CASE REPORT:
A 45-year-old Nepalese female presented with 4 months history of
moderately itchy erythematous plaque on bilateral soles with few
discrete pustules and yellow brown crust. Three months after onset of
lesions, she developed multiple pustules on erythematous base over the
extensor aspect of left leg associated with moderate pain. These
pustules ruptured to form ulcers over 3-4 days and coalesced rapidly
over 1-2 weeks to forma a large ulcer of irregularly shape, undermined
edge, measuring 10×8 cm2 and non-foul smelling scanty serous discharge
on floor and hyperpigmented surrounding skin. There was no history of
trauma prior to the onset of lesion, significant weight loss, fever,
arthralgia or gastrointestinal symptoms and was nonsmoker.
The biopsy specimen from the edge of the leg ulcer showed a hemorrhagic
ulcer adjoining an intact epidermis with large necrotizing suppurative
dermal inflammation, leukocytoclastic vasculitis within central
suppurative zone, angiocentric and intraneural lymphocyte infiltration
and uninvolved zones showed normal hair follicles and adnexal structures
surrounded by lymphocytes (Fig 1 a, b and c). Special stains were
negative for organism, tuberculous bacilli, mucin or fibrin.
Other laboratory tests, including complete blood count, liver and renal
functions, erythrocyte sedimentation rate and chest X-ray were
unremarkable. A culture from the ulcer did not demonstrates any
pathogenic organisms. A diagnosis of classical ulcerative type of
pyoderma gangrenosum with palmoplantar pustulosis was made. She was
treated with a tapering dose of oral prednisolone (50 mg/ day), and the
ulcer healed within 1 and 1/2 months to leave an atrophic scar. Then the
patient was lost to follow up.
Four months later, she developed again multiple pustules over previously
healed atrophic scar with similar progression as in the previous episode
to form a larger ulcer. On examination, an irregular shaped ulcer
approximately 7×4 cm2 was present over lower 1/3rd of dorsum of left leg
with undermined edge and hyperpigmented border with scanty serous
discharge, granulation tissue and adherent yellowish crust on the floor.
Similar smaller ulcers approximately 1-2 cm2 were present on the
periphery of larger ulcer. The ulcer was tender with mild induration of
the borders. An atrophic plaque with hyperpigmented margin was present
on the dorso-lateral aspect of leg surrounding the ulcer (Fig. 2 a and
b). Similarly, on bilateral soles, well defined erythematous plaque with
semi adherent whitish scales with multiple adherent yellow brown crust
and few pustules was present (Fig.2 c and d). Nail examination revealed
yellowish discoloration, distal onycholysis, few pits on almost all nail
plates of bilateral hands and feet with subungual hyperkeratosis of
great toes (Fig 2 e and f).
Biopsy of the erythematous plaque on right sole showed hyperkeratosis
with parakeratosis with munro’s micro-abscess and psoriasiform
hyperplasia of epidermis (Fig 3 a, b and c).
laboratory tests including complete blood count, liver and renal
functions, erythrocyte sedimentation rate and chest X-ray were
unremarkable. The culture of wound swab was sterile. The patient was
admitted in the dermatology ward and was given 8 mg dexamethasone
intravenously for 5 days, which was later changed to oral prednisolone
80 mg per day along with wound care. The patient was also started on
tablet methotrexate 15mg per week. The ulcer showed rapid improvement
over 1 week with 30-40% improvement in pain and discharge and 15 %
improvement in the size of the ulcer (Fig. 2 g and h). The ulcer of PG
and lesions of PPP showed about 90% improvement after 4 weeks of
treatment (Fig. 2 i and j). The dose of prednisolone was tapered weekly
10mg for 4 weeks and then every 2 weeks till 10 mg. The dose of
methotrexate was increased to 20 mg per week after 6 weeks and then
tapered to 17.5 mg/week dose which she continued for 4 weeks. The
patient was unable to come for follow up and she stopped all medicines
after 20 weeks of initiating treatment. However, she visited OPD after 9
months and was on complete remission with healed scar on the site of
previous ulcer (Fig 2 k, l m and n) with significant improvement on
nails (Fig 2 o and p).