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Lupus erythematosus profundusL93.2

Author:Prof. Dr. med. Peter Altmeyer

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Last updated on: 09.01.2024

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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

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HistoryThis section has been translated automatically.

Kaposi, 1875; Irgang, 1940

DefinitionThis section has been translated automatically.

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.

ManifestationThis section has been translated automatically.

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.

LocalizationThis section has been translated automatically.

Mostly face, buttocks, thighs.

Clinical featuresThis section has been translated automatically.

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.

HistologyThis section has been translated automatically.

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 diagnosisThis section has been translated automatically.

Malignant lymphomas (esp. subcutaneous panniculitis-like cutaneous T-cell lymphoma): Histologic exclusion

Panniculitis: Exclusion by histologic or serologic phenomena of lupus erythematosus.

Acne conglobata: Exclusion by absence of typical acne phenomena (seborrhea, comedones, acne history)

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

Complication(s)This section has been translated automatically.

Rare exacerbation; visceral involvement.

TherapyThis section has been translated automatically.

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/forecastThis section has been translated automatically.

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

LiteratureThis section has been translated automatically.

  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

Authors

Last updated on: 09.01.2024

Author: Prof. Dr. med. Peter Altmeyer