Correspondence Clinical Letter
Clinical letter Interstitial-type granuloma annulare associated with Sjögren syndrome
DOI: 10.1111/ddg.12263
Dear Editors, The association of granuloma annulare (GA) with autoimmune disease – especially autoimmune thyroid disease – have been reported; however, to the best of our knowledge, only one case of GA associated with Sjögren syndrome (SS) has been described [1]. Here we report a case of interstitial type GA associated with SS. A 53-year-old woman presented with a one-year history of annular skin lesions on the hands. She also had a three-year history of polyarthralgia of the knees and fingers, and had been treated with low-dose oral corticosteroids (prednisolone 1 mg/day). The rest of her history was unremarkable. Physical examination revealed annular red-brown skin lesions on the hands (Figure 1), which were neither painful nor pruritic. Laboratory tests revealed elevated values for erythrocyte sedimentation rate (26 mm/hr, normal 3–11); C-reactive protein (1.72 mg/ dl, normal ≤ 0.30); IgG (1 984 mg/dl, normal 870–1 700); IgA (475 mg/dl, normal 110–410); and IgM (252 mg/dl, normal 35–220). Serum was positive for antinuclear antibody (x320, speckled pattern), anti-Ro/SS-A antibodies (120.3, normal < 10), and anti-La/SS-B antibodies (102.3, normal < 10). The level of glycosylated hemoglobin A was normal, and other autoantibodies, including rheumatoid factor, anti-cyclic citrullinated peptide, anti-double-stranded DNA, anti-Sm, anti-RNP, anti-Jo-1, anti-Scl-70, and anti-cardiolipin, were absent. Schirmer test (right, 4 mm; left, 4 mm) and the tear break-up time (right, 3 sec; left, 3 sec) were positive. Sialography was also positive. Based on these findings, the patient was diagnosed with SS. The absence of bony erosions in radiographs, and the absence of rheumatoid factor and anti-cyclic citrullinated peptide suggested that her polyarthralgia was caused by SS. Skin biopsy revealed dermal interstitial CD68-positive histiocytes and lymphocytes infiltrated around deposits of alcian blue-positive mucin in the reticular dermis (Figure 2). Interstitial histiocytic infiltration was present throughout dermis; however, eosinophils and neutrophils were absent. These histopathological findings were consistent with interstitial-type GA. Topical corticosteroids (0.05 %
ifluprednate cream) produced improvement but complete d resolution was not achieved. A differential diagnosis in the present case is interstitial granulomatous dermatitis (IGD), a disorder described by Ackerman et al. in 1993 [2]. IGD is associated with rheumatoid arthritis and other autoimmune diseases and characterized by papules and plaques located on the trunk and extremities. Histopathological findings show an interstitial histiocytic infiltration that is localized primarily in the lower dermis. Eosinophils and neutrophils are often present, while mucin is typically absent. In contrast, interstitial GA is characterized by mucin associated with histiocytes. Although the presence of polyarthralgia initially suggested IGD in the present case, both the clinical appearance of the skin lesions and the histopathological findings were consistent with the diagnosis of GA. The pathomechanism of GA associated with autoimmune disease remains unknown. However, several reports support the possible association of GA and autoimmunity, including the hypothesis of the existence of a common immunological pathway for both conditions [3]. In addition, Paola et al. reported a case of GA, autoimmune thyroiditis, and lichen sclerosus in a woman. They suggested that GA, with lymphocyte-m ediated hypersensitivity type IV mechanism and a cell-mediated immune process, could help trigger an organ-specific autoimmune response [4]. The pathomechanism of IGD is also still unknown, but the association with rheumatoid arthritis and other autoimmune diseases suggests an immune complex-mediated mechanism [5]. As GA a ssociated with autoimmune disease and IGD share some similarities, the two disorders may belong to the same disease spectrum.
Figure 1 Annular red-brown papules on the hands.
© 2014 Deutsche Dermatologische Gesellschaft (DDG). Published by John Wiley & Sons Ltd. | JDDG | 1610-0379/2014/1205
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Correspondence Clinical Letter
Tomo Sakiyama, Ikuko Hirai, Akira Konohana, Yujiro Takae Division of Dermatology, Saiseikai Yokohama City Nanbu Hospital, Yokohama, Japan
Conflict of interest None. Correspondence to Tomo Sakiyama Division of Dermatology, Saiseikai Yokohama City Nanbu Hospital 3–2–10, Konandai Konan-ku, Yokohama-city Kanagawa 234–8503, Japan E-mail: [email protected]
References 1
2
3
4
5
Sumikawa Y, Ansai S, Kimura T et al. Interstitial type ranuloma annulare associated with Sjögren’s syndrome. g J Dermatol 2010; 37: 493–5. Ackerman AB, White WL, Guo Y et al. Clues to Diagnosis in Dermatopathology, Vol. 3. American Society of Clinical Pathologists Press, Chicago, 1993: 309–12. Vázquez-López F, Pereiro M Jr, Manjón Haces JA et al. Localized granuloma annulare and autoimmune thyroiditis in adult women: a case-control study. J Am Acad Dermatol 2003; 48: 517–20. Paola DC, Batsikosta A, Feci L et al. Granuloma annulare, autoimmune thyroiditis, lichen sclerosus in a woman: r andomness or significant association? Case Rep Dermatol Med 2013: 289084. Crowson AN, Magro C. Interstitial granulomatous dermatitis with arthritis. Hum Pathol 2004; 35: 779–80.
Figure 2 Biopsy shows dermal interstitial histiocytes and lymphocyte infiltration around the deposit of mucin in the reticular dermis (H&E; magnification ×40) (a). Alcian blue staining reveals mucin associated with histiocytes (magnification ×200) (b). Immunohistochemical staining for CD68 reveals interstitial infiltration of histiocytes (magnification ×200) (c).
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© 2014 Deutsche Dermatologische Gesellschaft (DDG). Published by John Wiley & Sons Ltd. | JDDG | 1610-0379/2014/1205
Clinical letter Interstitial-type granuloma annulare associated with Sjögren syndrome
DOI: 10.1111/ddg.12263
Dear Editors, The association of granuloma annulare (GA) with autoimmune disease – especially autoimmune thyroid disease – have been reported; however, to the best of our knowledge, only one case of GA associated with Sjögren syndrome (SS) has been described [1]. Here we report a case of interstitial type GA associated with SS. A 53-year-old woman presented with a one-year history of annular skin lesions on the hands. She also had a three-year history of polyarthralgia of the knees and fingers, and had been treated with low-dose oral corticosteroids (prednisolone 1 mg/day). The rest of her history was unremarkable. Physical examination revealed annular red-brown skin lesions on the hands (Figure 1), which were neither painful nor pruritic. Laboratory tests revealed elevated values for erythrocyte sedimentation rate (26 mm/hr, normal 3–11); C-reactive protein (1.72 mg/ dl, normal ≤ 0.30); IgG (1 984 mg/dl, normal 870–1 700); IgA (475 mg/dl, normal 110–410); and IgM (252 mg/dl, normal 35–220). Serum was positive for antinuclear antibody (x320, speckled pattern), anti-Ro/SS-A antibodies (120.3, normal < 10), and anti-La/SS-B antibodies (102.3, normal < 10). The level of glycosylated hemoglobin A was normal, and other autoantibodies, including rheumatoid factor, anti-cyclic citrullinated peptide, anti-double-stranded DNA, anti-Sm, anti-RNP, anti-Jo-1, anti-Scl-70, and anti-cardiolipin, were absent. Schirmer test (right, 4 mm; left, 4 mm) and the tear break-up time (right, 3 sec; left, 3 sec) were positive. Sialography was also positive. Based on these findings, the patient was diagnosed with SS. The absence of bony erosions in radiographs, and the absence of rheumatoid factor and anti-cyclic citrullinated peptide suggested that her polyarthralgia was caused by SS. Skin biopsy revealed dermal interstitial CD68-positive histiocytes and lymphocytes infiltrated around deposits of alcian blue-positive mucin in the reticular dermis (Figure 2). Interstitial histiocytic infiltration was present throughout dermis; however, eosinophils and neutrophils were absent. These histopathological findings were consistent with interstitial-type GA. Topical corticosteroids (0.05 %
ifluprednate cream) produced improvement but complete d resolution was not achieved. A differential diagnosis in the present case is interstitial granulomatous dermatitis (IGD), a disorder described by Ackerman et al. in 1993 [2]. IGD is associated with rheumatoid arthritis and other autoimmune diseases and characterized by papules and plaques located on the trunk and extremities. Histopathological findings show an interstitial histiocytic infiltration that is localized primarily in the lower dermis. Eosinophils and neutrophils are often present, while mucin is typically absent. In contrast, interstitial GA is characterized by mucin associated with histiocytes. Although the presence of polyarthralgia initially suggested IGD in the present case, both the clinical appearance of the skin lesions and the histopathological findings were consistent with the diagnosis of GA. The pathomechanism of GA associated with autoimmune disease remains unknown. However, several reports support the possible association of GA and autoimmunity, including the hypothesis of the existence of a common immunological pathway for both conditions [3]. In addition, Paola et al. reported a case of GA, autoimmune thyroiditis, and lichen sclerosus in a woman. They suggested that GA, with lymphocyte-m ediated hypersensitivity type IV mechanism and a cell-mediated immune process, could help trigger an organ-specific autoimmune response [4]. The pathomechanism of IGD is also still unknown, but the association with rheumatoid arthritis and other autoimmune diseases suggests an immune complex-mediated mechanism [5]. As GA a ssociated with autoimmune disease and IGD share some similarities, the two disorders may belong to the same disease spectrum.
Figure 1 Annular red-brown papules on the hands.
© 2014 Deutsche Dermatologische Gesellschaft (DDG). Published by John Wiley & Sons Ltd. | JDDG | 1610-0379/2014/1205
415
Correspondence Clinical Letter
Tomo Sakiyama, Ikuko Hirai, Akira Konohana, Yujiro Takae Division of Dermatology, Saiseikai Yokohama City Nanbu Hospital, Yokohama, Japan
Conflict of interest None. Correspondence to Tomo Sakiyama Division of Dermatology, Saiseikai Yokohama City Nanbu Hospital 3–2–10, Konandai Konan-ku, Yokohama-city Kanagawa 234–8503, Japan E-mail: [email protected]
References 1
2
3
4
5
Sumikawa Y, Ansai S, Kimura T et al. Interstitial type ranuloma annulare associated with Sjögren’s syndrome. g J Dermatol 2010; 37: 493–5. Ackerman AB, White WL, Guo Y et al. Clues to Diagnosis in Dermatopathology, Vol. 3. American Society of Clinical Pathologists Press, Chicago, 1993: 309–12. Vázquez-López F, Pereiro M Jr, Manjón Haces JA et al. Localized granuloma annulare and autoimmune thyroiditis in adult women: a case-control study. J Am Acad Dermatol 2003; 48: 517–20. Paola DC, Batsikosta A, Feci L et al. Granuloma annulare, autoimmune thyroiditis, lichen sclerosus in a woman: r andomness or significant association? Case Rep Dermatol Med 2013: 289084. Crowson AN, Magro C. Interstitial granulomatous dermatitis with arthritis. Hum Pathol 2004; 35: 779–80.
Figure 2 Biopsy shows dermal interstitial histiocytes and lymphocyte infiltration around the deposit of mucin in the reticular dermis (H&E; magnification ×40) (a). Alcian blue staining reveals mucin associated with histiocytes (magnification ×200) (b). Immunohistochemical staining for CD68 reveals interstitial infiltration of histiocytes (magnification ×200) (c).
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© 2014 Deutsche Dermatologische Gesellschaft (DDG). Published by John Wiley & Sons Ltd. | JDDG | 1610-0379/2014/1205
