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Pyoderma Vegetans: A Case Report in a Child Suspected to Primary Immunodeficiency and Review of the Literature.

Mansouri M, Rakhshan A, Shahidi-Dadras M, Karimi A, Alavi S - Iran J Med Sci (2015)

Bottom Line: It is believed that this entity is mostly associated with inflammatory bowel disease (IBD), chronic malnutrition, human immunodeficiency virus (HIV), malignancies, and other immunocompromised states.Our patient was an 11-year-old girl suspected to have primary combined immunodeficiency complicated by chronic recurrent vegetating pustular lesions on the face and postauricular area since one year of age.This is the first case of PV beginning from early infancy in the setting of primary immunodeficiency and in an unusual location.

View Article: PubMed Central - PubMed

Affiliation: Department of Immunology and Allergy, Mofid Children Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran;

ABSTRACT
Pyoderma vegetans (PV) is a rare inflammatory disorder characterized by vegetating pustules and plaques affecting the skin and mucosal membranes. It is believed that this entity is mostly associated with inflammatory bowel disease (IBD), chronic malnutrition, human immunodeficiency virus (HIV), malignancies, and other immunocompromised states. Pyoderma vegetans occurs more commonly in young and middle-aged adults. There is no sex predilection for this entity. The lesions could heal spontaneously, but usually recur and become chronic. Our patient was an 11-year-old girl suspected to have primary combined immunodeficiency complicated by chronic recurrent vegetating pustular lesions on the face and postauricular area since one year of age. The histological features of the lesions were consistent with pyoderma vegetans. This is the first case of PV beginning from early infancy in the setting of primary immunodeficiency and in an unusual location.

No MeSH data available.


Related in: MedlinePlus

Facial lesions of PV in our patient at the age of 11 years.
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Figure 1: Facial lesions of PV in our patient at the age of 11 years.

Mentions: An 11-year-old girl was referred to our department with a history of labial herpes infection, recurrent episodes of suppurative otitis media and suspected immunodeficiency for the past three years. Her past medical history was positive for skin eczema and recurrent chronic ulcers on the face since one year of age and repeated hospitalizations for pulmonary infections, anal abscess, and adenoidectomy since early childhood. Drug history was negative for any halogenic drug consumption. On physical examination and during the regular follow up visits, a group of well defined exudative vegetative ulcers with numerous pustules and elevated margins, exacerbating and remitting over the face, mainly around the nose, upper lip and cheek measuring from 2 to 8 cm in maximum diameter was noted (figure 1).


Pyoderma Vegetans: A Case Report in a Child Suspected to Primary Immunodeficiency and Review of the Literature.

Mansouri M, Rakhshan A, Shahidi-Dadras M, Karimi A, Alavi S - Iran J Med Sci (2015)

Facial lesions of PV in our patient at the age of 11 years.
© Copyright Policy
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4487467&req=5

Figure 1: Facial lesions of PV in our patient at the age of 11 years.
Mentions: An 11-year-old girl was referred to our department with a history of labial herpes infection, recurrent episodes of suppurative otitis media and suspected immunodeficiency for the past three years. Her past medical history was positive for skin eczema and recurrent chronic ulcers on the face since one year of age and repeated hospitalizations for pulmonary infections, anal abscess, and adenoidectomy since early childhood. Drug history was negative for any halogenic drug consumption. On physical examination and during the regular follow up visits, a group of well defined exudative vegetative ulcers with numerous pustules and elevated margins, exacerbating and remitting over the face, mainly around the nose, upper lip and cheek measuring from 2 to 8 cm in maximum diameter was noted (figure 1).

Bottom Line: It is believed that this entity is mostly associated with inflammatory bowel disease (IBD), chronic malnutrition, human immunodeficiency virus (HIV), malignancies, and other immunocompromised states.Our patient was an 11-year-old girl suspected to have primary combined immunodeficiency complicated by chronic recurrent vegetating pustular lesions on the face and postauricular area since one year of age.This is the first case of PV beginning from early infancy in the setting of primary immunodeficiency and in an unusual location.

View Article: PubMed Central - PubMed

Affiliation: Department of Immunology and Allergy, Mofid Children Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran;

ABSTRACT
Pyoderma vegetans (PV) is a rare inflammatory disorder characterized by vegetating pustules and plaques affecting the skin and mucosal membranes. It is believed that this entity is mostly associated with inflammatory bowel disease (IBD), chronic malnutrition, human immunodeficiency virus (HIV), malignancies, and other immunocompromised states. Pyoderma vegetans occurs more commonly in young and middle-aged adults. There is no sex predilection for this entity. The lesions could heal spontaneously, but usually recur and become chronic. Our patient was an 11-year-old girl suspected to have primary combined immunodeficiency complicated by chronic recurrent vegetating pustular lesions on the face and postauricular area since one year of age. The histological features of the lesions were consistent with pyoderma vegetans. This is the first case of PV beginning from early infancy in the setting of primary immunodeficiency and in an unusual location.

No MeSH data available.


Related in: MedlinePlus