Interstitial granulomatous dermatitis in an infant with juvenile
idiopathic arthritis
Authors:
- Ben Slimane Malek, Dermatology resident, Dermatology derpartment,
Charles Nicolle hospital, Tunis, Tunisia, Faculty of Medicine of
Tunis, University of Tunis El Manar, Tunis, Tunisia.
- Bacha Takwa, Dermatology professor assistant, Dermatology derpartment,
Charles Nicolle hospital, Tunis, Tunisia, Faculty of Medicine of
Tunis, University of Tunis El Manar, Tunis, Tunisia.
- Jones Meriem, Dermatology professor, Dermatology derpartment, Charles
Nicolle hospital, Tunis, Tunisia, Faculty of Medicine of Tunis,
University of Tunis El Manar, Tunis, Tunisia.
- Litaiem Nourreddine, Dermatology professor, Dermatology derpartment,
Charles Nicolle hospital, Tunis, Tunisia, Faculty of Medicine of
Tunis, University of Tunis El Manar, Tunis, Tunisia.
- Rameh Soumaya, pathology professor, Pathology derpartment, Charles
Nicolle hospital, Tunis, Tunisia, Faculty of Medicine of Tunis,
University of Tunis El Manar, Tunis, Tunisia.
- Zeglaoui Faten, Dermatology professor, Dermatology
derpartment, Charles Nicolle hospital, Tunis, Tunisia, Faculty of
Medicine of Tunis, University of Tunis El Manar, Tunis, Tunisia.
Corresponding author: Ben Slimane Malek; Charles Nicolle hospital,
Tunis, Tunisia; 50798509;benslimanemalek1990@gmail.com
- Key words : infant, juvenile idiopathic arthritis, dermatitis
- Manuscript words count: 636
- Running head: juvenile idiopathic arthritis.
- Number of figures: 2 figures
- Data availability statement: The data that support the findings of
this article are available from the corresponding author upon
reasonable request.
- Conflict of interest: none.
- Key Clinical Message: The association between infantile interstitial
granulomatous dermatitis and juvenile idiopathic arthritis is rare.
Physicians should be aware of this uncommon association in order to
set up proper investigation and treatment in these cases.
Introduction
Interstitial granulomatous dermatitis (IGD) is an uncommon entity
characterized histologically by an interstitial granulomatous
infiltrate. IGD has a polymorphic clinical presentation and is
associated with an underlying inflammatory disease. Autoimmune disease,
lymphoproliferative disorders, and medications are the main triggers
reported in literature (1). Juvenile idiopathic arthritis (JIA) is the
most common rheumatic disease of childhood. JIA is characterized by
arthritis with unknown cause, beginning before the age of 16 years old
and lasting more than 6 months (2). We describe a patient with JIA who
developed an IGD. This combination has been reported rarely in the
literature, never during the infancy.
Case report
A 15-month-old male infant, from a non-consanguineous marriage, was
referred to our department for a two weeks history of asymptomatic
erythematous plaques on the trunk and limbs. The infant had a JIA that
had been diagnosed two months earlier. He was treated with oral
non-steroidal anti-inflammatory drug. Physical examination revealed
multiple irregularly shaped, annular erythematous plaques on the trunk
(fig 1) and upper arms. Skin biopsy showed an inflammatory infiltrate of
the dermis composed mainly of histiocytes with an interstitial and
palisaded arrangement (fig 2). There were also areas of collagen
degeneration. The epidermis was normal. Alcian blue staining revealed
mucin deposition. These findings, together with the clinical
presentation, were diagnostic of IGD. Clobetasol proprionate 0.05%
ointment was prescribed for the patient to be applied once a day.
Reassessment after two weeks found a substantial improvement.
Discussion
Both adult and pediatric IGD are uncommon conditions. Infantile IGD is
extremely rare (1-3). Adult IGD primarily affects middle-aged women (1).
Akinshemoyin et al reported a female predominance in pediatric
population with mean age of 12.7 years at diagnosis and predilection to
ethnic minorities. As in our case, lesions of pediatric IGD are mostly
asymptomatic, edematous, annular and erythematous to violaceous plaques
with a predilection for extensor extremities and trunk (3). Palpable
cord-like lesions known as the rope sign are classic in adult IGD (1).
In pediatric cases, the rope sign is often absent (3). IGD is
characterized histologically by an interstitial infiltrate of
histiocytes in the reticular dermis with areas of collagen degeneration
(1). In children, it seems to be a strong association between IGD and
systemic lupus erythematous. The onset of IGD could be concomitant or
precede the diagnosis of SLE. In lupic children, IGD lesions imply a
flare of poorly controlled disease and could indicate a therapeutic
escalation (3). Melanie A et al reported an association between IGD and
uveitis in a 6-year-old girl. IGD had thus been included in the list of
granulomatous condition associated with uveitis (4). Furthermore, Magro
and Crowson described IGD in a 6-year-old girl with rheumatoid arthritis
(5).
Skin involvement is classically described among JIA extra articular
manifestations. Among the seven subgroups of JIA, skin findings occur in
psoriatic arthritis as well as systemic and polyarticular JIA (6). Tsai
HY et al reported a skin rash in 67.9% of cases of systemic onset JIA
(7). Rheumatoid nodules, guttate and plaque psoriasis are the other most
reported skin manifestation of JIA (6). An association between JIA and
Harlequin ichthyosis, although rare, remains plausible (8). Giani T et
al noted morphea in about 1% of JIA cases (9).
To the best of our knowledge, the first and unique case of a child with
IGD and IJA has been reported in 2013. It was about an 11-year-old girl
who had concomitant polyarthritis and skin lesions in the lower
extremities. Symptoms resolved after treatment by oral corticosteroids
and hydroxychloroquine (10). Our patient represents the second case of
an infantile IGD with IJA. The association could be explained by an
alteration in the balance of the immune system secondary to different
genetic and environmental factors. Physicians should be aware of this
uncommon association in order to set up proper investigation and
treatment in these cases.
Author contributions:
Drs Malek Ben Slimane and Takwa Bacha contributed to the first draft of
the manuscript. Drs Malek Ben Slimane, Takwa Bacha, Nourredine Litaiem
and Meriem Jones contributed to the literature search, analysis, and
interpretation of the data. Dr Soumaya Rameh and Dr Faten Zeglaoui
critically revised the manuscript and gave final approval. All authors
read and approved the final manuscript and agree to be finally
accountable for ensuring the integrity of and accuracy of the work.
Conflict of interest: none
Ethics statement: Consent for publication has been obtained
Funding sources: none
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Figures
Figure 1-a: Multiple irregularly shaped, annular erythematous plaques on
the trunk
Figure 1-b: Multiple irregularly shaped, annular erythematous plaques on
the upper arms
Figure 2 : Skin biopsy showed an inflammatory infiltrate of the dermis
composed mainly of histiocytes with an interstitial and palisaded
arrangement (HE; X40)