INTRODUCTION

Pyodermatitis-pyostomatitis vegetans (PD-PSV) is a rare chronic mucocutaneous dermatosis first described by François Hallopeau in 1898 [1]. Some authors refer to a spectrum of neutrophilic dermatoses, an oral variant of pyoderma gangrenosum. It is closely related to chronic inflammatory bowel disease, particularly ulcerative colitis [2,3]. PD-PSV usually appears in the late thirties, is more prevalent in females than males, and rarely affects children [4].

Herein, we report a case of PD-PSV in a primary immunodeficiency child without associated intestinal disease.

CASE REPORT

A five-year-old child followed for persistent perinatal candidiasis and resistant to symptomatic treatment was referred to our training for the management of skin lesions treated as impetigo. On examination at the hospital, we discovered erosive lesions with a polycyclic border, vegetating in some spots (Fig. 1), evolving in a context of tingling, indicating vegetating impetigo associated with a herpetic superinfection.

Figure 1: Multiple, erythematous, vegetative plaques on the trunk and face.

The patient was administered amoxicillin and aciclovir in hope of remission. In view of the recurrent infections, the delayed growth, and the young age of the patient, primary immune deficiency was suspected, and a first-line work-up was requested, showing hypereosinophilia with hyper-IgE.

During the development of the lesions associated with onychodystrophy (Figs. 2a – 2c), a cutaneous biopsy was performed in an attempt to determine whether the diagnosis was pyodermatitis-pyostomatitis vegetans or pemphigus vegetans. Histology revealed an inflammatory infiltrate rich in neutrophils and eosinophils, epidermal acanthosis, and intraepithelial abscesses associated with gigantic cellular epithelioid granuloma (Figs. 3a and 3b).

	Figures 2: (a) Clinical and (b-c) dermoscopic images of the nails.
	Figure 3: (a-b) Histology revealing a rich inflammatory infiltrate and abscesses with acantholysis associated with gigantic cellular granuloma without necrosis.

However, this granuloma may be consistent with his PID or the associated IBD.

A workup was performed to determine the association of the granuloma with DIP. However, the absence of ANCA and fecal calprotectin did not indicate associated IBD, which was still strongly suspected.

A low level of G6PD prevented us from administering Disulone, and the patient was treated with immunoglobulins and dermocorticoids with favorable evolution and the almost total disinflation of the lesions (Fig. 4).

Figure 4: Disinflation of the lesions.

A mycological sample from a nail was taken and was in favor of candida albicans, and the patient was initiated on Diflucan 50 mg/day for ten days and then 1 per week for three months with positive evolution (Fig. 5).

Figure 5: Positive evolution of onychodystrophy.

DISCUSSION

Pyodermatitis-pyostomatitis vegetans is a rare inflammatory skin disease. His original explanation for Hallopeau in 1989 was that the cause was unknown [1,3]. It may affect all ages, mainly young people and rarely children, mostly males [5]. Its exact etiology is unknown and poorly understood, yet appears to be an alteration of the neutrophil-mediated mucocutaneous response to an underlying systemic disease or cutaneous infection induced by a purulent agent [6]. Clinically, pyostomatitis vegetans affects the oral mucosa (mainly the gums, inner cheeks, palate, and lips), resulting in the formation of erythematous, linear, and tortuous bases of small, confluent pustules forming cochlear scars [1]. Pustules open and shallow ulcers form. Other mucous membranes are rare. Pyodermatitis vegetans (58%) may produce bullous, pustular, exudative, vegetative plaques with active erosive borders, often on the scalp, axillae, and groins. Acantholysis and intraepithelial or subepithelial microabscesses (or both) without granuloma are typical histological findings [7,8]. A dense infiltration of mixed inflammatory cells is also present in the underlying connective tissue. Direct immunofluorescence studies are commonly employed to exclude vegetative pemphigus [9].

A positive diagnosis is based on clinical and histological features, hypereosinophilia, and associated chronic inflammatory bowel disease [10,11]. Other diseases reported in association with PPV include T-cell lymphoma, chronic myeloid leukemia, liver diseases, malnutrition, HIV infection, and primary immunodeficiency disorders [12,13]. In our patient, the same clinical features, peripheral eosinophilia, and histological features associated with a granuloma were found, which led us to believe that it was associated with IBD or, in particular, hyper-IgE PID.

The treatment of PD-PSV consists of the administration of topical steroids and tacrolimus, dapsone, azathioprine, cyclosporine, isotretinoin, infliximab, and/or methotrexate [14,15]. The treatment of concomitant inflammatory bowel disease often results in the improvement of mucocutaneous lesions.

Given the patient’s ID terrain, all immunosuppressive drugs were contraindicated, as was dapsone (low G6PD dosage). Only dermocorticoids and immunoglobulins were administered, with favorable results.

However, to our knowledge, there was no reported association with primary immune deficiency, which remains a heterogeneous group of hereditary disorders caused by genetic mutations that alter the immune system, and which is associated with several skin manifestations, such as resistant bacterial and viral infections, recurrent mucocutaneous candidiasis (MCC), and granulomatous lesions. Therefore, it would be interesting to study the relationship between these mucocutaneous lesions and primary immunodeficiency, which are associated with numerous cutaneous conditions such as recurrent candidiasis.

However, there are no published examples describing PD-PSV in relation to PID.

CONCLUSION

In cases of recurrent infections in children, one should always look for primary immune deficiency, and in cases of resistant vegetative impetigo, one should recognize pyodermatitis-pyostomatitis vegetative.

In our case, we illustrate an association between DIP and PD-PSV, which remains hypothetical, as they may share a common pathogenic pathway, making their management rather challenging.

REFERENCES

Notes