Case Number : Case 1058 - 14th July Posted By: Guest

We are delighted to have a new DermpathPRO editor and contributor to the site, Dr Uma Sundram, Assistant Professor of Pathology and Dermatology at Stanford University. She is board certified in Anatomic Pathology and Dermatopathology. She is a Fellow of the American Society of Dermatopathology, Fellow of the College of American Pathologists, and a member of the International Society of Dermatopathology, the United States Cutaneous Lymphoma Consortiuma, the International Society of Cutaneous Lymphoma, and the United States and Canadian Academy of Pathology. She serves on the Editorial Board of the Journal of Cutaneous Pathology and has published over 50 peer reviewed original articles as well as several review articles and book chapters. Today's case has been submitted by her.

I think there is mismatch between the clinical presentation and the pathology.
This is the pathology of Superficial pemphigus or may be SSSS in this age and with the paucity of inflammatory infiltrate. Both do not include firm blue nodules in the clinical presentation??!!

I believe this is a case of Pemphigus foliaceus of neonatal onset probably initiated by transfer of antibodies from her mother across the placenta.

Welcome Dr. Uma Sundram!

i agree the pathology of superficial pemphigus

Localised pemphigus foliaceus. Agree with Igor about the possibility of transfer of antibodies, from the mother, across the placenta[font=Revival565BT-Roman][size=2][font=Revival565BT-Roman][size=2]. [/size][/font][/size][/font]

I'm considering two diagnoses: staphylococcal scalded skin syndrome and pemphigus foliaceus of Cazenave. I favor the former due to the age of the patient, the almost absent inflammatory infiltrate, and the exfoliated stratum corneum in the Image 4. Anyway, it's hard to correlate those two hypotheses with the clinical presentation of a blue firm nodule on a limb.

Welcome, Dr. Sundram.

The problem with the diagnosis of SSSS, in my opinion, is that the patient has one month old and this clinical picture began within her first week of age.

Agree with you, my friend Igor, that we cannot construe satisfactorily a diagnosis of staphylococcal scalded skin syndrome from the available case history since there's no information about scarlatiniform eruption, flaccid bullae and large areas of desquamation. However, nor can we construe satisfactorily a diagnosis of superficial pemphigus from the same case history because a blue firm nodule on a limb doesn't fit in the clinical spectrum of superficial pemphigus, to wit, there's no information about flaccid bullae that crop out recurrently and rupture, giving rise to shallow crusted erosions.
I'm curious about Dr. Sundram's expert explanation.

Yes, with this correct clinical, this is a case of SSSS. I have suggested it earlier due to paucity of inflammatory infiltrate...
Welcome Dr. Sundrum
Looking forward for your interesting cases

Yes, probably SSSS. But I would make a call to find out some informations about her mother

[size=4][font=arial,helvetica,sans-serif]Thanks everyone!

Diagnosis is Staphyloccal scalded skin syndrome. This is a disorder that results from the production of an epidermolytic toxin usually caused by a strain of [i]Staphylococcus aureus[/i]. The typical patient are usually healthy infants and children. There is a sudden onset of skin tenderness and scarletiniform rash. Flaccid bullae subsequently develop with a positive Nikolsky sign, and desquamation can occur, often in sheets. The face, neck, trunk and groin are often involved. Mucous membranes are spared, which tends to exclude both toxic epidermal necrolysis (TEN) and acute graft-versus-host disease (GVHD). The prognosis is very good in children and the disease is treated with antibiotics and appropriate nursing care. On histopathology, a split is seen at the level of the stratum granulosum, which is a subcorneal split rather than subepidermal. This further excludes both acute GVHD and TEN. Acantholytic cells and rare neutrophils can be seen within the blister cavity (if a relatively intact area is biopsied). In the denuded strip of stratum corneum, attached acantholytic cells may be seen. A sparse mixed dermal infiltrate can also be seen, and the presence of neutrophils would make the diagnoses of TEN and acute GVHD unlikely. Pemphigus foliaceus is an important differential diagnostic consideration but would be excluded with a negative direct immunofluorescence study (DIF). The DIF in this case was negative and the mother did not have pemphigus.[/font][/size]