Pyoderma gangrenosum: A clinico-epidemiological study.

BACKGROUND: Pyoderma gangrenosum is a neutrophilic dermatosis of unknown etiology, with inconstant systemic associations and a variable prognosis. AIMS: To study the clinical features and systemic associations of pyoderma gangrenosum and its response to treatment. METHODS: All patients diagnosed to have pyoderma gangrenosum at the dermatology department of the Government Medical College, Kozhikode, from January 01, 2005 to December 31, 2014 were included in this prospective study. RESULTS: During the 10-year study period, 61 patients were diagnosed to have pyoderma gangrenosum. A male predilection was noted. The most common clinical type was ulcerative pyoderma gangrenosum (90.2%). More than 60% of patients had lesions confined to the legs; 78.7% had a single lesion and 27.9% had systemic associations. Most patients required systemic steroids. Patients with disease resistant to steroid therapy were treated with intravenous immunoglobulin G and split-thickness skin grafts under immunosuppression induced by dexamethasone pulse therapy. All except one patient attained complete disease resolution. LIMITATIONS: The main limitation of our study was the small sample size. CONCLUSIONS: The male predilection documented by us was contrary to most previous studies. We found split-thickness skin graft to be a useful option in resistant cases. More prospective studies may enable the formulation of better diagnostic criteria for pyoderma gangrenosum and improve its management.

Cutaneous manifestations of chikungunya during a recent epidemic in Calicut, north Kerala, south India.

BACKGROUND: There was a recent epidemic of chikungunya (CKG) in Calicut and other northern districts of Kerala, South India, affecting thousands of people. AIMS: To study the cutaneous manifestations of CKG and to have a serological and histopathological correlation. METHODS: A total of 162 patients (63 males and 99 females) with cutaneous manifestations of CKG were enrolled in the study and serological confirmation was done with capture IgM ELISA for CKG. Skin biopsy was done in all representative cases. RESULTS: Cutaneous manifestations were found more in females. There were 23 children, the youngest being 39 days old. Generalized erythematous macular rash was the most common finding. Vesicles and bullae were also common especially in infants. Localized erythema of the nose and pinnae, erythema and swelling of the pre existing scars and striae and toxic epidermal necrolysis-like lesions sparing mucosae were the other interesting findings. Different types of pigmentation were observed with a striking nose pigmentation in a large number of patients, by looking at which even a retrospective diagnosis of CKG could be made. Hence we suggest this peculiar pigmentation may be called "chik sign". There was flare up of existing dermatoses like psoriasis, lichen planus and unmasking of Hansen's disease with type 1 reaction. Serological tests were positive in 97%. Some hitherto unreported histopathologic findings like melanophages in the erythematous rashes were observed. CONCLUSION: A spectrum of cutaneous manifestations of CKG with a wide variety of unusual presentations with confirmed serological and histopathological evidence was encountered.

Poland syndrome (anomaly) with congenital hemangioma: a new association.

Unilateral defect of pectoral muscle and ipsilateral syndactyly constitute Poland syndrome. Absence or hypoplasia of the breast and nipple, axillary hair loss and dermatoglyphic abnormalities have also been reported in this syndrome. The primary defect could be in the development of the proximal subclavian artery with early deficit of blood flow to the distal limb and the pectoral region, resulting in partial loss of tissue in those regions. However, the association of congenital hemangioma with Poland sequence has not been observed so far. Such an association is being reported here in a 1-year-old infant, second-born of nonconsanguineous parents, who also had polydactyly instead of the documented syndactyly.

Focal dermal hypoplasia (Goltz syndrome).

A 7-year-old girl born of non-consanguineous marriage was evaluated for facial dysmorphism. She had multiple skeletal anomalies like hypoplasia of the right mandible, narrow nasal bridge with broad tip and unilateral notching of the right ala nasi, concomitant squint and low set ears. She also had generalized hypopigmented, atrophic linear macules, multiple papillomas, fat herniations, umbilical hernia, hypoplastic nails, cicatricial alopecia, mild mental retardation, 'lobster-claw' hand and osteopathia striata of long bones, pointing to a diagnosis of Goltz syndrome. The unusual features noted were absence of the left first rib and aortic regurgitation.

Goldenhar syndrome with unusual features.

We report two cases of Goldenhar syndrome in whom bilateral ocular and auricular changes were observed. One patient also had hypertelorism, macrophthalmia and bulbous nose.

De sanctis cacchione syndrome.

A case of de Sanctis Cacchine syndrome in a female infant is presented.