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Sir,
A 27 year old patient without any notion of
unprotected sexual intercourse or known inflammatory pathology, admitted for
consultation for the management of a progressive genital ulcer for more than 3
months, painful, the clinical examination had objectified the presence of a
ulcer of 4 cm taking the whole glans, surmounted by meliceric crusts in some
places and hemorrhagic by others, foul-smelling with issue of pus at pressure,
the lymph nodes were free, especially inguinal. This clinical aspect was more
in favor of a pyoderma gangrenosum with genital location (Figure 1), the rest of the clinical examination had objectified
the presence of several erythematous papulo-nodules on the level of the back,
buttock, pubis and the trunk (Figures 2
and 3) , as well as retentional lesions on the level of the face,
associated with cysts on the level of the chin and the nape of the neck (Figure 4), by pushing the
interrogation the patient reported the notion of swelling of the left ankle
joint at the age of 12.
Given this
clinical presentation and the patient's history, the diagnosis of PAPA syndrome
(pyogenic arthritis, pyoderma gangrenosum and acne) was made and the patient
was put on a combination of systemic corticosteroids (CO) and isotretinoin low
dose for 15 days then stop CO and gradual increase in retinoid doses with good
progress, scarring of genital ulceration and improvement of acne lesions.
Pyoderma
gangrenosum (PG) is a rare sterile inflammatory neutrophilic dermatosis [1]
characterized by recurrent skin ulcers with mucopurulent or hemorrhagic
exudate, which can be reactive to or associated with several pathologies [2].
PAPA syndrome
is a rare inflammatory syndrome considered as an acne syndrome, grouping
together arthritis often appeared in pre-pubertal age, pyoderma gangrenosum and
severe nodulocystic or conglobata acne [3]; its treatment is sometimes
difficult requiring high doses of retinoids and sometimes even use more severe
therapies such as anti TNF alpha [4].
In our patient, genital ulceration was the sign which pushed him to consult, and the diagnosis could be diverted towards an infectious or even tumor cause given the chronicity of the evolution, and a good clinical examination with a precise interrogation allowed to correct the diagnosis, although, one of the criteria for the diagnosis of the PAPA syndrome which is arthritis was not objectified in our patient at our clinical examination and we just based on the history and the symptoms reported by the patient to describe this involvement, it must be said that it is currently difficult to say whether old arthritis can be included in the diagnosis of PAPA syndrome, but given the context and the association of genital pyoderma gangrenosum with nodular acne lesions, we have taken into consideration this notion of arthritis.
1.
Alavi A,
French LE, Davis MD, Brassard A, Kirsner RS (2017) Pyoderma gangrenosum: An
update on pathophysiology, diagnosis and treatment. Am J Clin Dermatol 18:
355-372.
2.
Chariatte
N, Lysitsa S, Lombardi T, Samson J (2011) Pyoderma gangrenosum (1ère partie):
mise au point. Med Buccale Chir Buccale 17: 121-131.
3.
Löffler
W, Lohse P, Weihmayr T, Widenmayer W (2017) Pyogenic arthritis, pyoderma
gangrenosum and acne (PAPA) syndrome: Differential diagnosis of septic
arthritis by regular detection of exceedingly high synovial cell counts.
Infection 45: 395-402.
4.
Sood AK,
McShane DB, Googe PB, Wu EY (2019) Successful treatment of PAPA syndrome with
dual adalimumab and tacrolimus therapy. J Clin Immunol 39: 832-835.
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