Occurrence and challenges in the management of severe chronic plaque type psoriasis in a limited resourced setting: a case report
Calypse Asangbe Ngwasiri1,2,&, Ngo-Mbaki Charmaine Kwangsa3, Martin Hongieh Abanda1, Leopold Ndemnge Aminde1,4
1Clinical Research Education, Networking & Consultancy (CRENC), Douala, Cameroon, 2Healing Touch Hospital, Muyuka, South-West Region, Cameroon, 3Banso Baptist Hospital (BBH), Kumbo, Cameroon, 4School of Public Health, Faculty of Medicine, The University of Queensland, Brisbane, Australia
Calypse Asangbe Ngwasiri, Healing Touch Hospital, Muyuka, South-West Region, Cameroon
Plaque-type psoriasis is a major dermatosis with significant effects on quality of life. Case complexity is often high in low-resourced settings such as in Africa where the incidence has been on the rise. Despite major advancements and newer therapeutic modalities over the last decade, an insight into the real-life, day to day challenges in low resourced settings reveal an interplay between the difficulty in obtaining these drugs and use of alternative traditional indigenous agents. We report the case of a 50 year old immunocompetent male who presented with chronic and extensive well demarcated plaques covered with silver-white scales occupying about 61% of his body surface area. Patient was however lost to follow up for about 8 months during which time, the lesions responded to some unknown homemade indigenous medications which was preferred to a systemic medication. Paramount importance on proper counselling and the need to retain patients in care is warranted by physicians and allied health personnel. Also, incentives aimed at subsidizing the newer systemic agents for patients in low resourced cohorts will go a long way to combat this multi-faceted disorder which is often unrecognized and under diagnosed.
Psoriasis is an immune-mediated, complex, chronic, noncontagious multisystem inflammatory disorder that affects approximately 2-3 % of people world. The disease is less common in the tropics and in dark skinned persons with an estimated prevalence of 1.3% in African Americans compared with the 2.5% in whites . There is a huge genetic predisposition for the illness which affects patients far beyond the skin; with joint involvement in about 30% of cases . No specific area limitation is ascribed to disease manifestation and multiple types have been identified, with plaque-type (discoid) psoriasis being the commonest. These plaques appear as well-demarcated, focal, raised edematous lesions covered with silver-white micaceous scales. Diagnosis is mainly clinical, however, a dermatologic biopsy is warranted in those cases where recognition remains a puzzle.
Treatment modalities are chosen based on disease severity, relevant comorbidities, patient preference (cost and convenience), efficacy and evaluation of individual patient response . In its severe form, psoriasis can have a remarkable socioeconomic influence as it impacts the affected individual and society at different levels.
Various objective and subjective assessments for severity of psoriasis have been discussed; generally relying on scoring systems for which the Psoriasis Area Severity Index score (PASI) is the most adequate available tool . Severe variants require treatment with systemic agents either as monotherapy or combination of multiple agents whose long term safety and laboratory monitoring have to be defined. Recently developed and upcoming biologics offer new therapeutic approaches but the difficulty in reliably obtaining, storing and using them coupled with their high costs is a dilemma for patients with lower socioeconomic status .
In low and middle income countries (LMIC), case complexity is often high and available resources from the individual to societal perspective (particularly primary health care) are often suboptimal. We describe herein the case of a 50 year old male in a low-resourced setting with severe chronic plaque-type psoriasis, lost to follow-up in whom management was challenging and difficult; our key objective being to emphasize on the complexity of this multi-system disease in a tropical setting and the need to retain patients in care.
Patient and observation
A 50 year old male cattle herdsman with no known personal or family
history of skin disorders presented for evaluation of skin eruptions which
had been evolving for about 2 years prior consultation. Patient was living
in the forest at the onset of these lesions and noticed they were painless,
pruritic and scaly eruptions which developed on his scalp, face and both
upper extremities. Initial thought was a supposed fungal infection for which
he took both topical and systemic terbinafine (taken liberally as an over
the counter drug) for an unknown duration to no benefit. Progress was characterized
by worsening of the lesions, with increased size of eruptions, thickening
of underlying skin and further involvement of the entire trunk. However,
the lower limbs, palms and soles were unaffected. About two weeks prior
consultation to our service, he developed red itchy eyes, generalized polyarthralgia,
frequent chills and diminished interest in almost all daily activities.
He denied previous history of tuberculosis, alcohol consumption and exposure
to toxic chemicals.
On physical examination, the patient looked well and comfortable with vital signs
within normal limits. He had corneal and conjunctival hyperemia with no
sign of oedema or cicatricial ectropion. There were well-defined inflammatory
plaques covering about 61% of his total body surface area (TBSA) with associated
scaling, lichenification and accentuated skin markings (Figure
1). Superficial exfoliation of the face was noted and the lesions
were discrete, firm, and silver grey, involving the entire integument but
for the lower limbs, palms and soles (Figure
Nail examination revealed no area of dystrophy or pitting. Examination of the
cardiovascular and respiratory systems revealed no abnormality likewise joint
examination for any tenderness or edema. Blood and tissue cultures were negative
for bacteria, fungi and atypical mycobacteria. Serological tests for HIV and
Syphilis were also negative likewise skin snip for filaria. His baseline lipid
profile was normal so too was work up for Hepatitis B and C.
Punch biopsy of skin was consistent with psoriasis and focal suppurative folliculitis
(Microscopic Description: Sections reveal skin with acanthosis, elongated retia,
hyperkeratosis with parakeratosis, focal neutrophilic infiltration of the epidermis,
loss of granular layer, focal spongiosis and suprapapillary thinning. One fragment
showed a suppurative folliculitis with dermal abscess. Dermis with inflammatory
infiltrates that include neutrophils is seen in other areas. PAS and Gram stain
failed to recover fungal and bacterial organisms). The diagnosis of severe chronic
plaque type psoriasis was made based on his psoriasis severity index (PASI) score
of 13.6 (> 12) and a BSA 61% (> 10%) (Table
). Owing to our low income resource, a topical steroid (Betamethasone
0.05% topical), topical moisturizer (petroleum jelly), systemic therapy (methotrexate:
10mg weekly initially) and daily sun exposure were the treatment modalities employed.
Fluorometholone Acetate was also added to aid with the ocular inflammation whilst
psychiatric evaluation was sort. Patient was however lost to follow up and was
seen about 8 months later with complete resolution of plaques following the repeated
ingestion of some unknown homemade indigenous medications (Figure
B) PASI 3.8, which he took in preference to medical treatment.
Severe flares of psoriasis can be induced by systemic and environmental factors like life stress events, infections and medications. The most severe forms closely resemble other forms of erythroderma like atopic dermatitis, seborrheic dermatitis, cutaneous T cell lymphoma, mycosis fungoides and pityriasis rubra piliaris both clinically and histologically. Often patient's history and subtle clues in the clinical presentation with skin biopsy specimen aid in the diagnosis in selected situations as was with ours.
Although familial incidence is high with adult onset psoriasis (37% having first degree affected family members) , it was difficult to ascertain in this patient if there was indeed any family history of psoriasis. The patient had areas of indurated plaques and associated silvery scale on his trunk that would be much more consistent with psoriasis than atopic dermatitis. Furthermore the negative blood tests and the cutaneous histology together ruled out drug induced, atopic, infectious or seborrheic erythroderma. Also, obtaining blood for flow cytometry and Sézary cell count (to assess the potential of blood involvement in cutaneous T cell lymphoma or Sézary syndrome) would've been useful but the absence of atypical lymphocytes on the biopsy specimen made the primary clue for the diagnosis of cutaneous T cell lymphoma unlikely. Pityriasis rubra piliaris is a close differential diagnosis which not uncommonly presents as a diffuse erythroderma with psoriatic-like scales. However, the lack of some clinical distinctive features, such as islands of pale skin (skip lesions), orange-red scaling erythema, and palmoplantar yellowish keratoderma makes psoriasis the more likely diagnosis in our patient.
Ocular manifestations are not uncommon in psoriasis with blepharitis being the most common finding. Other manifestations include ectropion and trichiasis, conjunctivitis and conjunctival hyperemia, corneal dryness/melt . Conjunctivitis and conjunctival hyperemia were the identified features in the patient.
Once diagnosis is made, treatment is based on the severity of the skin condition which is defined by various objective and subjective assessments for which the PASI is the most adequate instrument available to evaluate severity in plaque type psoriasis .
Also there has been a general consensus that involvement of the face, palms/soles or disease that is disabling should be considered as severe. Our patient had a PASI score of 13.6 and 61% BSA affected which defines severe psoriasis, qualifying him for treatment with systemic agents. Traditional systemic agents like retinoids, antimetabolites (methotrexate), immunomodulators (cyclosporine) and TNF inhibitors (etanercept) all improve the clinical symptoms in psoriasis by acting on the main pathways of the psoriatic lesion which can either be used alone or in combination (daily sun exposure, sea bathing, topical moisturizers and relaxation) but concerns lie with their long term safety and efficacy [7
Biologics (like ustekinumab, secukinumab), are the most recent and effective medications for psoriasis but present a big challenge to patients because aside from the difficulty in reliably obtaining, storing and using them, they are very expensive (30,000-80,000 USD/year) and most insurance companies tend not to approved [5
]. In sub-Saharan Africa where about 47% of the population live on less than 0.25 USD/day [8
] and where cost of treatment is essentially out of pocket, it represents a significant problem for both physicians and patients.
The folic acid antagonist methotrexate (MTX) which is relatively cheaper than at least some of the biologic agents was readily available in our context. It is also effective for the treatment of psoriatic arthritis and nail arthritis. Initial thoughts on the mechanism of action centered around the antiproliferative effects on DNA synthesis in epidermal cells; subsequent evidence supports the concept that it is the immunosuppressive effects of methotrexate on T-cells that controls psoriasis [9
]. It was started at an initial weekly dose of 10mg together with adjunctive therapy (phototherapy, sea bathing). Folic acid 1mg daily was also given to protect against common side effects like stomatitis. The patient however deferred medical treatment and was lost to follow-up, preferring some homemade indigenous traditional medication whose beneficial effects resulted in resolution of eruptions about 8 months later. No information about the drug name nor active ingredient could be got. This wasn't entirely surprising as it is well established that some indigenous methods like wet cupping Hijamah and some African traditional plants like Kigelia Africana have been shown to be beneficial in the treatment of psoriasis.
In our setting where physician inertia and lack of relatively well-
equipped dermatology units are common, the appropriate management
of severe skin conditions as such remains challenging and difficult.
patients' perceptions of themselves and this can potentially initiate
or exacerbate psychological disorders such as depression, thus
proper counselling, empathy and retaining patients in care is absolute.
A recent study
skin diseases among elderly patients attending a dermatology unit
in Tanzania revealed an overall prevalence of psoriasis to be 7.7%,
which is relatively
higher than the worldwide prevalence of 2.2-3.9% .
This could serve as a wakeup call to raise awareness of the complexity
of this multifaceted disease especially as most of the milder cases
undiagnosed or discovered incidentally. The need for early diagnosis,
appropriate referral and comprehensive state-of-the-art approach
in the management of
patients with psoriasis cannot be overemphasized.
The authors declare no competing interests.
Calypse Asangbe Ngwasiri managed the patient, wrote the manuscript and did the review of literature; Ngo-Mbaki Charmaine Kwangsa managed the patient; Martin Hongieh Abanda: proofreading; Leopold Ndemnge Aminde: critical revision to the manuscript. All authors have read and approved the final copy of this manuscript.
Table and figures
Table 1: psoriasis area and severity index (PASI)
Figure 1: focal raised inflamed edematous lesions covered with silvery-white micaceous scales
Figure 2: (A) superficial exfoliation of the face and lesions were discrete, firm, and silver grey; (B) resolution of skin eruptions
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