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44-Year-Old Male with Asymptomatic, Erythematous Lesion on Left Flank

Can you diagnose this case?

Alexander K. C. Leung, MD, and Benjamin Barankin, MD – 2016

Signs and Symptoms

A 44-year-old man presented with an asymptomatic, erythematous lesion on his left flank. The lesion had been first noted approximately a year ago and had gradually increased in size. The patient was otherwise in good health. There was no family history of similar skin disease or lupus erythematosus. He had been seen by his family physician, who had prescribed topical hydrocortisone and then subsequently a topical antifungal agent without much success.

Physical examination revealed an erythematous, indurated plaque without scaling or atrophy on the left flank. The rest of the physical examination findings were normal.

After referral to a dermatologist, blood work and a biopsy were performed. Results of a complete blood cell count, C-reactive protein test, renal function tests, hepatic function tests, and thyroid function tests were normal. Antinuclear antibody and antibodies against Ro/SSA and La/SSB were negative. A biopsy of the lesional skin revealed a normal epidermis and a dense perivascular lymphocytic infiltrate consisting mainly of T cells in the mid to lower dermis. Foci of plasmacytoid T cells also were seen within the cutaneous infiltrates.

What’s your diagnosis?

Choose one to reveal diagnosis and discussion

Granuloma annulare
Jessner lymphocytic infiltration of the skin
Polymorphic light eruption
Lymphocytoma cutis

Answer: Jessner lymphocytic infiltration of the skin

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A diagnosis of Jessner lymphocytic infiltration of the skin was made. Treatment with intralesional corticosteroids every 4 weeks and oral hydroxychloroquine resulted in resolution after 4 months of therapy, thus far with no recurrence.

Jessner lymphocytic infiltration of the skin (also known as lymphocytic infiltrate of Jessner) is a benign, cutaneous disease characterized clinically by asymptomatic, spontaneously resolving, infiltrated erythematous papules/plaques without scaling or atrophy that occur mainly on the face and trunk, and histologically by a coat-sleeve–like perivascular lymphoid infiltrate.1 The condition was first described by Max Jessner and Norman Kanof in 1953.2

EPIDEMIOLOGY: The exact incidence is not known, but the condition is believed to be uncommon.3 Jessner lymphocytic infiltration of the skin is most common in middle-aged men (mean age, 41 years).4 The occurrence is usually sporadic. Rarely, familial occurrence has been described.1

ETIOLOGY: In most cases, the exact etiology is not known. A few cases have been ascribed to Borrelia burgdorferi infection, drugs such as angiotensin-converting enzyme inhibitors, and contact allergy.5

HISTOPATHOLOGY: Histologic examination typically reveals a normal epidermis, dermal coat-sleeve–like perivascular, and to a less extent, periadnexal lymphoid infiltrates, consisting mainly of T lymphocytes.6 Foci of plasmacytoid monocytes are seen within the cutaneous infiltrates.1 It is believed that these plasmacytoid monocytes are involved in the activation, proliferation, and differentiation of T lymphocytes.7

CLINICAL MANIFESTATIONS: Jessner lymphocytic infiltration of the skin presents clinically as a single or multiple peripherally enlarging, erythematous, infiltrated papules or plaques that tend to take an annular or horseshoe-like configuration.3,4,6 Scaling and atrophy are characteristically absent.1 Some of the lesions may show central clearing.4 Sites of predilection include the face and the back.1,3 The lesions usually are asymptomatic, although some patients find them pruritic.4

DIAGNOSIS AND DIFFERENTIAL DIAGNOSIS: The diagnosis is based on clinical suspicion and is confirmed by histology. The main differential diagnosis is discoid lupus erythematosus. Discoid lupus erythematosus typically presents with well-demarcated, round or ovoid, erythematous/violaceous patches that may show adherent scales and follicular plugging.4 The lesions most often appear in the malar area. Atrophic scarring may be seen in older lesions.4 Histologically, discoid lupus erythematosus is characterized by hyperkeratosis with follicular plugging, atrophy of the epidermis with exocytosis of lymphocytes, and vacuolar degeneration of the basal layer of the epidermis.4 The absence of epidermal involvement and the presence of plasmacytoid monocytes within the dermal infiltrates in Jessner lymphocytic infiltration of the skin help to differentiate these 2 conditions.1 Although many authors consider Jessner lymphocytic infiltration of the skin to be a distinct disease entity, other authors believe that is an initial or variant stage of discoid lupus erythematosus.3

The differential diagnosis also includes polymorphic light eruption, granuloma annulare, morphea, annular erythema, and lymphocytoma cutis.3

COMPLICATIONS AND PROGNOSIS: The condition can be cosmetically unsightly and socially embarrassing, especially if it occurs on an exposed area of the body such as the face.

Although the disease is benign and spontaneous remissions are common, Jessner lymphocytic infiltration of the skin tends to run a chronic and relapsing/remitting course.6,8

MANAGEMENT: A variety of treatments have been tried with variable success, including topical and intralesional corticosteroids, topical calcineurin inhibitors, topical retinoids, oral corticosteroids, oral antimalarial agents (eg, hydroxychloroquine), oral methotrexate, psoralen plus ultraviolet A, and radiotherapy.8

References:

1. Toonstra J, van der Putte SCJ, de la Faille HB, van Vloten WA. Familial Jessner’s lymphocytic infiltration of the skin, occurring in a father and daughter. Clin Exp Dermatol. 1993;18(2): 142-145.

2. Jessner M, Kanof NB. Lymphocytic infiltration of the skin. AMA Arch Derm Syphilol. 1953:68(4): 447-449.

3. Cerio R, Oliver GF, Jones EW, Winkelmann RK. The heterogeneity of Jessner’s lymphocytic infiltration of the skin: immunohistochemical studies suggesting one form of perivascular lymphocytoma. J Am Acad Dermatol. 1990;23(1):63-67.

4. Akasu R, Kahn HJ, From L. Lymphocyte markers on formalin-fixed tissue in Jessner’s lymphocytic infiltrate and lupus erythematosus. J Cutan Pathol. 1992;19(1):59-65.

5. Laurinaviciene R, Clemmensen O, Bygum A. Successful treatment of Jessner’s lymphocytic infiltration of the skin with methotrexate. Acta Derm Venereol. 2009;89(5):542-543.

6. Poenitz N, Dippel E, Klemke C-D, Qadoumi M, Goerdt S. Jessner’s lymphocytic infiltration of the skin: A CD8+ polyclonal reactive skin condition. Dermatology. 2003;207(3):276-284.

7. Toonstra J, van der Putte SCJ. Plasmacytoid monocytes in Jessner’s lymphocytic infiltration of the skin: a valuable clue for the diagnosis. Am J Dermatopathol. 1991;13(4):321-328.

8. Tzung TY, Wu JC. Topical calcineurin inhibitors in treating Jessner’s lymphocytic infiltration of the skin: report of a case. Br J Dermatol. 2005;152(2):383-384.

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