Acute Syndrome of Apoptotic Panepidermolysis L93.1

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.

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).

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.

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6. Subepidermal blister with exocytosis and superficial perivascular infiltration of lymphocytes and neutrophils, marked necrosis of keratinocytes of the suprabasal epithelial layer (H&E).
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