A pyoderma gangrenous-like cutaneous leishmaniasis in a Libyan woman with rheumatoid arthritis: a case report
Al‑Dwibe et al. BMC Res Notes
A pyoderma gangrenous‑like cutaneous leishmaniasis in a Libyan woman with rheumatoid arthritis: a case report
Hamida Al‑Dwibe 3
Ahmad Amro 0 2
Aisha Gashout 1
Ali El‑Zurghany 3
Said El‑zubi 3
Mohamed El‑Hashme 3
Omar Hamarsheh 4
Mokhtar Maree 3
0 Faculty of Pharmacy, Al‐ Quds University , Jerusalem, Palestine
1 Pathol‐ ogy Department, Faculty of Medical Technology, University of Tripoli , Tripoli , Libya
2 Faculty of Pharmacy, Al‐ Quds University , Jerusalem, Palestine
3 Dermatology Department, Faculty of Medicine, University of Tripoli , Tripoli , Libya
4 Department of Biological Sciences, Faculty of Science & Technology, Al‐ Quds University , Jerusalem, Palestine
Background: Several case reports describe diseases presenting with skin ulcerations, which resemble pyoderma gangrenosum especially in immune‑ compromised patients, often proven on further workup, to have an infective or malignant etiology. However, treatment of pyoderma gangrenosum by systemic steroids or other immunosuppressive agents may worsen the condition. Case presentation: We report here, a 45 year‑ old Libyan woman with rheumatoid arthritis on low dose steroids with pyoderma gangrenosum‑ like skin lesions and positive pathergy. Slit-smear was positive for Leishmania amastigotes and histopathological examination confirmed the diagnosis of cutaneous leishmaniasis. The lesions healed completely by parenteral sodium stibogluconate (Pentostam) 600 mg daily. Conclusion: We report for the first time, a rare and unusual presentation of pyoderma gangrenosum like‑ cutaneous leishmaniasis in a patient with rheumatoid arthritis. Atypical cutaneous leishmaniasis should not be ruled out in the differential diagnosis of unresponsive skin diseases, with slit/smear and a skin biopsy is required.
Leishmania; Pyoderma gangrenous; Cutaneous leishmaniasis; Rheumatoid arthritis
Cutaneous leishmaniasis (CL) is a protozoan skin
infection caused by various species of Leishmania parasites
and transmitted by correspondent species of sand flies.
CL is highly endemic in Libya especially after the armed
conflict that outbreak in the country [
]. CL has a wide
clinical spectrum, presenting in different clinical forms
of the disease depending particularly on host immune
response rather than on the parasite species [
However, there are some clinical manifestations that are more
prevalent in one host than in another [
clinical variants of CL include mucocutaneous
leishmaniasis (MCL), diffuse cutaneous leishmaniasis (DCL),
disseminated cutaneous leishmaniasis (DCL), and
leishmaniasis recidivans (LR). Rare forms of CL were reported
with sporotrichoid, psoriasiform, and zosteriform [
]. In immune-compromised patients, skin ulcerations
are often associated with infections or malignancy [
However, treatment of pyoderma gangrenous by systemic
steroids or other immunosuppressive drugs proved to
worse the condition. Herein, we report for the first time,
a rare and unusual presentation of PG like CL in a patient
with rheumatoid arthritis (RhA).
A 46-year-old, women from Tawirgha, Libya was referred
to the Leishmaniasis Clinic, Tripoli Central Hospital. She
was admitted to the clinic with multiple papules,
pustules, ulcerated nodules, and painful ulcerative lesions
over upper and lower extremities for 2 years. The lesions
started as painful papules and pustules, which gradually
enlarged in size, and eventually ulcerated with pus
discharge. Her worsened conditions, prompted her to seek
advanced medical care. Two years later, the patient had
been admitted twice to the hospital in her local village
for the same problem. She was treated with topical and
systemic antibiotics without any improvement, so she
was referred to the Leishmaniasis Clinic in Tripoli
Central Hospital. The patient had suffered from rheumatoid
arthritis for 22 years, hypertension, and diabetes
mellitus and has been prescribed a low dose of prednisolone
(20 mg/days) for 20-years, beta-1 selective adrenergic
antagonist, and an oral antihyperglycaemic medication.
No previous history of trauma or insect bites were
reported by the patient. However, she had a history of
CL in the buttocks, which was treated with intra-lesional
injections of pentostam.
Physical examination revealed good general condition,
a red puffy face with telangiectasia over both checks and
mild lower limb edema. On cutaneous examination, the
patient was found to have multiple erythematous
papules, pustules, and ulcerated nodules as well as painful
well defined erythematous ulcers of varying sizes with
raised irregular indurated margins on both forearms
(Fig. 1a) and multiple painful punched out ulcers on
lower limbs (Fig. 1b). The base of ulcers contained
yellowish exudate and necrotic yellowish slough (Fig. 1a,
b). The ulcers on extensor and flexore surfaces on the
right leg was associated with swelling of the leg with
surrounding skin inflammation (Fig. 1b). In addition, large
hypo pigmented atrophic scare surrounded by a rim of
hyper-pigmentation over the right buttock (healed CL
lesions), and pustules at site of injections were observed
(pathergy) (Fig. 1c).
Skin examination revealed ulcers presenting as PG, or
as vasculitic ulcers secondary to Rh.A or an unusual
variant of CL. Routine hematological investigations revealed
elevated white blood cell count (12.3 × 109/l),
hemoglobin level of 11.7 g/dl, platelet counts of 230 × 109/l,
ESR 15/h, CRP of 54 mg/l, FBS of 135 mg/dl and high
levels of transaminases (GGT 528 U/l, GPT 80 U/l).
Renal function tests, rheumatoid factor, and cortisol
were at normal levels. Ultrasonography examination of
the abdomen revealed enlarged liver with marked fatty
changes. Serologic tests for HIV and viral hepatitis were
negative. Chest radiograph, ECG, and Echo were normal.
A skin biopsy obtained from the lesions revealed
hyperkeratosis, focal parakeratosis, acanthosis, spongiosis, and
exocytosis. Dermis showed heavy diffuse granulomatous
infiltrate composed of epithelioid cells, and lymphocytes
admixed with plasma cells, neutrophils, and mast cells
with some extravasated RBCs (Fig. 2a). A perivascular
chronic inflammatory cell infiltrate with extravasated
RBCs, nuclear dust, thickening of endothelium and
fibrinoid necrosis of few blood vessels and few Leishmania
parasites was seen especially in biopsies obtained from
leg ulcers. However, biopsies taken from the
papulo–pustular lesions showed many Leishmania parasites inside
and outside the macrophages (Fig. 2b). Molecular
identification of the causative Leishmania spices was not done.
The patient was treated with systemic antibiotics, and
sodium stibogluconate (Pentostam) (600 mg daily-I.M)
was started after reduction of the transaminases enzymes
by stopping all medications that have been used by the
patient for long time. Complete healing of lesions was
achieved after 15 days, except a small erythematous
papule on the left forearm which was treated by cryotherapy.
After 2 years follow up, no relapse was noted. The
important milestones related to diagnosis, interventions and
follow up of this case are listed in Additional file 1.
Discussion and conclusions
Cutaneous leishmaniasis has been endemic in
Northwestern regions of Libya for a long time. The incidence
of this infection is rising since the year of 1971, and new
foci have been reported recently [
]. Clinically, CL can
resemble many skin diseases such as bacterial skin
infections, fungal skin infections, mycobacterial infections,
eczema, sarcoidosis, insect bites, and malignancies like
basal and squamous cell carcinomas, and can be
misdiagnosed as other diseases [
]. When the ulcerating
lesion of CL is located on the extremities especially legs,
other diseases like pyoderma gangrenosum, a typical
mycobacterial infection, venous, and arterial ulcers must
be considered in the differential diagnosis . In
addition, venous, infective, and inflammatory leg ulcers such
as vasculitis or pyoderma gangrenosum may develop
in patients with rheumatoid arthritis. These ulcers are
often painful, difficult to heal, and may last for years as
described in this case.
The clinical diagnosis of CL was suspected since this
patient came from an endemic area, having multiple
lesions on exposed parts of body, not responding to
treatment with antibiotics, had positive personal and
family history of CL which are confirmed as risk
factors for CL [
] as well as confirmation of the case
by a slit–skin smear and histopathological
examination. The reason for this rare unusual clinical type of
Pyoderma gangrenosum like CL is unknown and raises
speculation and assumptions about the host parasite
relationship and the capacity of Leishmania parasites
to modulate the host immune response [
infection by specific Leishmania strains, and an altered
host immune response caused by systemic
administration of steroid drugs may have a role for persistence
of the lesions. However, patients on systemic
corticosteroids are likely to be immune-compromised with
increased susceptibility to infections, particularly
with intra-cellular microorganisms, which may lead to
the development of unusual types of leishmaniasis as
explained by the blockade of cytokine expression by
glucocorticoids released from T lymphocyte cells [
Pentavalent antimony compounds such as sodium
stiboglyconate and meglumine antimoniate still remain
the first choice for the treatment of CL [
2–4, 6, 7, 18
Sodium stiboglyconate 600 mg/day intramuscularly for
15 days was sufficient to completely heal the lesions in
Atypical CL should not be ruled out in the differential
diagnosis of unresponsive skin diseases, with slit/smear
and a skin biopsy being required. Moreover, history of
previous exposure to sand fly bites or previous
Leishmania infection may help in the diagnosis. According
to the author’s knowledge, this case represented the
first reported case of CL presenting as pyoderma
gangrenosum like lesions in Libya.
Additional file 1. Time line: Important milestones related to diagnosis,
interventions and follow up of the case.
CL: cutaneous leishmaniasis; DCL: diffuse cutaneous leishmaniasis; LR:
leishmaniasis recidivans; MCL: mucocutaneous leishmaniasis; PG: pyoderma
HD, AA, OH and AG designed the study. HD, AE, SE, ME and MM did the
clinical evaluation, HD did the histological examination. HD and AG treated
the patient and did the follow up. HD, OH and AA analysed the data and
supervised the concept and design of the manuscript. All authors read and
approved the final manuscript.
The authors declare that they have no competing interests.
Availability of data and materials
The data sets used and/or analysed during the current study are available
from the corresponding author upon reasonable request.
Consent for publication
Written informed consent was signed by the patient for participating in this
study and for publication of this case report and any accompanying images.
Ethics approval and consent to participate
The authors declare that no funding support was obtained for this study.
Springer Nature remains neutral with regard to jurisdictional claims in pub‑
lished maps and institutional affiliations.
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