Lupus erythematosus profundus L93.2

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 29.10.2020

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Synonym(s)

Kaposi's gait syndrome; LEP; lupus erythematosus panniculitis; Lupus erythematosus Panniculitis; Lupus paniculitis; Lymphocytic infiltration of the skin; Panniculitis; panniculitis lupus; Panniculitis Lupus erythematosus panniculitis; Sarcoid Darier-Roussy

History
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Kaposi, 1875; Irgang, 1940

Definition
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Rare special form of lupus erythematosus integumentalis (about 2-3% of the cases) with painful, inflammatory reddened nodes reaching into the subcutis without significant epidermal involvement. Healing with formation of retracted scars. Combinations with lupus erythematosus chronicus discoides (about 30% of cases) or, less frequently, with systemic lupus erythematosus are possible.

Remark: The clinical picture is probably identical with the"Lymphocytic infiltration of the skin" described by Jessner and Kanof.

Manifestation
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Occurs several years before a manifest lupus erythematosus. Predominantly occurring in adults between the ages of 20 and 60; women are affected 2-4 times more frequently than men.

Localization
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Mostly face, buttocks, thighs.

Clinical features
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Usually several, often symmetrically localized, firm, sharply defined mostly symptom-free, subcutaneous nodules or plaques. The overlying skin is discrete red or brown-red and may be slightly bsi clearly retracted. An epidermal involvement is usually absent.

Isolated slight atrophy with delicate scaling.

Healing occurs with dents of varying depth. The disease tends to be intermittent chronic. The overlying epidermis occasionally shows pigmentary shifts.

Histology
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Lobular panniculitis with pronounced inflammatory infiltration of the subcutis. Hyaline necrosis of the fatty tissue is characteristic. The infiltrate is characterized by small lymphocytes with chromatin dense nuclei and plasma cells of varying amounts. Nodular densification of the lymphocytes with formation of germinal centres is frequent. In the periphery also smaller epitheloid cell nodules with strong lymphocyte border. Edema in the upper corium. Perivascular lymphocytic infiltrates with CDLE-typical epidermal changes with orthohyperkeratosis, follicular keratosis, hydropic degeneration of the cells of the stratum basale may be detectable.

Differential diagnosis
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Malignant lymphomas: Histological exclusion

Panniculitis: exclusion by histological or serological phenomena of lupus erythematosus.

Acne conglobata: exclusion due to the absence of typical acne phenomena (seborrhea, comedones, acne history)

Syphilitic gums (very rare today; see Syphilis acquisita below): exclusion due to negative syphilis serology.

Complication(s)
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Rare exacerbation; visceral involvement.

Therapy
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Local glucocorticoids under occlusion; in case of localized process also intralesional application of triamcinolone, if necessary. In addition, continuous therapy with antimalarials ( chloroquine) in a dosage of 1-2 times/day 200 mg p.o. Good long-term effects with cyclophosphamide have been reported. There are some positive reports about the use of Infliximab.

Progression/forecast
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Systemic lupus erythematosus develops in about 30-35% of patients.

Literature
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  1. Diaz-Cascajo C, Borghi S (2002) Subcutaneous pseudomembranous fat necrosis: new observations. J Cutan catholic 29: 5-10
  2. Grossberg E et al (2001) Lupus profundus: not a benign disease. Lupus 10: 514-516
  3. Günther C et al (2011) Infliximab treatment of a patient with lupus erythematosus profundus. Abstract CD 46th DDG meeting: P16/13
  4. Hebra F, Kaposi M (1875) On Diseases of the Skin, Including Exanthema. New Sydenham Society, London, 4:1-247
  5. Irgang S (1940) Lupus erythematosus profundus: report of example with clinical resemblance to Darrier-Roussy sarcoid.Archives of Dermatology and Syphilology, Chicago, 42: 97-108
  6. Kaposi M (1883) Pathology and therapy of skin diseases. 2nd edition, Urban & Schwarzenberg, S. 642
  7. Ng PP, Tan SH, Tan T (2002) Lupus erythematosus panniculitis:a clinicopathologic study. Int J Dermatol 41: 488-490
  8. Wätzig V et al (1989) Lupus panniculitis (Lupus erythematodes profundus). Act Dermatol 15: 109-111
  9. Wimmershoff MB et al (2003) Discoid lupus erythematosus and lupus profundus in childhood: a report of two cases. Pediatric dermatol 20: 140-145

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Last updated on: 29.10.2020