Wieland, Carilyn N.

AbstractAcral localized acquired cutis laxa (ALACL) is a rare variant of acquired cutis laxa, and the clinical appearance is characterized by loose, redundant and wrinkled skin of the distal extremities. By definition, histopathology of affected tissue reveals sparse or fragmented elastic fibers. However, this can be difficult to assess on routine staining, and sometimes requires electron microscopy. The condition has been associated with plasma cell dyscrasias or recurrent inflammatory states. We present a case of a 65‐year‐old man who presented with enlarged and doughy finger pads. Skin biopsy showed diffuse dermal amyloid deposition displacing dermal stroma and reduction of elastic fibers, although these changes were subtle on routine hematoxylin and eosin staining. Mass spectrometry of laser capture microdissected tissue showed AL kappa‐type amyloid and further workup revealed a diagnosis of primary systemic AL‐kappa amyloidosis requiring bone marrow transplantation. This case represents an unusual presentation of acquired cutis laxa and highlights the need for a high index of suspicion when reviewing histopathology of this entity. In addition, the case highlights the importance of investigation into possible systemic associations, such as plasma cell dyscrasias.

BackgroundGraft‐versus‐host disease (GVHD) following solid organ transplantation (SOT) is extremely rare and infrequently described in the dermatologic literature.MethodsWe performed a retrospective clinicopathologic review of our institution's experience with patients diagnosed with SOT‐associated GVHD (SOT GVHD) (May 1, 1996 to September 1, 2017).ResultsOf nine patients with SOT GVHD, seven had undergone liver transplantation, while two had undergone lung transplantation. All presented initially with a skin eruption, which developed an average of 63 days (range: 11‐162 days) post transplant. The average time to diagnosis following the onset of the skin eruption was 12 days (range: 0‐54 days). Diagnosis was often delayed because of a competing diagnosis of drug reaction. Frequent skin findings included pruritic erythematous to violaceous macules and papules with desquamation. Histopathology showed vacuolar interface dermatitis in 12 of 15 cases (80.0%). Of the 11 specimens in which a hair follicle was present for evaluation, vacuolar interface changes around the hair follicle were present in eight (72.7%) cases. Seven patients (77.8%) died from complications during the follow‐up period.ConclusionsSOT GVHD presents initially with skin involvement, is associated with vacuolar interface changes on skin biopsy, and is associated with a high mortality rate. Clinicopathologic correlation is required for accurate diagnosis.

AbstractDermatomyofibroma is a rare, benign mesenchymal proliferation not commonly reported in children. Two patients with biopsy‐proven dermatomyofibroma were identified (one female, one male) at our institution, both with rather atypical clinical presentations. The clinical and histopathologic findings and a review of the literature are presented here. Features of dermatomyofibroma should be recognized and differentiated from those of similar‐appearing entities since the prognosis of this benign tumor is favorable after complete excision.

Amicrobial pustulosis of the folds (APF) is a rare disease characterized by aseptic pustular lesions involving cutaneous folds, typically occurring in the context of an autoimmune disorder. We present 4 patients with APF, focusing on clinical and histopathologic characteristics to improve the recognition of this entity. All 4 patients had intertriginous and extra‐intertriginous involvement. Common histopathologic features of skin biopsies in these patients were intracorneal, subcorneal, intraepidermal, perivascular, perifollicular and interstitial neutrophilic inflammation. Pustules overlying adnexal ostium and papillary dermal edema were consistently observed. The pustules were negative for microorganisms on stain testing. In these cases, associated conditions were undifferentiated connective tissue disease, systemic lupus erythematosus, antiphospholipid syndrome, Crohn disease and Hashimoto thyroiditis. The aforementioned clinical and histopathologic characteristics of patients with suspected or diagnosed connective tissue disorders should lead to suspicion for APF.

A multiplex antibody-mediated capture technique reveals patient-specific protein complexes.

AbstractBackground: Penile and scrotal swelling can occur as an extraintestinal manifestation of Crohn's disease (CD) and is thought to be an uncommon form of metastatic CD (MCD). Because of the rarity of this manifestation, much is unknown concerning the presentation, treatment, and response to therapy in children with genital MCD. Methods: Boys ages 1 to 17 years presenting with genital edema and a confirmed diagnosis or strong suspicion of CD who were evaluated at the Mayo Clinic between 1996 and 2014 were included for review. We sought to characterize the clinical and pathologic features of genital MCD and response to treatment in our cohort of patients. Results: Eight patients with genital MCD were identified from our institution (mean age 11.4 yrs, range 7–16yrs). Seven (88%) patients experienced cutaneous symptoms before a formal diagnosis of CD was made, and two of the seven had no adverse gastrointestinal symptoms at that time. Patients were prescribed an average of 3.4 medications (topical and systemic) for management of their gastrointestinal CD and MCD. Conclusions: Penile and scrotal swelling can occur as an extraintestinal manifestation of CD and is thought to be an uncommon form of MCD. In boys, genital swelling typically precedes intestinal CD. Treatment of the underlying CD with systemic medications was most helpful in this series. An evaluation for CD is necessary in all patients presenting with unexplained genital swelling.

BackgroundCutis laxa‐like features were observed in a subset of patients with scleromyxedema. Given this observation, clinical and histopathologic features of scleromyxedema were reviewed in correlation with elastic tissue staining.MethodsWe retrospectively reviewed clinical records and histopathologic features from patients with scleromyxedema seen at our institution from 1992 through 2013. We also evaluated available skin biopsies with an elastin stain and assessed whether dermal elastin fibers were diminished in density or were fragmented (or both).ResultsNineteen patients with scleromyxedema and 34 skin biopsies were identified. Alcian blue (mucin) stain was used to grade mucin deposition as weakly positive (24%), positive (44%) and markedly positive (32%). Eight patients (42%) had clinical findings of cutis laxa, which were often observed in conjunction with areas of papular eruption or induration. Elastic tissue fibers were normal in 9 of 34 skin specimens (26%), 18 of 34 specimens (53%) had diminished elastic fiber density and 7 of 34 (21%) had markedly decreased density. The elastic tissue was fragmented in 25 specimens (74%).ConclusionsA cutis laxa‐like clinical presentation and decreased elastic tissue density on skin biopsy were consistent findings. Dermatologists and dermatopathologists should be aware of these previously unreported clinical and histopathologic findings.