A worrying trend

Inflammatory dermatosis

Dr. Emmanuelle Bourrat

Saint-Louis Hospital

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

Clinical case presentation

A 9-year-old girl with no previous history presented with a left basithoracic infiltrated plaque.

This was a small congenital lesion of the left hypochondrium, which seemed to have grown and changed with age and was still evolving toward both the back and the umbilicus.
The lesion was tender to the touch, but not spontaneously, and had no functional impact.

On examination, a firm, nipple-like, subcutaneous induration without epidermal abnormality was found, tender to palpation, measuring 27 × 7 cm, vaguely linear. At the dorsal end of the plaque, there were papules of normal skin color, giving an orange peel appearance. The rest of the examination was strictly normal. A biopsy and skeletal X-rays were suggested.

Your turn

What is your diagnosis?

Select 1 answer(s) from the following choices:

Deep collagenous conjunctival hamartoma

Deep morphea

Shulman’s fasciitis

Dermatofibrosarcoma of Darier-Ferrand

Wrong answer!

Good answer!

Selected diagnosis

Skin biopsy reveals thick, hyalinized, all-directional collagen bundles in the deep dermis. The elastic network, on the other hand, is normal. There is no inflammation. This is a deep collagenous connective tissue nevus. Bone radiology is normal.

Histologically, a connective tissue nevus (CTN) is defined by alteration of one or more components of the extracellular matrix of the dermis: elastin, collagen or glycosaminoglycans.

The basic lesion of a connective tissue nevus is a papule of normal or slightly yellow skin color, which may be single or multiple; in the latter case, the papules are disseminated or grouped together in more or less extensive plaques.

Sometimes syndromic, CTN may be associated with bone dysplasia (osteopoikilosis of Buschke-Ollendorff syndrome), tuberous sclerosis of Bourneville (shagreen plaque) or Proteus syndrome (plantar collagenoma).

Explanation of wrong answers

In deep morphea, the inflammatory and sclerotic process mainly involves the hypodermis, which is why the “lilac ring” and pearly-white appearance are often absent, giving way to skin infiltration in the form of a “peau d’orange” or “shield-like” appearance. The prognosis is mainly functional with regard to limb joints (risk of flexion contracture) or the thoracic cage (restrictive syndrome).
In Schulman’s fasciitis, which also belongs to the spectrum of cutaneous scleroderma, sclerosis is even more profound as it affects the fascia, giving the skin a highly suggestive padded appearance and is frequently associated with hypereosinophilia of the blood and subcutaneous tissues.
Any progressive lesion with a fibrous appearance, even congenital, should raise suspicion of a Darier dermatofibroma and should therefore be the subject of a deep biopsy.

Treatment

The prognosis is unknown (duration of evolution?), but to date there is no medical treatment.

Message from the expert

Extensive fibrous and progressive forms of deep collagenous hamartoma, as in this observation, are often non-syndromic but pose problems of differential diagnosis and prognosis.

References :

^{O lafargue and coll. Extensive conjunctival hamartoma of the child. Ann Dermatol Venereol 2017; 144:}^700-704.