DefinitionThis section has been translated automatically.
The term "acute syndrome of apoptotic pan-epidermolysis" (ASAP) denotes a severe clinical clinical picture with features of erythema multiforme (up to its maximum variant, Stevens-Johnson syndrome) and (bullous) systemic lupus erythematosus. Histologically striking is a prominent apoptotic cleavage of the epidermis due to acute damage of the epidermal basal cells. Triggering factors are frequently drugs, if necessary in combination with UV exposure.
Although no symptom is conclusive on its own, a combination of recent (S)LE exacerbation, obvious photodistribution, anular lesions, absent or only mild focal erosive mucosal involvement may support the diagnosis of LE.
HistologyThis section has been translated automatically.
Subepidermal blister with exocytosis and superficial, moderately dense perivascular infiltration of lymphocytes and neutrophils, marked necrosis of keratinocytes of the suprabasal epithelial layer (H&E).
Note(s)This section has been translated automatically.
Such apoptotic damage of the surface epithelium typically occurs in drug-induced classic toxic epidermal necrolysis (TEN). However, they can also occur in non-drug-induced disease patterns (TEN-like diseases) such as lupus erythematosus bullosus or acute graft-versus-host disease.
The independence of the clinical picture is disputed by various authors. In particular, it is not distinguished from Rowell's syndrome, which was described in the 1960s and in which the overlapping aspect between systemic lupus erythematosus(bullous systemic lupus erythematosus) and erythema multiforme was emphasized. In this syndrome, the following criteria apply:
- Major criteria: skin lesions of lupus erythematosus and erythema exudativum, positive ANA with speckled pattern.
- Minor criteria: positive SS-A and SS-B antibodies, reactive rheumatoid factor, chilblain-like skin lesions.
- For diagnosis, all major criteria and at least one minor criterion must be present.
LiteratureThis section has been translated automatically.
- Boontaveeyuwat Eet al.(2012) Toxic epidermal necrolysis-like acute cutaneous lupus erythematosus (TEN-like ACLE) in SLE patients: a report of two cases. Asian Pac J Allergy Immunol 30:83-88 ( illus.).
- Burger Eet al. (2018) Acute syndrome of pan-epidermolysis and thrombotic storm arising in a patient with systemic lupus erythematosus. JAAD Case Rep 4: 877-879.
- Jang HW et al (2016) Stevens-Johnson syndrome-like skin lesions in a patient with juvenile systemic lupus erythematosus. Ann Dermatol 28:117-118.
- Obermoser G et al (2010) Overview of common, rare and atypical manifestations of cutaneous lupus erythematosus and histopathological correlates. Lupus 19:1050-1070.
- Ryan E et al (2012) Toxic epidermal necrolysislike subacute cutaneous lupus erythematosus. Australas J Dermatol 53:303-306.
- Subepidermal blister with exocytosis and superficial perivascular infiltration of lymphocytes and neutrophils, marked necrosis of keratinocytes of the suprabasal epithelial layer (H&E).
- Ting W et al. (2004) Toxic epidermal necrolysis-like acute cutaneous lupus erythematosus and the spectrum of the acute syndrome of apoptotic pan-epidermolysis (ASAP): a case report, concept review and proposal for new classification of lupus erythematosus vesiculobullous skin lesions. Lupus13:941-950.
- Yildirim Cetin G et al. (2013) A case of toxic epidermal necrolysis-like skin lesions with systemic lupus erythematosus and review of the literature. Lupus 22:839-846.
- Zargham H et al. (2020) A case of toxic epidermal necrosis-like cutaneous eruption as the first manifestation and clue to the diagnosis of systemic lupus erythematosus: A case report. SAGE Open Med Case Rep 8:2050313X20940420. (Figure)
- Ziemer M et al. (2012) Stevens-Johnson syndrome and toxic epidermal necrolysis in patients with lupus erythematosus: a descriptive study of 17 cases from a national registry and review of the literature. Br J Dermatol166:575-600.
Incoming links (1)Bullous systemic lupus erythematosus;
Outgoing links (4)Bullous systemic lupus erythematosus; Erythema multiforme; Graft-versus-host disease acute; Rowell's syndrome;
Please ask your physician for a reliable diagnosis. This website is only meant as a reference.