Case report
Nodular cutaneous mucinosis associated with systemic lupus erythematosus Laila Elkeeb, MD, Kerith E. Spicknall, MD, and Diya F. Mutasim, MD
Department of Dermatology, University of Cincinnati College of Medicine, Cincinnati, OH, USA Correspondence Diya F. Mutasim, MD Department of Dermatology University of Cincinnati 231 Albert Sabin Way, ML 0592 Cincinnati OH 45267-0592 USA E-mail: [email protected] Conflicts of interest: None.
Case report A 71-year-old, African-American woman with systemic lupus erythematosus (SLE) presented with a 2-month history of asymptomatic nodules on her chest and both upper arms. Her SLE manifestations included oral ulcers, pancytopenia, arthritis, chronic kidney disease, fatigue, alopecia, and Raynaud’s phenomenon. Her past medical history included aortic and mitral regurgitation. Laboratory findings were positive for antinuclear, anti-Smith, anti-double-stranded DNA, and anti-ribonucleoprotein antibodies, hypocomplementemia, elevated creatinine, and proteinuria. On clinical examination, multiple skincolored, soft, smooth papules and papulonodules measuring 4–6 mm in diameter were apparent on her chest and proximal upper arms bilaterally (Fig. 1). Histopathological examination of two biopsy specimens revealed similar features. The epidermis was unremarkable. There was diffuse mucin deposition throughout the superficial and mid-reticular dermis and perieccrine fat. There was a mild perifollicular and focally perivascular infiltrate within the superficial and mid-reticular dermis (Figs. 2 and 3). These findings are characteristic of nodular cutaneous mucinosis (NCM). The patient was being treated for SLE with hydroxychloroquine and methotrexate. Four weeks after diagnosis, the lesions had spontaneously resolved.
(a)
(b) Figure 1 (a, b) Clinical photographs showing multiple, soft, dermal nodules on the patient’s chest 1389
ª 2014 The International Society of Dermatology
International Journal of Dermatology 2014, 53, 1389–1391
1390
Case report
Nodular cutaneous mucinosis
Elkeeb, Spicknall and Mutasim
Figure 2 Histology shows an unremarkable epidermis and moderate, diffuse mucin deposition in the mid and deep reticular dermis. (Hematoxylin and eosin stain; original magnification 940)
Discussion In 1954, Gold1 reported NCM in association with SLE. Since then, several additional case reports have confirmed the association.2–5 Nodular cutaneous mucinosis usually presents as multiple asymptomatic, smooth, dermal nodules favoring the trunk and extremities. It is sometimes the initial clue to the diagnosis of SLE as patients may present with these lesions many years prior to the development of other manifestations of SLE.2,6,7 Nodular cutaneous mucinosis may wax and wane with SLE activity and according to circulating antibody titers. Lesions may worsen with increased ultraviolet light exposure.8 Approximately 79% of patients with NCM have been found to have SLE, primarily as renal and articular disease.5 In NCM, lesions reveal mucin in the upper and midreticular dermis, sometimes extending to the deep dermis and subcutaneous fat. There is a slight to moderate perivascular lymphocytic infiltrate. The epidermal changes of lupus are usually absent. Direct immunofluorescence may reveal linear or granular deposits of immunoglobulins (IgG and IgM) and C3 at the dermoepidermal junction.9 In some cases, NCM lesions may remit spontaneously. Occasionally, intralesional hyaluronidase has been used to clear NCM lesions.10 However, treatments that are used for lupus erythematosus have also been used for NCM with variable results. These include antimalarials, and systemic and intralesional steroids.11 References 1 Gold SC. An unusual papular eruption associated with lupus erythematosus. Br J Dermatol 1954; 66: 429–433. 2 De Graciansky P, Hewitt J, Boulle S, et al. Eruption papuleuse du dos au cours dun lupus érythémateux: International Journal of Dermatology 2014, 53, 1389–1391
(a)
(b) Figure 3 Histology shows moderate, diffuse mucin deposition in the reticular dermis and perieccrine fat. [H&E stain; (a) 9100, (b) 9200]
altération de la substance fondamentale. Ann Dermatol Syphiligr (Paris) 1956; 83: 636–644. 3 Gammon WR, Caro I, Long JC, et al. Secondary cutaneous mucinosis with systemic lupus erythematosus. Arch Dermatol 1978; 114: 432–435. 4 Lamberts RJ, Rapids G, Lynch PJ. Nodular cutaneous mucinosis associated with lupus erythematosus. Cutis 1981; 28: 294–299. 5 Ronglioletti F, Parodi A, Rebora A. Papular and nodular mucinosis as a sign of lupus erythematosus. Dermatologica 1990; 180: 221–223. ª 2014 The International Society of Dermatology
Elkeeb, Spicknall and Mutasim
6 Weigand DA, Walter HC, Gregg LJ. Dermal mucinosis in discoid lupus erythematosus. Arch Dermatol 1981; 117: 735–738. 7 Moulin G, Bouchet B, Souteyrand P, et al. Mucinose papuleuse et lupus érythémateux dissémine. Ann Dermatol Venereol 1980; 107: 1193–1198. 8 Revier J, Kienzler J-L, Blanc D, et al. Mucinose papuleuse et lupus erythemateux. Ann Dermatol Venereol 1982; 109: 331–338.
ª 2014 The International Society of Dermatology
Nodular cutaneous mucinosis
Case report
9 Nishimoto M, Takaiwa T, Kodama H, et al. Cutaneous mucinosis associated with SLE, a case provoked by PUVA. J Dermatol (Tokyo) 1989; 16: 374–378. 10 Maruyama M, Miyauchi S, Hashimoto K. Massive cutaneous mucinosis associated with systemic lupus erythematosus. Br J Dermatol 1997; 137: 450–453. 11 Rongioletti F, Rebora A. Papular and nodular mucinosis associated with lupus erythematosus. Br J Dermatol 1986; 115: 631–636.
International Journal of Dermatology 2014, 53, 1389–1391
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Nodular cutaneous mucinosis associated with systemic lupus erythematosus Laila Elkeeb, MD, Kerith E. Spicknall, MD, and Diya F. Mutasim, MD
Department of Dermatology, University of Cincinnati College of Medicine, Cincinnati, OH, USA Correspondence Diya F. Mutasim, MD Department of Dermatology University of Cincinnati 231 Albert Sabin Way, ML 0592 Cincinnati OH 45267-0592 USA E-mail: [email protected] Conflicts of interest: None.
Case report A 71-year-old, African-American woman with systemic lupus erythematosus (SLE) presented with a 2-month history of asymptomatic nodules on her chest and both upper arms. Her SLE manifestations included oral ulcers, pancytopenia, arthritis, chronic kidney disease, fatigue, alopecia, and Raynaud’s phenomenon. Her past medical history included aortic and mitral regurgitation. Laboratory findings were positive for antinuclear, anti-Smith, anti-double-stranded DNA, and anti-ribonucleoprotein antibodies, hypocomplementemia, elevated creatinine, and proteinuria. On clinical examination, multiple skincolored, soft, smooth papules and papulonodules measuring 4–6 mm in diameter were apparent on her chest and proximal upper arms bilaterally (Fig. 1). Histopathological examination of two biopsy specimens revealed similar features. The epidermis was unremarkable. There was diffuse mucin deposition throughout the superficial and mid-reticular dermis and perieccrine fat. There was a mild perifollicular and focally perivascular infiltrate within the superficial and mid-reticular dermis (Figs. 2 and 3). These findings are characteristic of nodular cutaneous mucinosis (NCM). The patient was being treated for SLE with hydroxychloroquine and methotrexate. Four weeks after diagnosis, the lesions had spontaneously resolved.
(a)
(b) Figure 1 (a, b) Clinical photographs showing multiple, soft, dermal nodules on the patient’s chest 1389
ª 2014 The International Society of Dermatology
International Journal of Dermatology 2014, 53, 1389–1391
1390
Case report
Nodular cutaneous mucinosis
Elkeeb, Spicknall and Mutasim
Figure 2 Histology shows an unremarkable epidermis and moderate, diffuse mucin deposition in the mid and deep reticular dermis. (Hematoxylin and eosin stain; original magnification 940)
Discussion In 1954, Gold1 reported NCM in association with SLE. Since then, several additional case reports have confirmed the association.2–5 Nodular cutaneous mucinosis usually presents as multiple asymptomatic, smooth, dermal nodules favoring the trunk and extremities. It is sometimes the initial clue to the diagnosis of SLE as patients may present with these lesions many years prior to the development of other manifestations of SLE.2,6,7 Nodular cutaneous mucinosis may wax and wane with SLE activity and according to circulating antibody titers. Lesions may worsen with increased ultraviolet light exposure.8 Approximately 79% of patients with NCM have been found to have SLE, primarily as renal and articular disease.5 In NCM, lesions reveal mucin in the upper and midreticular dermis, sometimes extending to the deep dermis and subcutaneous fat. There is a slight to moderate perivascular lymphocytic infiltrate. The epidermal changes of lupus are usually absent. Direct immunofluorescence may reveal linear or granular deposits of immunoglobulins (IgG and IgM) and C3 at the dermoepidermal junction.9 In some cases, NCM lesions may remit spontaneously. Occasionally, intralesional hyaluronidase has been used to clear NCM lesions.10 However, treatments that are used for lupus erythematosus have also been used for NCM with variable results. These include antimalarials, and systemic and intralesional steroids.11 References 1 Gold SC. An unusual papular eruption associated with lupus erythematosus. Br J Dermatol 1954; 66: 429–433. 2 De Graciansky P, Hewitt J, Boulle S, et al. Eruption papuleuse du dos au cours dun lupus érythémateux: International Journal of Dermatology 2014, 53, 1389–1391
(a)
(b) Figure 3 Histology shows moderate, diffuse mucin deposition in the reticular dermis and perieccrine fat. [H&E stain; (a) 9100, (b) 9200]
altération de la substance fondamentale. Ann Dermatol Syphiligr (Paris) 1956; 83: 636–644. 3 Gammon WR, Caro I, Long JC, et al. Secondary cutaneous mucinosis with systemic lupus erythematosus. Arch Dermatol 1978; 114: 432–435. 4 Lamberts RJ, Rapids G, Lynch PJ. Nodular cutaneous mucinosis associated with lupus erythematosus. Cutis 1981; 28: 294–299. 5 Ronglioletti F, Parodi A, Rebora A. Papular and nodular mucinosis as a sign of lupus erythematosus. Dermatologica 1990; 180: 221–223. ª 2014 The International Society of Dermatology
Elkeeb, Spicknall and Mutasim
6 Weigand DA, Walter HC, Gregg LJ. Dermal mucinosis in discoid lupus erythematosus. Arch Dermatol 1981; 117: 735–738. 7 Moulin G, Bouchet B, Souteyrand P, et al. Mucinose papuleuse et lupus érythémateux dissémine. Ann Dermatol Venereol 1980; 107: 1193–1198. 8 Revier J, Kienzler J-L, Blanc D, et al. Mucinose papuleuse et lupus erythemateux. Ann Dermatol Venereol 1982; 109: 331–338.
ª 2014 The International Society of Dermatology
Nodular cutaneous mucinosis
Case report
9 Nishimoto M, Takaiwa T, Kodama H, et al. Cutaneous mucinosis associated with SLE, a case provoked by PUVA. J Dermatol (Tokyo) 1989; 16: 374–378. 10 Maruyama M, Miyauchi S, Hashimoto K. Massive cutaneous mucinosis associated with systemic lupus erythematosus. Br J Dermatol 1997; 137: 450–453. 11 Rongioletti F, Rebora A. Papular and nodular mucinosis associated with lupus erythematosus. Br J Dermatol 1986; 115: 631–636.
International Journal of Dermatology 2014, 53, 1389–1391
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