Dermatoses you ve probably seen but never heard of Summer AAD 2017, New York, NY. Anneli R Bowen, MD

Transcription

1 Summer AAD 2017, New York, NY Case 1 Anneli R Bowen, MD Diagnosis: Pretibial pruritic papular dermatitis (PPPD) Forty-four patients described by Annessi Pruritic, erythematous, smooth papules Thought to be a response to gentle, chronic rubbing, akin to dermal lichen simplex chronicus Chronic course, responds to topical steroids, but often recurs after stopping treatment Mild orthokeratosis and mild acanthosis, flattening of rete ridge pattern Superficial to mid-dermal perivascular lymphocytic inflammation with variable numbers of histiocytes and eosinophils Superficial dermal fibrosis with multinucleated fibroblasts Main differential diagnoses: Lichen simplex chronicus Lichen amyloidosis PPPD is a distinctive clinical and histologic reaction to persistent rubbing of the skin Reference: Annessi G, Petresca M, Petresca A. Pretibial pruritic papular dermatitis: a distinctive cutaneous manifestation in response to chronic rubbing. Am J Dermatopathol 2006;28:117-21

2 Case 2 Anneli R Bowen, MD Diagnosis: Insulin-derived nodular amyloidosis Hard, hyperpigmented nodule at site of insulin injection Longstanding insulin-dependent diabetic patients who don t rotate insulin injection site Usually have high or increasing insulin requirements Erratic glucose control May be associated with acanthosis nigricans-like epidermal hyperplasia Dermal nodules of hypereosinophilic, homogenous material that stains with Congo red, showing typical apple-green birefringence under polarized microscopy Infiltrate of plasma cells, the presumed source of amyloid deposits Amyloid deposit positive for insulin by immunohistochemistry Insulin-derived nodular amyloidosis is a potential complication of insulin injection in diabetics that do not rotate insulin injection site and can result in poor glucose control Reference: Endo JO, et al. Nodular amyloidosis in a diabetic patient with frequent hypoglycemia: sequelae of injecting insulin without site rotation. JAAD 2010;63: Kudo-Watanuki S, et al. Coexistence of insulin-derived amyloidosis and an overlying acanthosis nigricans-like lesion at the site of insulin injection. Clin Exp Dermatol 2013;38:25-9

3 Case 3 Anneli R Bowen, MD Diagnosis: Hyperkeratotic and lichenified dermatosis of the gluteal region (senile gluteal dermatosis) Common dermatosis of the elderly who spend most of the day sitting Thin, male:female 130:7 Itching or pain of varying intensity, may be asymptomatic Brownish plaques on the gluteal cleft of the buttocks three corners of a triangle Horizontal hyperkeratotic linear ridges a characteristic sign Treatment difficult Hyperkeratosis Acanthosis Follicular plugging No amyloid deposits Main differential diagnoses: Anosacral amyloidosis (lichen amyloid) Irritant/allergic contact dermatitis Lichen simplex chronicus Mycosis fungoides This is a common dermatosis of the elderly that has received little attention Reference: Niiyama S, Sakurai S, Katsuoka K. Hyperkeratotic lichenified skin lesion of gluteal region. J Dermatol 2006;33: Liu H-N, et al. Senile gluteal dermatosis: a clinical study of 137 cases. Int J Dermatol 2014;53:51-5.

4 Case 4 Anneli R Bowen, MD Diagnosis: Cutaneous collagenous vasculopathy Described in 2000 by Salama and Rosenthal Telangiectasias usually start in middle age on lower legs, progresses to become more generalized Male=female May darken over time No associated underlying disease, no history of hemorrhage Likely under recognized/confused with generalized essential telangiectasia Dilated superficial vessels Thickened walls (reduplicated basement membrane material) o Seen well on PAS and immunohistochemical staining for Collagen IV o These characteristic changes can be subtle or focal Main differential diagnoses: Without systemic implications o Generalized essential telangiectasia o Unilateral nevoid telangiectasia o Hereditary benign telangiectasia With systemic implications o Hereditary hemorrhagic telangiectasia o Ataxia telangiectasia o Liver disease o Increased estrogen states Benign, progressive, asymptomatic generalized telangiectasia associated with characteristic reduplication of basement membrane histologically Salama S, Rosenthal D. Cutaneous collagenous vasculopathy with generalized telangiectasia: an immunohistochemical and ultrastructural study. J Cutan Pathol Jan;27(1):40-8 Salama SS. Cutaneous collagenous vasculopathy: a new case series with clinicopathologic and ultrastructural correlation, literature review, and insight into the pathogenesis. Am J Dermatopathol May;37(5):

5 Case 5 Anneli R Bowen, MD Diagnosis: Granulomatous pigmented purpuric dermatosis Uncommon variant of pigmented purpuric dermatosis Clinically, lesions resemble other pigmented purpuric dermatoses Like other pigmented purpuric dermatoses, most common on lower extremities but may involve trunk Tenuous association with hyperlipidemia- requires more study to confirm Superficial and sometimes deep dermal perivascular lymphocytes and histiocytes with extravasated erythrocytes. May have siderophages Main differential diagnoses: Other pigmented purpuric dermatoses o Schamberg s purpura o Lichenoid purpura of Gougerot and Blum o Eczematoid purpura of Doucas and Kapetanakis o Lichen aureus o Purpura annularis telangiectodes Capillaritis-like mycosis fungoides Granulomatous pigmented purpuric dermatosis is an uncommon variant of pigmented purpuric dermatoses- possibly associated with hyperlipidemia, may demonstrate deep dermal involvement histologically Reference: Macquarrie EK1, Pasternak S, Torok M, Veerassamy S, Walsh NM. Persistent pigmented purpuric dermatitis: granulomatous variant. J Cutan Pathol Dec;38(12): Hanson C, Fischer R, Fraga G, Rajpara A. Granulomatous pigmented purpuric dermatosis: an unusual variant associated with hyperlipidemia. Dermatol Online J Nov 16;21(2).

6 Summer AAD 2017, New York, NY Case 1 Scott R Florell, MD Diagnosis: Collagenous and elastotic marginal plaques of the hands Other names: Keratoelastoidosis marginalis, degenerative collagenous plaques of the hands, digital papular calcific elastosis Five cases reported as degenerative collagenous plaques of the hands in 1960 Male predominance, mean age ~ 70 in a series of 20 patients, ranges from years Bilateral, symmetrical linear plaques at the junction of the dorsal and palmar skin on medial thumb, extending to radial index finger Slow progression for many years before presentation, most patients asymptomatic with stiffness occasionally reported Etiology unknown, hypotheses include chronic actinic damage, chronic pressure, altered collagen and elastin Hyperkeratosis and epidermal acanthosis with loss of rete ridge pattern Haphazard arrangement of thickened collagen bundles in the dermis, some oriented perpendicular to the epidermis Dermis is relatively acellular and avascular Fragmented elastic fibers Basophilic elastotic masses rounded degenerative aggregates of elastic material that may calcify The findings are distinctive clinically and histologically Burks JW, Wise LJ, Clark WH. Degenerative collagenous plaques of the hands. Arch Dermatol 1960;82: Abulafia J, Vignale RA. Degenerative collagenous plaques of the hands and acrokeratoelastoidosis: pathogenesis and relationship with knuckle pads. Int J Dermatol 2000;39: Mortimore RJ, Conrad RJ. Collagenous and elastotic marginal plaques of the hands. Australas J Dermatol 2001;42: Tieu KD, Satter EK. Thickened plaques on the hands. Arch Dermatol 2011;147:

7 Case 2 Scott R Florell, MD Diagnosis: Annular lichenoid dermatitis of youth Twenty-three cases described in 2003, ~ 50 cases described to date Lesions present mostly on the torso and groin Red macules to patches, become hypopigmented centrally leaving an annular ring with a brown edge A few cases have been described in adults Clinical differential diagnosis includes mycosis fungoides, morphea, vitiligo, other annular erythemas Etiology unknown - Austrian group found Borrelia spirochetes in 11/14 biopsies, suggested ALDY may be an early, superficial stage of morphea Treatment empiric, including topical corticosteroids, tacrolimus, phototherapy Usually chronic/recurrent clinical course Lichenoid interface dermatitis Hyperplastic epidermis with thinned, sometimes quadrangular rete Exocytosis of lymphocytes Keratinocyte apoptosis Can be difficult to distinguish from mycosis fungoides; T-cell monoclonality has not been described in ALDY so T-cell gene rearrangement studies could be helpful in some cases Main Differential Diagnoses: Mycosis fungoides Inflammatory vitiligo Morphea Annular erythema Annular lichenoid dermatitis (of youth) shares clinical and histologic features with mycosis fungoides; remember this differential diagnostic consideration with truncal, round to annular clinical lesions with lichenoid histology Annessi G, et al. Annular lichenoid dermatitis of youth. J Am Acad Dermatol 2003;49: Kazlouskaya V, Trager JDK, Junkins-Hopkins JM. Annular lichenoid dermatitis of youth: a separate entity or on the spectrum of mycosis fungiodes? Case report and review of the literature. J Cutan Pathol 2015;42: Di Mercurio M, et al. Annular lichenoid dermatitis of youth: report of six new cases with review of the literature. Dermatology 2015 July 9, Epub. Cesinaro AM, et al. Annular lichenoid dermatitis of youth and beyond: a series of 6 cases. Am J

8 Dermatopathol 2009;31: Wilk M, et al. Annular lichenoid dermatitis (of youth) Immunohistochemical and serological evidence for another clinical presentation of Borrelia infection in patients of Western Austria. Am J Dermatopathol 2017; 39:

9 Case 3 Scott R Florell, MD Diagnosis: Circumscribed palmar hypokeratosis Ten patients described by Perez in 2002 ~ 70 cases described to date, mostly in middle age or elderly Most patients present with a solitary lesion on the right (dominant) palm, mostly thenar or hypothenar Etiology unknown,? localized disorder of keratinization No specific treatment typically prolonged course Abrupt loss and thinning of the stratum corneum Main Differential Diagnosis: Porokeratosis The findings are distinctive clinically and histologically Annessi G, et al. Annular lichenoid dermatitis of youth. J Am Acad Dermatol 2003;49: Perez G, et al. Circumscribed palmar or plantar hypokeratosis: a distinctive epidermal malformation of the palms or soles. J Am Acad Dermatol 2002;47:21-7. Urbina F, et al. Circumscribed palmar or plantar hypokeratosis 10 years after the first description: what is known and issues under discussion. Actas Dermosifiliogr 2014;105: Huh W, Kanitakis J. Annular lichenoid dermatosis of youth: report of the first Japanese case and published work review. J Dermatol 2010;37: Barry CI, et al. Circumscribed palmar hypokeratosis: two cases and a review of the literature. J Cutan Pathol 2008;35:484-7.

10 Case 4 Scott R Florell, MD Diagnosis: Demodex folliculorum-associated spinulosis Tiny follicular spicules involving face Predisposing factors include increased age, diabetes mellitus, hemodialysis and immunosuppression Several cases have been reported in the setting of polycythemia rubra vera and hydroxyurea therapy Treatment: topical permethrin, ivermectin, oral ivermectin Follicular spicules composed of Demodex folliculorum mites with associated hyperkeratosis Minimal associated dermal inflammation Treatment: Crotamiton topical Permethrin topical Ivermectin topical Ivermectin oral Demodex folliculorum-associated spinulosus is an important entity to remember when evaluating a patient with facial follicular spicules because it is easy diagnosed and may be easily treatable Curtis JA, Florell SR, Zussman J. Sandpapery skin. Arch Dermatol 2015;151: Farina MC, et al. Spinulosis of the face as a manifestation of demodicidosis. Br J Dermatol 1998;138:901-3.

11 Case 5 Scott R Florell, MD Diagnosis: White fibrous papulosis of the neck (WFPN) Multiple asymptomatic whitish to yellowish papules on the lateral and posterior neck Men and women older than 40 years of age Etiology unknown,? intrinsic cutaneous aging or chronic UV exposure No specific treatment Routine H&E may look like normal skin, may show mild thickening of collagen bundles Decreased to absent dermal elastic tissue Main Differential Diagnoses: Pseudoxanthoma elasticum-like papillary dermal elastolysis (PXE-PDE) Papillary dermal elastosis (PDE) Mid-dermal elastolysis Linear focal elastolysis The findings are distinctive clinically and histologically. White fibrous papulosis of the neck may be part of a spectrum of similar disorders termed fibroelastolytic papulosis, including WFPN, PXE-PDE, and PDE. Chan Song Y, et al. A case of fibroelastolytic papulosis on the neck of a young man. Ann Dermatol 2011;23: Alves R, et al. Pseudoxanthoma elasticum papillary dermal elastolysis: a case report. Derm Res Prac 2010, epub. Patterson AT, Beasley KJ, Kobayashi TT. Fibroelastolytic papulosis: histopathologic confirmation of disease spectrum variants in a single case. J Cutan Pathol 2016;43: Shimuzu H, et al. White fibrous papulosis of the neck: a new clinicopathologic entity? JAAD 1989;20; Schaffer PR, et al. White fibrous papulosis of the neck. Derm Online J 2015;20:6. Val-Bernal JF, Gonzalez-Vela MC, Leon-Castillo A, Armesto S. Papillary dermal elastosis. Am J Dermatopathol 2017;39:150-2.