Fig. 33.1
Upper panel shows an elderly woman with painful deep erythema and focal sloughing caused by Stevens Johnson syndrome secondary to Dilantin received following brain surgery. Lower panel shows a middle-aged woman with deep redness and sloughing due to acute photoinduced lupus erythematosus as the initial presentation of systemic lupus erythematosus (SLE). Later, the patient developed extensive lesions of discoid lupus erythematosus and lupus panniculitis
Case
A patient presents with diffusely red skin with pain and sloughing.
The clinical differential diagnosis includes
acute phototoxicity,
acute graft versus host disease,
Paraneoplastic pemphigus,
acute photosensitive lupus,
staphylococcal scalded skin syndrome,
Stevens Johnson syndrome SJS, and
toxic epidermal necrolysis, TEN.
How Helpful Is the Pathology?
Only somewhat + / + +
Clinical and Histological Differential Diagnosis
The clinical presentation of red, sloughing patches results from the process of “cytotoxic dermatitis” in which epidermal cells undergo death by apoptosis or other mechanisms. The histological findings are so similar that it is quite difficult to distinguish among the disorders based solely on histological findings. A biopsy from a patient with sloughing skin is valuable in excluding other disorders in which blistering occurs within the epidermis and without cell death, such as staph-scalded skin syndrome or extensive pemphigus.
The diagnosis of acute phototoxicity is usually easily made by the acute nature of the clinical presentation of a sunburn-like reaction in the presence of a phototoxic medication. The histopathology is that of acute sunburn that is epidermal cell necrosis with no infiltrate. Later neutrophils may be seen in the epidermis.
The diagnosis of acute graft versus host disease (GVHD) is also generally easy to make in the right setting of recent bone marrow transplant. Grade 4 or severe acute GVHD results in full-thickness epidermal necrosis and sloughing.
The diagnosis of severe acute photosensitive lupus with epidermal necrosis is not as easy to make. This presentation of lupus is rare but may occur as the initial manifestation of systemic lupus erythematosus (SLE) when the patient is not yet known to have SLE with photosensitivity, and extremely rarely, subacute cutaneous lupus erythematosus (SCLE) , in both disorders following extensive ultraviolet light exposure in an unsuspecting patient.
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