Rare case presentation EDS

1. Unusual presentation of
some dermatology cases
Presented by
Dr Maha Assem Fahmy
Dermatology specialist. Mediclinic Al-Ain
Master of dermatology and andrology
Ain-shams university
Diploma of laser and its application in dermatology
NILES, Cairo university
Board certification in aesthetic medicine AAAM
Member of American academy of aesthetic medicine

2. This presentation was prepared in
compliance with all ethical and
confidentiality guidelines and principles.
Patient consent for biopsy and
photography was obtained at the time of
care.
Statement of ethics:

3. Case 1

4. Medical
History
4-yrs-old male child presented
with perianal lesions 3m
duration progressive course.

5. DD:
 Condyloma accuminata
 Condyloma lata
Child sexual abuse????

6. Biopsy was performed for
medico-legal issues

7. H/P: (200x,400x)
Dermis was infiltrated by sheets of dyscohesive round cells with reniform nuclei
and nuclear in-folding. Also the infiltrate showed eosinophils, lymphocytes, and
plasma cells

8. Pan- CK (-ve):

9. CD1 a, S100 protein

10. Langerhans cell
Histiocytosis

11.  LCH is a rare disease of unknown etiology
caused by clonal proliferation of
Langerhans cells.
 LCH its childhood incidence is1/200,000
the incidence in adults is extremely rare
1/2 million.
Oguzkurt P, Sarialioglu F, Ezer SS, Ince E, Kayaselcuk F, Hicsonmez A. An uncommon presenting
sign of Langerhans cell histiocytosis: focal perianal lesions without systemic involvement. J Pediatr
Hematol Oncol 2008;30:915e6.
Y. Dere et al. Dermatologica Sinica 34 (2016) 99e101

12.  LCH may present with solitary
lesions in the bone or visceral
organs, in addition to the
involvement of multiple organ
systems.
Tinsa F, Brini I, Kharfi M, Mrad K, Boussetta K, Bousnina S. Perianal presentation of
Langerhans cell histiocytosis in children. Gastroenterol Clin Biol 2010;34:95e7.

13. Imaging and blood
work up were done
to roll out systemic
involvement
All results were
normal

14. Treatment:
Imiqumode 5% was used for 2
weeks results in complete cure
of the lesions.

15.  LCH involving the
perianal skin is rare.
 Searching the Literature
(17) cases were reported.

16. Treatment
Age, Sex
Study
THAL, TAC
34y, M
Mango et al.
THAL
20y, M
Shakoei et al.
MTX
33y, M
Abdou AG,
Taie DM.
Shakoei S, Shahidi-Dadras M, Saeedi M, Ayatollahi A. Langerhans cell histiocytosis: an uncommon presentation, successfully
treated by thalidomide. Indian J DermatolVenereol Leprol. 2011;77(5):587-90. PMid:21860158. http://dx.doi.org/10.4103/0378-
6323.84064.
Mango JCC, D’Almedia DG, Magalhaes JP, et al. Perianal Langerhans cell histiocytosis case report and review of literature. Rev
Bras Coloproctol. 2007;27:1.
Abdou AG, Taie DM. Perianal Langerhans cell histiocytosis: a rare presentation in an adult male. Autops Case Rep [Internet].
2017;7(3):38-43. http://dx.doi.org/10.4322/acr.2017.028

17. Case 2

18. 42-yr gentle man presented firm
non-tender 2-3cm skin colored
nodule freely movable with 3 m
duration slightly increasing in size
affecting left check.
Medical History:

20. DD:
 Dermatofibroma (unusual site)
 Lipoma (other mesenchymal tumors)
 Lymphocytoma cutis

21.  Dense dermal infiltrate of large cells with
indistinct cell boarders and foamy cytoplasm
the over lying epidermis shows
unremarkable changes.
 Immunohistochemistry stains:
The majority of the infiltrate shows CD3, CD20,
CD30, CD68 positive cell, CD23 highlights a
couple of small dendritic cells. BCL-6 and
Mum-1were positive, Ki-67 demonstrate high
proliferative index
H/P:

23. Diffuse Large B-cell
lymphoma
(Leg-type)

24. Swerdlow SH, Campo E, PileriSA. The 2016 revision of world health organization classification of lymphoid
neoplasm. Blood 2016: 127(20):2375-2390.
National institutes of health, National cancer institute. Surveillance, epidemiology: on Hodgkin lymphoma.
Accessed September 2020 , https://seer.cancer.gov/csr/1975_2016/results_merged/sect_19_nhl.pdf

25. PET/CT scan was done
 Focal intense avid in the SC
lesion and also at the left check
involving the parotid gland.
 Few mild avid left cervical level II

26. Complete removal of the
lesion with ipsilateral parotid
gland.
skin and SC defect was
treated with direct skin flap.
Management:

27. Discrepancy between the
preoperative and postoperative
histopathological diagnosis.

28. Swerdlow SH, Campo E, PileriSA. The 2016 revision of world health organization classification of lymphoid
neoplasm. Blood 2016: 127(20):2375-2390.
National institutes of health, National cancer institute. Surveillance, epidemiology: on Hodgkin lymphoma.
Accessed September 2020 , https://seer.cancer.gov/csr/1975_2016/results_merged/sect_19_nhl.pdf

29. H/P:( from the removed lesions )

30. Bradford PT, Devesa SS, Anderson WF, Toro JR: Cutaneous lymphoma incidence patterns in the
Unites States: A population-based study of 3884 cases. Blood, 2009; 113(21): 5064–73
PCFCL accounts for approximately
30% of all cases of BCL with an
estimated annual incidence of 0.9
new cases per 1,000,000
population.

31. King M.L. et al.:Transformation of PCFCL to DLBCL. Am J Case Rep, 2019; 20: 1273-1278
PCFCL is a low-grade B-cell (NHL) that has an
indolent clinical course with a 80-95% 5year survival
rate.
However, transformation of to high-grade BCL was
reported

32. King M.L. et al.:Transformation of PCFCL to DLBCL. Am J Case Rep, 2019; 20: 1273-1278

33. NHL is among the more unusual
diagnoses seen in Dermatology
clinic.
Appropriate diagnosis requires
tissue biopsy and a complete
systemic workup to rule out
secondary extension.

34. Case 3

35. 30-yrs-old lady presented with 5
slightly erythematous indurated
nodules on butter fly area and
over both eye brows without
scarring or ulcerations.
+ve history photosensitivity.
Medical history:

37. DD:
 PLE
 lymphocytic infiltrate of
jessner.
 Other LE (with atypical presentation).
 Sarcoidosis.

38. H/P:
Normal epidermis with no vacuolar deg. in basal layer. Sup. and deep perivascular
infiltrate with lymphocyte. Alcine blue –ve for mucin

39. The H/P was consistent
with TLE
BUT not definitive

40. Serological profile and DIF were
ordered to confirm diagnosis.
Topical steroids and tacrolimous oint.
were prescribed, with proper use of
sunscreen.

41.  ANA, Anti-ds-DNA and other
serological labs were done all
were negative.
 DIF showed moderate IgG, and
weak IgA, IgM, C3.
 CD4 and CD8 staining was not
available.

42. Tumid LE

44.  Topical tacrolimus showed
efficacy in DLE and cutaneous
manifestation of SLE.
 However, to my knowledge, there
is no evidence of the use of
tacrolimus in TLE in the literature
except in one publication.
Tzellos TG, Kouvelas D. Topical tacrolimous and pimacrolimous in the treatment od cutenous
LE. Eur.J clin Pharmacol.2008;64(4):337-341

45. Prashant V, Sonal S, Pravesh Y, Chaitanya N, Gauri M. Tumid Lupus Erythematosus: An Intriguing
Dermatopathological Connotation Treated Successfully with Topical Tacrolimus and
Hydroxyxhloroquine Combination. Indian Journal of Dermatology 2013; 58(5)

46. Despite being first described in
1909, there are few case reports
in the literature describing TLE
and even fewer that discuss
treatment.
Kuhn A, Bein D, Bonsmann G. The 100th anniversary of lupus erythematosus tumidus.
Autoimmun Rev. 2009;8:441-448.

47. Take home message
Although case reports are in the base of
scientific evidence hierarches, they
provides resources of unusual information
that may lead advances in clinical practice.
“Always note and record the unusual
…..Publish it. Such information's are
always of value”. (William Osler)