THIRD SELF-ASSESSMENT IN DERMATOPATHOLOGY

BRITISH SOCIETY OF DERMATOPATHOLOGYMANCHESTER, JULY 06/2015

E Calonje, MD DipRCPath

St John’s Institute of Dermatology

London

CLINICAL HISTORYCASE 33

•Male, 78 year

•3-year history of papular rash on chest

•PMH: thrombocytopaenia (MDS), and diabetes mellitus (oral therapy)

CD33 Lysozyme

CD163 CD123

MPO CD117

CD34 CD56

CD3 CD2 CD5

CD4 CD20CD30

Ki67

CASE 33

The best diagnosis is:

1.Interstitial granulomatous dermatitis2.Monocytic cutaneous infiltrate associated

with myelodysplasia3.Histiocytic Sweet syndrome4.Granulomatous CTCL

CASE 33

The best diagnosis is:

1.Interstitial granulomatous dermatitis2.Monocytic cutaneous infiltrate associated

with myelodysplasia3.Histiocytic Sweet syndrome4.Granulomatous CTCL

Diagnosis: Monocytic cutaneous infiltrate associated withmyelodysplasia

Phenotype

+: CD33, CD4, CD163, lysozyme± CD163-: CD34, CD117, CD56, TdT, MPO, panT cell markers, B cell markers

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

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%

Arch Dermatol. 2011;147(3):331-335

Granulomatous Dermatitis Accompanied by MyelodysplasticSyndrome

Acta Derm Venereol 2014; 94: 223–224

Aleukemic cutaneous myeloidsarcoma

J Cutan Pathol 2013: 40: 996–1005

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

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