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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

Eosinophilic dermal abscess formation (Hematoxylin-eosin stain, ×400).
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Figure 4: Eosinophilic dermal abscess formation (Hematoxylin-eosin stain, ×400).

Mentions: At first, the lesions used to respond to a broad spectrum of antibiotics and acyclovir, however, two years later, similar vegetative ulcers appeared in the postauricular area. On examination, the ear lobe was severely inflamed and a purulent discharge from external ear canal was noticeable (figure 2). Mild coarse facies, submandibular lymphadenopathies and eczematous, dry skin specially over the elbows were also detected during the recurrence of the disease. Routine blood tests showed raised erythrocyte sedimentation rate equal to 75/1st hr, mild lymphopenia (WBC: 13.5x10³/µl with 8% lymphocytes), and marked eosinophilia (11 to 35% of white blood cells). Immunological profile was as follows: Nitroblue tetrazolium test was 99%, which was interpreted as normal. Immunoglobulin G, M, A and E (IgG, IgM, IgA and IgE) levels were 2550, 18, 29 mg/dl and 127 IU/ml, respectively. Specific antibody response to diphtheria and tetanus were 0 and 0.01, respectively. Peripheral blood cells flow cytometry showed CD3: 53%, CD4: 25%, CD8: 28%, CD19: 22%, CD16+56: 11%, and CD4/CD8: 0.89 .The results of lymphocyte transformation test (LTT) for phytohemaglutinin (PHA/mitogen) disclosed stimulation index (SI) of 10.05 for patient and 10.67 for control with normal range of 10-20. The lymphocyte SI with Bacille calmette guerin (BCG) and Candida antigens illustrated 1.14 for patient comparing to 5.37 for control and also 1.19 for patient contrasting 7.07 for control, correspondingly with the normal range of 3-10, which were severely impaired. HIV serology was negative. According to clinical features and laboratory data in favor of primary immunodeficiency, parenteral immunoglubolin (IVIG) therapy was started. Microbial culture of the skin lesions was positive for coagulase negative staphylococci. Tissue Polymerase Chain Reaction (PCR) was positive for Ctomegalovirus (CMV) and Herpes Simplex Virus Type 1 (HSV-1), but negative for Herpes Simplex Virus Type 2 (HSV-2). It was also negative for acid fast bacilli and fungi. Tzanc smear on the ulcers were positive for HSV. RIDA allergy screen food panel (R-Biopharm, Darmstadt, Germany) was negative. The hematological and gastroenterological assessments were in favor of neither malignancy nor inflammatory bowel disease. Chest X-ray disclosed bilateral basal tubular bronchiectatic changes with peribronchial thickening. Spiral Computerized Tomography (CT) scan of the thorax demonstrated multiple bilateral lymph nodes in axillary and paratracheal areas with air trapping and peribronchial thickening in both lung fields. Abdominopelvic contrast enhanced CT scan showed increased mucosal thickening of the small bowel, small mesenteric lymphadenopathies, and bilateral mild hydronephrosis. A core biopsy of the lesion of the postauricular area was performed. The characteristic histological findings consisted of pseudoepitheliomatous hyperplasia and intraepithelial or dermal microabscess composed of eosinophils, along with focal ulceration, eosinohilic spongiosis and heavy infiltration of eosinophils, lymphocytes and neutrophils in dermis (figures 3 and 4). Periodic Acid-Schiff stain (PAS) was negative for fungi organisms. A perilesional skin biopsy was negative for direct immunofluorescence. According to the above mentioned clinical features9 and pathological findings the definite diagnosis of pyoderma vegetans was applied. The lesions were unresponsive to a two-month period of treatment with broad spectrum antibiotics and acyclovir following which the patient was determined to receive systemic corticosteroid therapy with unfavorable response neither to one month of oral prednisone (2 mg/kg/day) nor to a three-day course of intravenous corticosteroid pulse-therapy (30 mg/kg/day). It should be noted that a written informed consent was already obtained from the parents.


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)

Eosinophilic dermal abscess formation (Hematoxylin-eosin stain, ×400).
© Copyright Policy
Related In: Results  -  Collection

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

Figure 4: Eosinophilic dermal abscess formation (Hematoxylin-eosin stain, ×400).
Mentions: At first, the lesions used to respond to a broad spectrum of antibiotics and acyclovir, however, two years later, similar vegetative ulcers appeared in the postauricular area. On examination, the ear lobe was severely inflamed and a purulent discharge from external ear canal was noticeable (figure 2). Mild coarse facies, submandibular lymphadenopathies and eczematous, dry skin specially over the elbows were also detected during the recurrence of the disease. Routine blood tests showed raised erythrocyte sedimentation rate equal to 75/1st hr, mild lymphopenia (WBC: 13.5x10³/µl with 8% lymphocytes), and marked eosinophilia (11 to 35% of white blood cells). Immunological profile was as follows: Nitroblue tetrazolium test was 99%, which was interpreted as normal. Immunoglobulin G, M, A and E (IgG, IgM, IgA and IgE) levels were 2550, 18, 29 mg/dl and 127 IU/ml, respectively. Specific antibody response to diphtheria and tetanus were 0 and 0.01, respectively. Peripheral blood cells flow cytometry showed CD3: 53%, CD4: 25%, CD8: 28%, CD19: 22%, CD16+56: 11%, and CD4/CD8: 0.89 .The results of lymphocyte transformation test (LTT) for phytohemaglutinin (PHA/mitogen) disclosed stimulation index (SI) of 10.05 for patient and 10.67 for control with normal range of 10-20. The lymphocyte SI with Bacille calmette guerin (BCG) and Candida antigens illustrated 1.14 for patient comparing to 5.37 for control and also 1.19 for patient contrasting 7.07 for control, correspondingly with the normal range of 3-10, which were severely impaired. HIV serology was negative. According to clinical features and laboratory data in favor of primary immunodeficiency, parenteral immunoglubolin (IVIG) therapy was started. Microbial culture of the skin lesions was positive for coagulase negative staphylococci. Tissue Polymerase Chain Reaction (PCR) was positive for Ctomegalovirus (CMV) and Herpes Simplex Virus Type 1 (HSV-1), but negative for Herpes Simplex Virus Type 2 (HSV-2). It was also negative for acid fast bacilli and fungi. Tzanc smear on the ulcers were positive for HSV. RIDA allergy screen food panel (R-Biopharm, Darmstadt, Germany) was negative. The hematological and gastroenterological assessments were in favor of neither malignancy nor inflammatory bowel disease. Chest X-ray disclosed bilateral basal tubular bronchiectatic changes with peribronchial thickening. Spiral Computerized Tomography (CT) scan of the thorax demonstrated multiple bilateral lymph nodes in axillary and paratracheal areas with air trapping and peribronchial thickening in both lung fields. Abdominopelvic contrast enhanced CT scan showed increased mucosal thickening of the small bowel, small mesenteric lymphadenopathies, and bilateral mild hydronephrosis. A core biopsy of the lesion of the postauricular area was performed. The characteristic histological findings consisted of pseudoepitheliomatous hyperplasia and intraepithelial or dermal microabscess composed of eosinophils, along with focal ulceration, eosinohilic spongiosis and heavy infiltration of eosinophils, lymphocytes and neutrophils in dermis (figures 3 and 4). Periodic Acid-Schiff stain (PAS) was negative for fungi organisms. A perilesional skin biopsy was negative for direct immunofluorescence. According to the above mentioned clinical features9 and pathological findings the definite diagnosis of pyoderma vegetans was applied. The lesions were unresponsive to a two-month period of treatment with broad spectrum antibiotics and acyclovir following which the patient was determined to receive systemic corticosteroid therapy with unfavorable response neither to one month of oral prednisone (2 mg/kg/day) nor to a three-day course of intravenous corticosteroid pulse-therapy (30 mg/kg/day). It should be noted that a written informed consent was already obtained from the parents.

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