Lupus erythematosus profundus

• Kaposi, 1875
• Irgang, 1940

Lupus panniculitis, Kaposi-Irgang syndrome, lupus erythematosus-panniculitis, LEP.

Special form of chronic cutaneous discoid lupus erythematosus.

• Peak of frequency of first manifestation: 20th-60th year
• Women:men = 2-4:1

Rarely periorbital oedema as an initial symptom. Mostly symmetrically arranged, sharply demarcated, erythematous, subcutaneous nodules or plaques with very subtle to no scaling. Ulceration and calcification may occur. Scarless healing, sometimes with lipatrophy.

• S. cutaneous lupus erythematosus
• Note:
  • ANAs (in 75% ↑)
  • Anti-dsDNA antibodies (rare)

Face, gluteal, upper arms and thighs.

Lobular panniculitis, hyaline necrosis of adipose tissue are characteristic, lymphoid cell infiltrates with formation of germinal centres, mucin deposition, adipose tissue necrosis, "interfacedermatitis", epitheloid cell nodules, oedematous upper corium. Orthohyperkeratosis and follicular keratosis.

• S. cutaneous lupus erythematosus

Avoid common mistakes:

• History should always include photosensitivity
• Medication history to rule out "drug-induced" lupus erythematosus
• Family and self history for autoimmune diseases
• Do not overestimate the ARA criteria! The ARA criteria are only conditionally suitable for the exclusion of systemic lupus erythematosus
• Avoid a purely superficial biopsy
• Therapy should not be changed until preventive measures to avoid possible trigger factors have been exhausted and sunscreen with a high sun protection factor is applied daily!

1. Irgang S. LUPUS ERYTHEMATOSUS PROFUNDUS. Archives of Dermatology and Syphilology 1940;42:97. 
2. Burrows NP, Walport MJ, Hammond AH, Davey N, Jones RR. Lupus erythematosus profundus with partial C4 deficiency responding to thalidomide. Br J Dermatol 1991;125:62-7. 
3. Süss R, Meurer M, Schirren CG, Lübke S, Ruzicka T. Lupus erythematodes profundus Kaposi-Irgang. Hautarzt 1994;45:38-41. 
4. Nitta Y. Lupus erythematosus profundus associated with neonatal lupus erythematosus. Br J Dermatol 1997;136:112-4. 
5. Grilli R, Soriano M, Izquierdo M, et al. Panniculitis Mimicking Lupus Erythematosus Profundus. The American Journal of Dermatopathology 1999;21:365-8. 
6. Grossberg E, Scherschun L, Fivenson D. Lupus profundus: not a benign disease. Lupus 2001;10:514-6. 
7. Massone C, Kodama K, Salmhofer W, et al. Lupus erythematosus panniculitis (lupus profundus): Clinical, histopathological, and molecular analysis of nine cases. J Cutan Pathol 2005;32:396-404. 
8. Wenzel J, Proelss J, Wiechert A, Zahn S, Bieber T, Tüting T. CXCR3-mediated recruitment of cytotoxic lymphocytes in lupus erythematosus profundus. Journal of the American Academy of Dermatology 2007;56:648-50. 
9. Walling HW, Sontheimer RD. Cutaneous lupus erythematosus: issues in diagnosis and treatment. Am J Clin Dermatol 2009;10:365-81. 
10. Okon LG, Werth VP. Cutaneous lupus erythematosus: diagnosis and treatment. Best Pract Res Clin Rheumatol 2013;27:391-404. 
11. Kuhn, A. (2016). S1-Leitlinie: Kutaner Lupus Erythematodes. Awmf.org. Retrieved 30 May 2016, from http://www.awmf.org/leitlinien/detail/ll/013-060.html