Panniculitis in Children Moulonguet, Isabelle; Fraitag, Sylvie
Dermatopathology,
08/2021, Volume:
8, Issue:
3
Journal Article
Peer reviewed
Open access
Panniculitides form a heterogenous group of inflammatory diseases that involve the subcutaneous adipose tissue. These disorders are rare in children and have many aetiologies. As in adults, the ...panniculitis can be the primary process in a systemic disorder or a secondary process that results from infection, trauma or exposure to medication. Some types of panniculitis are seen more commonly or exclusively in children, and several new entities have been described in recent years. Most types of panniculitis have the same clinical presentation (regardless of the aetiology), with tender, erythematous subcutaneous nodules. Although the patient’s age and the lesion site provide information, a histopathological assessment is sometimes required for a definitive diagnosis and classification of the disorder. In children, most panniculitides are lobular. At present, autoimmune inflammatory diseases and primary immunodeficiencies have been better characterised; panniculitis can be the presenting symptom in some of these settings. Unexplained panniculitis in a young child should prompt a detailed screen for monogenic immune disorders because the latter usually manifest themselves early in life. Here, we review forms of panniculitis that occur primarily in children, with a focus on newly described entities.
Background A rare variant of mycosis fungoides (MF), syringotropic MF (STMF) is characterized by a particular tropism of the lymphocytic infiltrate for the eccrine structures, and included in the ...follicular subtype of MF in the World Health Organization–European Organization for Research and Treatment of Cancer classification of cutaneous lymphomas. Objective We sought to determine the clinicopathologic features and disease course of patients with STMF. Methods A retrospective study was conducted to identify patients with STMF from 1998 to 2013. Results Nineteen patients were included: 15 men and 4 women, mean age 55 years (range, 24-86). Most had multiple lesions (n = 16, 84%) with associated alopecia (n = 12, 63%) and/or punctuated aspect (n = 12, 63%). Palms or soles were involved in 10 cases (53%). Folliculotropism was found in 13 cases (68%). After a median follow-up of 70 months (range, 2-140), 3 patients died, 1 from disease-related death. The 5-year overall and disease-specific survival were 100%. The disease-specific survival was significantly higher than in 54 patients with folliculotropic MF without syringotropism (5-year disease-specific survival, 74%; 95% confidence interval, 58%-94%, P = .02). Limitations Retrospective setting is a limitation. Conclusions In the spectrum of adnexotropic MF, STMF appears as a distinct entity from follicular MF, with peculiar clinical characteristics and natural history.
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GEOZS, IJS, IMTLJ, KILJ, KISLJ, NUK, OILJ, PNG, SAZU, SBCE, SBJE, UL, UM, UPCLJ, UPUK
Introduction
Keratoacanthomas (KA) are common cutaneous skin tumors originating from the hair follicles. Unlike squamous cell carcinoma, KA can regress spontaneously and have a benign evolution. ...Solitary KA is the most common form but familial multiple KA (Ferguson–Smith type), genetically predisposed KA (such as in xeroderma pigmentosum, or Muir–Torre syndrome), or sporadic multiple eruptive KA (Grzybowski type) have been described. Generalized eruptive KA of Grzybowski (GEKA) is a rare condition (around 40 reported cases). The pathophysiology is still unclear. Human papillomavirus (HPV) has been detected in sporadic KA but the presence of
HPV39
has never been reported, to our knowledge, in GEKA.
Case Report
GEKA in an 80-year-old woman was successfully treated with acitretin (0.5 mg/kg/day) combined with surgical removal of the largest lesions. Treatment was well tolerated and led to decreased pruritus and tumor regression within 6 months. The presence of
HPV39
was detected in a lesion by polymerase chain reaction and Sanger sequencing. No genetic alteration was found, in particular in the genes usually altered in squamous cell carcinoma (including
NOTCH1
,
NOTCH2
,
CDKN2A
,
TP53
).
Conclusion
We report a case of GEKA associated with the presence of
HPV39
and the successful use of acitretin combined with surgical removal of the larger lesions.
A new resorbable filler, Novabel, became commercially available in January 2010 in France. A 52-year-old general practitioner injected 0.1 cm3 of a 1 cm3 alginate solution (Novabel) into the deep ...dermis of her left arm to test it before using it on her patients. Ten days later, she observed a small pink nodule at the injection site forming secondary a bluish papule. A biopsy was performed 2 months after the injection. Histopathology showed a granulomatous reaction involving the deep dermis and the subcutaneous fat. The inflammatory reaction surrounded a nonpolarizing exogenous material consisting of slightly bluish deposits of variable size and shape, some of which were well delineated, others with a blurred or spiky perimeter, frequently showing retraction in a clear vacuole. The papule regressed, resolving completely 5 months after the injection. Novabel is a totally new type of aesthetic injectable resorbable filler consisting of a purified polysaccharide, alginate, which is extracted from crusted brown algae. No side effects have been reported to date and we describe here a first granulomatous reaction after filler injection which has a distinctive appearance in keeping with the histopathological findings.
Extra Mammary Paget disease Moulonguet, Isabelle; Plantier, Francoise
Annales de pathologie
42, Issue:
1
Journal Article
Peer reviewed
Extramammary Paget's disease is a rare malignancy. The underlying cause of VPD remains not well understood. We provide an overview of the histopathology of vulvar Paget's disease, with or without ...invasion.
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