Diffuse skin infiltration

Paediatric dermatology

Dr. Emmanuelle Bourrat

Saint-Louis Hospital

The clinical case
Now it's your turn!
Co-prescription and advice

Clinical case presentation

On vacation for 8 days on the Mediterranean coast, this 9-year-old boy, a former asthmatic, suddenly presented with tense, painful inflammatory edema of the face and four limbs. There were no known triggers and, in particular, no medications.

Antihistamine treatment was ineffective, and the worsening of symptoms led the patient to the emergency room. He was apyretic and had no extracutaneous complaints. Blood tests (renal and liver function, CPK, CRP, albumin) and urine tests (proteinuria) were normal, apart from blood hypereosinophilia of 1,500 × 10⁶/ml.

Skin biopsy of an indurated area at the root of the thigh showed an eosinophil-rich dermohypodermal infiltrate with evidence of flame figures.

Your turn

What is your diagnosis?

Select 1 answer(s) from the following choices:

Scleredema

Angioedema

Shulman’s fasciitis

Wells' cellulitis

Wrong answer!

Good answer!

Selected diagnosis

This histological appearance is typical of Wells’ cellulitis. Wells’ cellulitis is rare in children and most often occurs in the context of trauma, insect bites or infection. Atopy appears to be a predisposing factor.

The clinical picture is either that of a unifocal or multifocal inflammatory cellulitis, even diffuse as in our patient, or that of fixed urticaria.

The histological appearance is identical in the two presentations. Blood hypereosinophilia is present in three-quarters of cases, with no visceral repercussions. The course of the disease is highly variable, sometimes spontaneously favorable, but may also require treatment with topical corticosteroids or systemic treatment (systemic corticosteroids or sulfones).

Explanation of wrong answers

Buschke's scleredema is very rare. In children, it usually appears after a febrile infectious episode, presenting as an indurated edematous cape-like distribution over the nape of the neck and shoulders, then extending to all four limbs while sparing the extremities. Histology shows a dermal thickening with collagen bundles separated by mucin, the specific marker; evolution is spontaneously favorable within a few months.
Angioedema, whether histaminic or bradykinin-mediated, is a non-inflammatory, rarely generalized edema that progresses in flare-ups.
Shulman’s fasciitis is a deep morphea, sometimes quite diffuse and of abrupt onset, often accompanied by blood and skin hypereosinophilia but with a different histological appearance. Any progressive, fibrous-appearing lesion, even congenital, should raise suspicion of a Darier-Ferrand dermatofibroma and therefore be the subject of a deep biopsy.

Treatment

Strong topical corticosteroids will help the condition improve in two weeks, but systemic corticosteroids or an immunomodulator such as dapsone may be required.

The expert's message

Not all edemas are angioedemas; inflammatory edema of non-infectious origin should be examined histologically to guide the etiologic diagnosis.

References:

^{J. Brun et al. Wells syndrome in children and atopy: retrospective study of 11 cases and review of the literature. Ann Dermatol Venereol 2015; 142: 320-331}