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THIRD SELF-ASSESSMENT IN DERMATOPATHOLOGY
BRITISH SOCIETY OF DERMATOPATHOLOGYMANCHESTER, JULY 06/2015
E Calonje, MD DipRCPath
St John’s Institute of Dermatology
London
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CLINICAL HISTORYCASE 33
•Male, 78 year
•3-year history of papular rash on chest
•PMH: thrombocytopaenia (MDS), and diabetes mellitus (oral therapy)
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CD33 Lysozyme
CD163 CD123
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MPO CD117
CD34 CD56
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CD3 CD2 CD5
CD4 CD20CD30
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Ki67
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CASE 33
The best diagnosis is:
1.Interstitial granulomatous dermatitis2.Monocytic cutaneous infiltrate associated
with myelodysplasia3.Histiocytic Sweet syndrome4.Granulomatous CTCL
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CASE 33
The best diagnosis is:
1.Interstitial granulomatous dermatitis2.Monocytic cutaneous infiltrate associated
with myelodysplasia3.Histiocytic Sweet syndrome4.Granulomatous CTCL
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Diagnosis: Monocytic cutaneous infiltrate associated withmyelodysplasia
Phenotype
+: CD33, CD4, CD163, lysozyme± CD163-: CD34, CD117, CD56, TdT, MPO, panT cell markers, B cell markers
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Myelodysplastic syndrome (MDS)
• Represent a spectrum of ineffective haematopoiesis
• WHO classification: dysplasia, excess of marrow myeloblasts, and specific clonal chromosomal abnormalities
Modified from Medscape Education Oncology
30-40%
≥20% blast
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Skin lesions and MDS
Non-specific
• Neutrophilic dermatoses (Sweet syndrome and pyodermagangrenosum)
• Leucocytoclastic vasculitis and Behçet disease
Specific Lesions
• Leukaemia cutis
• “Denotes the cutaneous infiltration of neoplastic myeloid cells or lymphoid blasts”
• Often occurring in the setting of AML
Granulomatous dermatitis?
10-25%
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Arch Dermatol. 2011;147(3):331-335
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Granulomatous Dermatitis Accompanied by MyelodysplasticSyndrome
Acta Derm Venereol 2014; 94: 223–224
Aleukemic cutaneous myeloidsarcoma
J Cutan Pathol 2013: 40: 996–1005
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The presence of leukaemia cutis is regarded as a sign ofrecurrent or progressing systemic disease
JEADV 2010, 24, 1171–1175
International Journal of Dermatology 2012, 51, 383–388
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Conclusions
• Monocytic infiltrates might represent the initial stage of a cutaneous dysgranulopoiesis syndrome and should lead to the search for atypical myelo-monocytic cells in peripheral blood, and bone marrow for the early detection of an associated myelodysplastic syndrome
• It is still not clear whether the specific skin manifestations of MDS, is or could be considered an early sign of evolving acute leukaemia