuma, Shoko Fujii, and Shin-ichiro Koyama for insightful discussions during the course of diagnosis and therapy. Financial support: none. Conflict of interest: none.

References

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