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Jessner’s lymphocytic infiltration of the skin: A new case
Journal of the Saudi Society of Dermatology & Dermatologic Surgery (2012) 16, 77–79
King Saud University
Journal of the Saudi Society of Dermatology & Dermatologic Surgery www.ksu.edu.sa www.jssdds.org www.sciencedirect.com
CASE REPORT
Jessner’s lymphocytic infiltration of the skin: A new case Besma Ben Dhaou a,b,*, Fatma Ben Dahmen c, Amira Ben Amor c, Imen Ben Ahmed c, Farid Marzouki c, Fatma Boussema a,b, Lilia Rokbani a b c
a,b
Internal Medicine Department, Habib Thameur Hospital, Tunis, Tunisia Faculty of Medicine, University of Tunis, ElManar, Tunis, Tunisia Internal Medicine Department, Mohamed Tahar Maamouri Hospital, Nabeul, Tunisia
Received 13 May 2012; accepted 25 May 2012 Available online 18 June 2012
KEYWORDS Jessner’s lymphocytic infiltration of the skin; Antimalarial treatment
Abstract Jessner’s lymphocytic infiltration of the skin is a chronic, benign T-cell infiltrative disorder, usually manifesting as erythematous papules or plaques on the face, neck and back. We report the case of a 47-year-old woman, who presented multiple erythematous infiltrated papules and plaques interesting the face, the forearms and hands. Skin biopsy confirmed the diagnosis of Jessner’s lymphocytic infiltration of the skin. The evolution was favorable with antimalarial treatment. ª 2012 King Saud University. Production and hosting by Elsevier B.V. All rights reserved.
1. Introduction
2. Case report
Jessner’s lymphocytic infiltration of the skin (JLIS) is a skin condition of unknown etiology characterized by erythematosus papules and plaques located on the head, neck and upper back. The eruption resolves spontaneously after months or a few years but can recur for several years. A variety of empirical treatments has been tried with limited success. We report a new case of JLIS.
A 47-year-old woman had been, in March 2011, an erythematous papules and plaques photosensitive. The lesions were asymptomatic, without burning or pruritus, but cosmetically disfiguring. Skin examination revealed multiple erythematous infiltrated papules and plaques with no epidermal changes interesting the face (forehead, cheeks, bridge of the nose); the forearms and hands (Figs. 1 and 2). The rest of physical examination was normal. Blood cell count showed leucopenia at 3900 mm 3 and lymphopenia at 1300 mm 3, hemoglobin and platelet were normal. Renal, liver and thyroid function, borrelia serology, screening for antinuclear antibody, antidsDNA antibody and antibodies to Ro and La were negative or normal. Chest radiography, abdominal ultrasound and the echocardiogram were normal. Skin biopsy showed a dense perivascular and periadnexal lymphocytic infiltration in the reticular dermis evoking Jessner’s lymphocytic infiltration of the skin (JLIS) (Figs. 3 and 4). Direct immunofluorescence was negative. The diagnosis was a JLIS. We started treatment with hydroxychloroquine 250 mg daily associated with sun
* Corresponding author at: Internal Medicine Department, Habib Thameur Hospital, Tunis, Tunisia. Tel.: +216 24144955. E-mail address: [email protected] (B.B. Dhaou). Peer review under responsibility of King Saud University.
Production and hosting by Elsevier
2210-836X ª 2012 King Saud University. Production and hosting by Elsevier B.V. All rights reserved. http://dx.doi.org/10.1016/j.jssdds.2012.05.002
78
Figure 1 Erythematous infiltrated papules and plaques interesting the face.
Figure 2 Erythematous infiltrated papules and plaques interesting the face.
Figure 3 Dense perivascular and periadnexal lymphocytic infiltration in the reticular dermis.
protection creams. The evolution was favorable. The current decline was one year.
B.B. Dhaou et al.
Figure 4 Dense perivascular and periadnexal lymphocytic infiltration in the reticular dermis.
The incidence of JLIS is unknown, but it is considered uncommon. It mostly affects middle-aged adults, with equal incidence in men and women, and very rarely occurs in children (Lipsker et al., 2006; Toonstra et al., 1989). Familial occurrence has been described in the literature (Dippel et al., 2002; Ashworth and Morley, 1988). JLIS is characterized by single or multiple erythematous papules or plaques and, less commonly, nodules, typically localized on the face, neck, chest, arms and upper back (Toonstra et al., 1989). Sometimes an arciform configuration with central clearing is seen. Usually the lesions are asymptomatic, but they can be itching or burning. The relationship to sun exposure is variable and there is no regional variation in incidence (Toonstra et al., 1989). Whether JLIS is a separate entity, or belongs to the disease spectrum of cutaneous lupus erythematosus or polymorphous light eruption is still a matter of debate, since clinical and histopathological features may overlap in particular with lupus erythematosus tumidus. Histopathologically JLIS is characterized by a superficial and deep, primarily perivascular, sleeve-like lymphocytic dermal infiltrate with a predominance of small mature polyclonal lymphocytes and without epidermal involvement (Lipsker et al., 2006; Poenitz et al., 2003). Deposits of mucin in the reticular dermis have been described (Re´my-Leroux et al., 2008). However, other authors have been unable to demonstrate mucin in JLIS (Lipsker et al., 2006; Teixeira et al., 2006). A variety of treatments has been tried, with variable, and often limited, success, including topical, intralesional or systemic corticosteroids, antimalarials, thalidomide, tetracyclines, cryotherapy and photoprotection. A few cases of successful treatment with dapsone, auranofin, methotrexate, chemotherapy and pulsed dye laser have been reported (Ardavanis et al., 2008; Rai and Balachandran, 2006; Hafejee et al., 2004; Laurinaviciene et al., 2009; Michel and Perrin, 2010). Treatment with antimalarials is usually effective in cases with photo-sensitivity (Adamski et al., 2002). In our case, the evolution was favorable with antimalarials. The current decline was one year.
3. Discussion 4. Conclusion JLIS, described by (Jessner and Kanof, 1953), is known as a benign chronic T-cell infiltrative disorder with lesions persisting for several months or years. Spontaneous remission may be seen, but JLIS has a tendency to relapse.
JLIS is a rare and benign disorder. Its clinical course is cyclic with remission and exacerbations. Treatment has been of limited success and purely on the basis of anecdotal reports.
Jessner’s lymphocytic infiltration of the skin: A new case References Adamski, H., Labrousse, A.L., Sparsa, A., Leonard, F., Le Gall, F., Labrousse, F., Ollivier, I., Bonnetblanc, J.M., Chevrant-Breton, J., Bedane, C., Bernard, P., 2002. Photo-de´clenchement des le´sions dans la maladie de Jessner-Kanof. Ann. Dermatol. Venereol. 129, 1370–1373. Ardavanis, A., Orphanos, G., Skafida, S., Basioukas, S., Rigatos, G., 2008. Coincidential successful treatment of Jessner–Kanof disease with chemotherapy. Ann. Oncol. 19, 1360–1361. Ashworth, J., Morley, W.N., 1988. Jessner and Kanof’s lymphocytic infiltration of the skin: a familial variant. Dermatologica 177, 120– 122. Dippel, E., Poenitz, N., Klemke, C.D., Orfanos, C.E., Goerdt, S., 2002. Familial lymphocytic infiltration of the skin: histochemical and molecular analysis in three brothers. Dermatology 204, 12– 16. Hafejee, A., Winhoven, S., Coulson, I.H., 2004. Jessner’s lymphocytic infiltrate responding to oral auranofin. J. Dermatolog. Treat. 15, 331–332. Jessner, M., Kanof, B., 1953. Lymphocytic infiltration of the skin. Arch. Dermatol. 68, 447–449. Laurinaviciene, R., Clemmensen, O., Bygum, A., 2009. Successful treatment of Jessner’s lymphocytic infiltration of the skin with methotrexate. Acta Derm. Venereol. 89, 542–543.
79 Lipsker, D., Mitschler, A., Grosshans, E., Cribier, B., 2006. Could Jessner’s lymphocytic infiltrate of the skin be a dermal variant of lupus erythematosus? An analysis of 210 cases. Dermatology 213, 15–22. Michel, J.L., Perrin, D., 2010. Infiltrat lymphocytaire de Jessner et Kanof traite´ par laser a` colorant pulse´. Ann. Dermatol. Venereol. 137, 803–807. Poenitz, N., Dippel, E., Klemke, C.D., Qadoumi, M., Goerdt, S., 2003. Jessner’s lymphocytic infiltration of the skin: a CD8+ polyclonal reactive skin condition. Dermatology 207, 276–284. Rai, V.M., Balachandran, C., 2006. Multiple infiltrated nodules and plaques. Lymphocytic infiltrate of Jessner. Dermatol. Online J. 12, 26. Re´my-Leroux, V., Le´onard, F., Lambert, D., Wechsler, J., Cribier, B., Thomas, P., et al., 2008. Comparison of histopathologic–clinical characteristics of Jessner’s lymphocytic infiltration of the skin and lupus erythematosus tumidus: multicenter study of 46 cases. J. Am. Acad. Dermatol. 58, 217–223. Teixeira, M., Ferreira, M., Alves, R., Selores, M., 2006. Lupus erythematosus tumidus: an underestimated entity. Lupus 15, 296– 300. Toonstra, J., Wildschut, A., Boer, J., Smeenk, G., Willemze, R., van der Putte, S.C., et al., 1989. Jessner’s lymphocytic infiltration of the skin. A clinical study of 100 patients. Arch. Dermatol. 125, 1525– 1530.
King Saud University
Journal of the Saudi Society of Dermatology & Dermatologic Surgery www.ksu.edu.sa www.jssdds.org www.sciencedirect.com
CASE REPORT
Jessner’s lymphocytic infiltration of the skin: A new case Besma Ben Dhaou a,b,*, Fatma Ben Dahmen c, Amira Ben Amor c, Imen Ben Ahmed c, Farid Marzouki c, Fatma Boussema a,b, Lilia Rokbani a b c
a,b
Internal Medicine Department, Habib Thameur Hospital, Tunis, Tunisia Faculty of Medicine, University of Tunis, ElManar, Tunis, Tunisia Internal Medicine Department, Mohamed Tahar Maamouri Hospital, Nabeul, Tunisia
Received 13 May 2012; accepted 25 May 2012 Available online 18 June 2012
KEYWORDS Jessner’s lymphocytic infiltration of the skin; Antimalarial treatment
Abstract Jessner’s lymphocytic infiltration of the skin is a chronic, benign T-cell infiltrative disorder, usually manifesting as erythematous papules or plaques on the face, neck and back. We report the case of a 47-year-old woman, who presented multiple erythematous infiltrated papules and plaques interesting the face, the forearms and hands. Skin biopsy confirmed the diagnosis of Jessner’s lymphocytic infiltration of the skin. The evolution was favorable with antimalarial treatment. ª 2012 King Saud University. Production and hosting by Elsevier B.V. All rights reserved.
1. Introduction
2. Case report
Jessner’s lymphocytic infiltration of the skin (JLIS) is a skin condition of unknown etiology characterized by erythematosus papules and plaques located on the head, neck and upper back. The eruption resolves spontaneously after months or a few years but can recur for several years. A variety of empirical treatments has been tried with limited success. We report a new case of JLIS.
A 47-year-old woman had been, in March 2011, an erythematous papules and plaques photosensitive. The lesions were asymptomatic, without burning or pruritus, but cosmetically disfiguring. Skin examination revealed multiple erythematous infiltrated papules and plaques with no epidermal changes interesting the face (forehead, cheeks, bridge of the nose); the forearms and hands (Figs. 1 and 2). The rest of physical examination was normal. Blood cell count showed leucopenia at 3900 mm 3 and lymphopenia at 1300 mm 3, hemoglobin and platelet were normal. Renal, liver and thyroid function, borrelia serology, screening for antinuclear antibody, antidsDNA antibody and antibodies to Ro and La were negative or normal. Chest radiography, abdominal ultrasound and the echocardiogram were normal. Skin biopsy showed a dense perivascular and periadnexal lymphocytic infiltration in the reticular dermis evoking Jessner’s lymphocytic infiltration of the skin (JLIS) (Figs. 3 and 4). Direct immunofluorescence was negative. The diagnosis was a JLIS. We started treatment with hydroxychloroquine 250 mg daily associated with sun
* Corresponding author at: Internal Medicine Department, Habib Thameur Hospital, Tunis, Tunisia. Tel.: +216 24144955. E-mail address: [email protected] (B.B. Dhaou). Peer review under responsibility of King Saud University.
Production and hosting by Elsevier
2210-836X ª 2012 King Saud University. Production and hosting by Elsevier B.V. All rights reserved. http://dx.doi.org/10.1016/j.jssdds.2012.05.002
78
Figure 1 Erythematous infiltrated papules and plaques interesting the face.
Figure 2 Erythematous infiltrated papules and plaques interesting the face.
Figure 3 Dense perivascular and periadnexal lymphocytic infiltration in the reticular dermis.
protection creams. The evolution was favorable. The current decline was one year.
B.B. Dhaou et al.
Figure 4 Dense perivascular and periadnexal lymphocytic infiltration in the reticular dermis.
The incidence of JLIS is unknown, but it is considered uncommon. It mostly affects middle-aged adults, with equal incidence in men and women, and very rarely occurs in children (Lipsker et al., 2006; Toonstra et al., 1989). Familial occurrence has been described in the literature (Dippel et al., 2002; Ashworth and Morley, 1988). JLIS is characterized by single or multiple erythematous papules or plaques and, less commonly, nodules, typically localized on the face, neck, chest, arms and upper back (Toonstra et al., 1989). Sometimes an arciform configuration with central clearing is seen. Usually the lesions are asymptomatic, but they can be itching or burning. The relationship to sun exposure is variable and there is no regional variation in incidence (Toonstra et al., 1989). Whether JLIS is a separate entity, or belongs to the disease spectrum of cutaneous lupus erythematosus or polymorphous light eruption is still a matter of debate, since clinical and histopathological features may overlap in particular with lupus erythematosus tumidus. Histopathologically JLIS is characterized by a superficial and deep, primarily perivascular, sleeve-like lymphocytic dermal infiltrate with a predominance of small mature polyclonal lymphocytes and without epidermal involvement (Lipsker et al., 2006; Poenitz et al., 2003). Deposits of mucin in the reticular dermis have been described (Re´my-Leroux et al., 2008). However, other authors have been unable to demonstrate mucin in JLIS (Lipsker et al., 2006; Teixeira et al., 2006). A variety of treatments has been tried, with variable, and often limited, success, including topical, intralesional or systemic corticosteroids, antimalarials, thalidomide, tetracyclines, cryotherapy and photoprotection. A few cases of successful treatment with dapsone, auranofin, methotrexate, chemotherapy and pulsed dye laser have been reported (Ardavanis et al., 2008; Rai and Balachandran, 2006; Hafejee et al., 2004; Laurinaviciene et al., 2009; Michel and Perrin, 2010). Treatment with antimalarials is usually effective in cases with photo-sensitivity (Adamski et al., 2002). In our case, the evolution was favorable with antimalarials. The current decline was one year.
3. Discussion 4. Conclusion JLIS, described by (Jessner and Kanof, 1953), is known as a benign chronic T-cell infiltrative disorder with lesions persisting for several months or years. Spontaneous remission may be seen, but JLIS has a tendency to relapse.
JLIS is a rare and benign disorder. Its clinical course is cyclic with remission and exacerbations. Treatment has been of limited success and purely on the basis of anecdotal reports.
Jessner’s lymphocytic infiltration of the skin: A new case References Adamski, H., Labrousse, A.L., Sparsa, A., Leonard, F., Le Gall, F., Labrousse, F., Ollivier, I., Bonnetblanc, J.M., Chevrant-Breton, J., Bedane, C., Bernard, P., 2002. Photo-de´clenchement des le´sions dans la maladie de Jessner-Kanof. Ann. Dermatol. Venereol. 129, 1370–1373. Ardavanis, A., Orphanos, G., Skafida, S., Basioukas, S., Rigatos, G., 2008. Coincidential successful treatment of Jessner–Kanof disease with chemotherapy. Ann. Oncol. 19, 1360–1361. Ashworth, J., Morley, W.N., 1988. Jessner and Kanof’s lymphocytic infiltration of the skin: a familial variant. Dermatologica 177, 120– 122. Dippel, E., Poenitz, N., Klemke, C.D., Orfanos, C.E., Goerdt, S., 2002. Familial lymphocytic infiltration of the skin: histochemical and molecular analysis in three brothers. Dermatology 204, 12– 16. Hafejee, A., Winhoven, S., Coulson, I.H., 2004. Jessner’s lymphocytic infiltrate responding to oral auranofin. J. Dermatolog. Treat. 15, 331–332. Jessner, M., Kanof, B., 1953. Lymphocytic infiltration of the skin. Arch. Dermatol. 68, 447–449. Laurinaviciene, R., Clemmensen, O., Bygum, A., 2009. Successful treatment of Jessner’s lymphocytic infiltration of the skin with methotrexate. Acta Derm. Venereol. 89, 542–543.
79 Lipsker, D., Mitschler, A., Grosshans, E., Cribier, B., 2006. Could Jessner’s lymphocytic infiltrate of the skin be a dermal variant of lupus erythematosus? An analysis of 210 cases. Dermatology 213, 15–22. Michel, J.L., Perrin, D., 2010. Infiltrat lymphocytaire de Jessner et Kanof traite´ par laser a` colorant pulse´. Ann. Dermatol. Venereol. 137, 803–807. Poenitz, N., Dippel, E., Klemke, C.D., Qadoumi, M., Goerdt, S., 2003. Jessner’s lymphocytic infiltration of the skin: a CD8+ polyclonal reactive skin condition. Dermatology 207, 276–284. Rai, V.M., Balachandran, C., 2006. Multiple infiltrated nodules and plaques. Lymphocytic infiltrate of Jessner. Dermatol. Online J. 12, 26. Re´my-Leroux, V., Le´onard, F., Lambert, D., Wechsler, J., Cribier, B., Thomas, P., et al., 2008. Comparison of histopathologic–clinical characteristics of Jessner’s lymphocytic infiltration of the skin and lupus erythematosus tumidus: multicenter study of 46 cases. J. Am. Acad. Dermatol. 58, 217–223. Teixeira, M., Ferreira, M., Alves, R., Selores, M., 2006. Lupus erythematosus tumidus: an underestimated entity. Lupus 15, 296– 300. Toonstra, J., Wildschut, A., Boer, J., Smeenk, G., Willemze, R., van der Putte, S.C., et al., 1989. Jessner’s lymphocytic infiltration of the skin. A clinical study of 100 patients. Arch. Dermatol. 125, 1525– 1530.