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MOC Self-Assessment MDSA 2016 Meeting M. Yadira Hurley, MD Professor Dermatology and Pathology Department of Dermatology Saint Louis University I have no relevant conflicts of interest disclosures 1) A biopsy shows a dense infiltrate of atypical lymphocytes within the dermis. The atypical lymphocytes are CD3+, CD4+, and CD30+. The histologic differential diagnosis includes lymphomatoid papulosis and cutaneous anaplastic large cell lymphoma. Which of the following is the most critical information for clinicopathologic correlation? A. B. C. D. E. B-symptoms Clinical course of complete regression Location of skin lesions Patient age Ulceration A biopsy shows a dense infiltrate of atypical lymphocytes within the dermis. The atypical lymphocytes are CD3+, CD4+, and CD30+. The histologic differential diagnosis includes lymphomatoid papulosis and cutaneous anaplastic large cell lymphoma. Which of the following is the most critical information for clinicopathologic correlation? A. B. C. D. E. B-symptoms Clinical course of complete regression Location of skin lesions Patient age Ulceration • Lymphomatoid papulosis and Cutaneous anaplastic large cell lymphoma are included in the WHO/EORTC CD30+ cutaneous lymphoproliferative disorders • Lymphomatoid papulosis • Typically seen in adults but children may be affected • Characterized by chronic, recurrent papules and nodules that can ulcerate and heal within 6 weeks • There are 5 histologic subtypes • Cutaneous anaplastic large cell lymphoma • Affects adults but younger patients may be affected • Solitary tumors are characteristic and frequently ulcerate • Histopathology shows nodular of diffuse infiltrate involving the dermis and extending into the subcutaneous fat that are CD30+ • Lesions are persistent and may show only partial regression • B-symptoms are fever, weight loss, and night sweats • Typically seen in systemic lymphomas • Primary cutaneous CD30+ lymphoproliferative disorder are not associated with Bsymptoms 2) A 65 year old male with scaly patches on the trunk and buttocks has a prior punch biopsy that showed psoriasiform dermatitis with dermal fibrosis and a dermal lymphocytic infiltrate without evidence of epidermotropism. You have a high clinical suspicion for mycosis fungoides. What additional type of biopsy would be best to confirm a diagnosis of mycosis fungoides? A. B. C. D. E. 2 mm punch biopsy in formalin 3 mm punch biopsy in formalin 3 mm punch biopsy in Roswell Park Memorial Institute medium Broad shave biopsy in formalin Incisional biopsy to include subcutaneous tissue A 65 year old male with scaly patches on the trunk and buttocks has a prior punch biopsy that showed psoriasiform dermatitis with dermal fibrosis and a dermal lymphocytic infiltrate without evidence of epidermotropism. You have a high clinical suspicion for mycosis fungoides. What additional type of biopsy would be best to confirm a diagnosis of mycosis fungoides? A. B. C. D. E. 2 mm punch biopsy in formalin 3 mm punch biopsy in formalin 3 mm punch biopsy in Roswell Park Memorial Institute medium Broad shave biopsy in formalin Incisional biopsy to include subcutaneous tissue • Histologically early (patch stage) mycosis fungoides shows papillary dermal fibrosis, a superficial perivascular infiltrate that spares the underside of the post-capillary venule, mild vacuolar interface dermatitis, and an epidermotropic lymphocytic infiltrate • The reticular dermis and subcutaneous tissue are not involved • A broad shave biopsy that samples the upper reticular dermis is ideal to observe the characteristic histologic findings including epidermotropism • Roswell Park Memorial Institute medium (RPMI) is used to transport fresh tissue • Clonality of T-cells can be established by polymerase chain reaction of T-cell receptor gene rearrangements (TCRs) • Until recently, fresh tissue was of higher yield for TCRs; however, these studies can now be performed on formalin-fixed paraffin-embedded tissue • A broad shave biopsy is also preferred for molecular studies as more DNA can be extracted by maximizing the malignant epidermotropic infiltrate • Clonality may be stage dependent and is seen in only 20-40% of patch stage mycosis fungoides as there are admixed inflammatory T-cell • Several benign dermatoses such as pityriasis lichenoides, have shown monoclonal populations of T-cell lymphocytes 3) A 60 year old female that is neutropenic and has septicemia presents with a gangrenous ulcer with a dark eschar and an erythematous halo. You suspect this is ecthyma gangrenosum. Which is most likely causative organism? A. B. C. D. E. Corynebacterium minutissimum Group A Beta-haemolytic Streptococcus Pseudomonas aeuriginosa Rhizopus arrhizus Staphylococcus aureus 3) A 60 year old female that is neutropenic and has septicemia presents with a gangrenous ulcer with a dark eschar and an erythematous halo. You suspect this is ecthyma gangrenosum. Which is most likely causative organism? A. B. C. D. E. Corynebacterium minutissimum Group A Beta-haemolytic Streptococcus Pseudomonas aeuriginosa Rhizopus arrhizus Staphylococcus aureus A 60 year old female that is neutropenic and has septicemia presents with a gangrenous ulcer with a dark eschar and an erythematous halo. You suspect this is ecthyma gangrenosum. Which is most likely causative organism? A. B. C. D. E. Corynebacterium minutissimum Group A Beta-haemolytic Streptococcus Pseudomonas aeuriginosa Rhizopus arrhizus Staphylococcus aureus • Ecthyma gangrenosum is a condition which is pathognomonic of Pseudomonas septicemia (Pseudomonas aeruginosa) and is usually seen in immunocompromised patients, particularly those with underlying malignant disease • There are cases in the literature that have been reported in immunocompetent patients, not associated with septicemia and caused by other bacterial or fungal microorganisms • Ecthyma-like lesions • Clinically these lesions present as an erythematous macule or patch that progresses to vesiculopustular papules or plaque resulting in a gangrenous ulcer with a necrotic eschar and surrounding erythema • Erythrasma – Corynebacterium minutissimum • Infectious Perianal Erythema - Group A Beta-haemolytic Streptococcus • Mucormycosis (zygomycosis) – Rhizopus arrhizus • Skin abscess – Staphylococcus aureus 4) A 25 year old female presents with pustules on the lip and gingiva. A diagnosis of pyostomatitis vegetans is rendered. Pyostomatitis vegetans is associated with which of the following? A. B. C. D. E. Celiac disease Colon cancer Esophageal carcinoma Inflammatory bowel disease Recurrent epistaxis 4) A 25 year old female presents with pustules on the lip and gingiva. A diagnosis of pyostomatitis vegetans is rendered. Pyostomatitis vegetans is associated with which of the following? A. B. C. D. E. Celiac disease Colon cancer Esophageal carcinoma Inflammatory bowel disease Recurrent epistaxis A 25 year old female presents with pustules on the lip and gingiva. A diagnosis of pyostomatitis vegetans is rendered. Pyostomatitis vegetans is associated with which of the following? A. B. C. D. E. Celiac disease Colon cancer Esophageal carcinoma Inflammatory bowel disease Recurrent epistaxis • Pyostomatitis vegetans (PV) has similarities with pyoderma gangrenosum • PV is characterized by pustules and ulceration of the lips and buccal mucosa • PV is an oral manifestation of inflammatory bowel disease, more commonly seen in patients with ulcerative colitis • Inflammatory bowel disease can precede PV by years but PV can also be a marker of subclinical intestinal disease • Pathogenesis is attributed to antigens that cross-react between the bowel and the skin and immune dysregulation is considered the cause. • Dermatitis herpetiformis is associated with celiac disease • Sebaceous neoplasms are associated with colon cancer in the setting of Muir-Torre syndrome • Tylosis is focal non frictional and non epidermolytic palmoplantar hyperkeratosis • Type A occurs between the age of 5 and 15 years and is associated with a high incidence of esophageal carcinomas (Howell–Evans syndrome) • Multiple, small punctate telangiectases predominantly on the face or mouth are associated with hereditary hemorrhagic telangiectasia with recurrent epistaxis being the primary presenting manifestation 5) A 60 year old female is has a pink pearly papule that on biopsy shows a sebaceous tumor. What clinicopathologic finding has the highest specificity for DNA mismatch repair (MMR) deficiency and a diagnosis of Muir-Torre syndrome? A. B. C. D. E. Age Associated erythema Non head and neck location Sex Tumor type A 60 year old female has a pink pearly papule that on biopsy shows a sebaceous tumor. What clinicopathologic finding has the highest specificity for DNA mismatch repair (MMR) deficiency and a diagnosis of Muir-Torre syndrome? A. B. C. D. E. Age Associated erythema Non head and neck location Sex Tumor type • Several clinicopathological features (age, sex, tumor type, tumor infiltrating lymphocyte count, peritumoral lymphocytic response) show some correlation with mismatch repair protein (MMR) deficiency • MMR deficiency has been significantly associated with site • Tumors on non head and neck locations are more likely to be MMR deficient (specificity 96% in a recent study) • Sensitivity for non head and neck location (41% in a recent study) • There may be a role for MMR protein deficiency testing by immunohistochemistry (IHC) as an initial screening tool, regardless of site • Patient is diagnosed with a sebaceous neoplasm and MMR protein deficiency by IHC • Additional family history • Application of the Modified Amsterdam Criteria and/or Revised Bethesda Guidelines • There is a risk of a false-negative MMR protein IHC results • Any patients with multiple sebaceous neoplasms OR a patient with one sebaceous tumor and a personal or family history of a Lynch Syndrome associated malignancy should be referred for genetic counseling • Lynch Syndrome associated malignancies: colorectal, endometrial, stomach, ovarian, small bowel, urinary tract, prostate, and hepatobiliary