KEY WORD
S: Pru
rigo pigm
entosa, Adult Onset Still’s
Disease, S
til
l’s
ash.KEY WORDS: Prurigo pigmentosa, Adult Onset Still’s
Disease, Still’sash.
Abbreviations: Adult onset still’s disease (AOSD). Prurigo Pigmentosa (PP).
Pak J Med Sci October - December 2007 (Part-I) Vol. 23 No. 5 792-797
1. Tarek Abdel Hamed Mostafa Dowod,
Specialist, Dept. of Medicine,
2. Mohammed El-Sayed Hanafy Khalel,
Specialist, Dept. of Dermatology,
3. Magdy Abas Ali Mohammed,
Registrar, Dept. of Medicine,
4. Maha Abdel Wahab Mohamed El Sharawy,
Lecture of Pathology, Dept. of Pathology,
Ain Shames University, EGYPT.
5. Sara Jassim Al-Ghabandi,
Trainee, Dept. of Medicine,
6. Anwaar Yahya Al-Sumait
Trainee, Dept. of Medicine,
1-3,5,6: Al-Adan Hospital, Kuwait.
Correspondence
Dr. Tarek Abdel Hamed Mostafa Dowod,
Al-Adan Hospital, Ministry of Health,
Fahaheel, P.O Box: 47854, Kuwait.
E-mail: doriya70@hotmail.com
* Received for Publication: August 9, 2007
* Accepted: September 15, 2007
INTRODUCTION
Both prurigo pigmentosa (PP) and adult onset still’s disease (AOSD) independently are uncommon diseases. Expectedly the association of PP and AOSD is extremely rare.
PP is a rare dermatosis with unknown etiology.
1 It is seen most commonly among young adult Japanese females.2 Clinically, it presents itself as pruritic urticarial papules, papulo-vesicular and vesicles arranged in a reticulate pattern and distributed symmetrically on the face, neck, back, chest and extremities.3 Lesions involute in a matter of days leaving behind net like pigmentation. Exacerbations and recurrences are the rule.4AOSD is an inflammatory disorder used to describe a series of adult patients who did not fulfill criteria for classic rheumatoid arthritis but had features similar to children with systemic rheumatoid arthritis.
5,6 To establish a diagnosis of AOSD requires the presence of certain major or minor criteria or a combination of both, and absence of certain exclusions.7Prurigo pigmentosa has been described in association with ketosis related to diabetes mellitus, anorexia nervosa and strict diet,
8 pregnancy,9 primary biliary cirrhosis and Sjorgen syndrome.10 Kahori T, et al described PP like lesions in a patient with AOSD in addition to the typical still’s rash and it is the only case report that exists in English literature.11We report a second case of prurigo pigmentosa in association with adult onset still’s disease in absence of the typical still’s rash. As the clinicians and most of the dermatologist are unaware of this unusual association of PP and AOSD, we decided to report this case and to shed more light on both conditions.
CASE REPORT
A 26 - year old Nepali female, a mother of one child with no significant past medical history presented with a ten-day history of daily spiking fever, chills, arthralgias, myalgias and pruritic skin eruption.
On examination, she looked pale, febrile (temperature 39.2ºC), had non-suppurative pharyngitis and generalized lymphadenopathy. The lymph nodes were slightly tender, small and discrete. The cardio-respiratory, abdominal and neurological systems were normal. There was no evidence of active arthritis. The skin eruption was in the form of pruritic papular eruption arranged in a reticulate pattern and distributed symmetrically on the face, neck, trunk and legs (Figs-1a, 1b, 1c).
Investigations: CBC: WBC 13.95 X 10
9/l, differential count showed neutrophilic leukocytosis (11.65 X 10 vWww Datingbikini O Girls En Videos 2006 08 Dating Bikini Adult onset still’s disease and prurigo pigmentosa: An unusual association and review of literature u Dating hWww Datingbikini O Girls En Videos 2006 08 Dating Bikini Adult onset still’s disease and prurigo pigmentosa: An unusual association and review of literature l g Music Dating